КНИГА СО ТРУДОВИ - zpm.org.mk · Remedika - Skopje and Acibadem Sistina Hospital Skopje, each. Only one newborn with CH was - Only one newborn with CH was - obtained

ШЕСТТИ КОНГРЕС НА ЗДРУЖЕНИЕТО НА ПЕДИЈАТРИ НАРЕПУБЛИКА МАКЕДОНИЈА СО ИНТЕРНАЦИОНАЛНО УЧЕСТВО

КНИГА СО ТРУДОВИ

ГЕНЕРАЛЕН СПОНЗОР:

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ШЕСТТИ КОНГРЕС НА ЗДРУЖЕНИЕТО НА ПЕДИЈАТРИ НА РЕПУБЛИКА МАКЕДОНИЈА СО ИНТЕРНАЦИОНАЛНО УЧЕСТВО

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ОРГАНИЗАЦИОНЕН ОДБОР

ПРЕТСЕДАТЕЛ НА ЗДРУЖЕНИЕТО НА ПЕДИЈАТРИТЕ НА РЕПУБЛИКА МАКЕДОНИЈАДоц.д-р.Сци Аспазија Софијанова

ПРЕТСЕДАТЕЛ НА ОРГАНИЗАЦИОНЕН ОДБОРД-р. Оливер Зафировски

ГЕНЕРАЛЕН СЕКРЕТАР:Доц. д-р Сци Соња Бојаџиева

ПОТПРЕТСЕДАТЕЛИ:Д-р Татјана Баевска Вучковиќ Д-р Македонка ГлигороваД-р Беса Ислами Поцеста

Управен одбор: Проф Д-р Роза КацарскаД-р Катерина СтарковскаД-р Олгица СтефановскаМ-р Д-р Кировски ИлијаД-р Лидија Спасова Д-р Светлана Крстевска Д-р Светлана ЗотовскаД-р Методија ТомевскиД-р Весна ЃорѓевскаД-р Славица ТодоровскаД-р Слаѓана РистовскаД-р Ферид Муча Д-р Аголи АрбенД-р Билјана РуменоваД-р Габриела ТавчиовскаД-р Теута Јакупи Д-р Џабир БајрамиД-р Катерина КузмановскаД-р Дончо ДервишовД-р Валентина ЗлатановскаД-р Аднан СулејманиД-р Весна Марковска Д-р Лидија НешковскаД-р Розита Стојковска

НАУЧЕН ОДБОР

ПРЕТСЕДАТЕЛ НА НАУЧНИОТ ОДБОРПроф Д-р Розана Кацарска

ЧленовиПроф Д-р Ката МартиноваПроф Д-р Филип ДумаПроф Д-р Стојка Фуштиќ Проф д-р Емилија ВлашкиДоц Д-р Сци Аспазија СофијановаДоц Д-р Лидија КареваДоц Д-р Сци Катерина Ставриќ Доц.Д-р Сци Кочева Светлана Доц Д-р Сци Соња БојаџиеваДоц Д-р Сци Констандина Кузевска-МаневаНауч сор Д-р Емилија Шахпазова

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UDK: 61+061.231=866=20CODEN: MK MPA 3ISSN0025-1097

МАКЕДОНСКО ЛЕКАРСКО ДРУШТВО

Мaкедонски медицински прегледСписaние нa Мaкедонското лекaрско друштво

Год: 89 (supl.91), стр. 1- 187, 2015

Зборник на Апстракти за Шести Конгрес на лекарите на педијатрите на Р. Македонија со меѓународно учество

08-11, октомври, 2015, Струга

UDK:61+061.231=866=20 Год: 89 (supl.91), Page 1 CODEN:MK MPA 3 ISSN0025-1097

Acute peritoneal dialysis in children with acute kidney injury: our experience.

N. Emini 1, E. Shahpazova1, V.Tasic 1, D. Kuzmanovska1

1 Nephrology Department, University Children`s Hospital, Skopje, Macedonia

Objective: While the use of continuous renal replacement therapy in the management of children with acute kidney injury (AKI) in the recent era has increased, peritoneal dialysis remains a viable option, particularly for patients who are hemodynamically compromised or have severe coagulation anomalies, or when other modalities are not available.

Methods: Retrospective review of medical histories from children with AKI managed with acute PD during the period from January 2004 to June 2015 in University Children`s Hospital Skopje.

Results: Twenty children with a mean age 19.2 months (age range 1 – 91 months), were managed with acute PD during 1 – 23 days (mean duration 11.65 days). Twelve of the patients were infants, and eleven of them were male. Causes of AKI were hemolytic - uremic syndrome 30 %, acute tubular necrosis 25 %, congenital heart malformation (after open heart surgery) 25 %, septicemia 15 % and other 5 % of cases. Access for PD in 13 cases consisted in surgically placed Tenckhoff catheters and in 7 cases the catheter was placed percutaneosly at the bedside. Automated pertitoneal dialysis was performed at 15 cases. Outcome at 11 of patients was good with complete resolution of renal function, 2 ended with chronic kidney disease, 6 died (half of them after open heart surgery) and 1 was lost from follow up.

Conclusions: Peritoneal dialysis remains safe and effective solution for management of acute kidney injury in children, especially in resource limited countries. From our experience complications and catheter leaking is very rare in surgically placed peritoneal catheter. Automated peritoneal dialysis is also very comfortable and gentle method during acute peritoneal dialysis.


Pharmacist role in pharmacotherapy counseling, a necessary obligation – our experience Author: Mr.Sci.PH.Milaim Abdullahu¹ President of pharmaceutical company SANTEPHARM Coauthor: Rinor Abdullahu¹ Pharmacy student Manager at KF SANTEFARM Mr.Sci.Dr.Armend Vuqiterna² MD,PhD Candidate – Pediatric Neurologist University Clinical Centre of Kosovo, Pediatrics Clinic – Neuropediatrcs department Dr.Vlora Nimani² University Clinical Centre of Kosovo, Pediatrics Clinic Introduction: Based on the continuous changes on the health care system, in recent years the role of pharmacologist has undergone some changes as well. Pharmacologist’s responsibilities nowadays are based on the resolution of the World Health Organization. The role of pharmacologists on supporting the new strategy on medicament usage, highlights the duty of pharmacologist on counseling how to use the medicaments and their active role on disease prevention and health upkeep. This way the pharmacologist is a necessary member of health care team and carries a part of responsibility on pharmacotherapy results and life quality of the patients. His role should be clearly assigned on the health care system and on the national policy on medicament usage equally in every country. As a health care professional he should provide the securest and most effective pathway usage of medicaments that doctors prescribe. Purpose of work: is to show our experience, value and give important role to the pharmacologist on pharmacology consultation as a necessary duty, making it so the pharmacologist to be an important part of the health care team, and taking the responsibilities on pharmacotherapy results and life of the patients. Work method: study group was made of 50 pharmacies, who had present a pharmacologist. In a prospective way, on dynamic follow we have analyzed all the pharmacies where the pharmacologist was present. The work was done during the period February- June 2015. Results: Based on the results we came to a conclusion that: we have achieved e higher health care of patients on the presence of pharmacologist, on pharmacotherapy counseling where the pharmacologist plays a big and necessary part on pharmacotherapy. Conclusions: based on our experience, we recommend that every pharmacologist should play an active part on disease prevention and health upkeep. Key words: The pharmacologist role an obligation, pharmacotherapy, our experience.


Neonatal hyperlipidemia

Aluloska N1, Palchevska S1, Papazovska A1, Zdraveska N1, Piperkova K1, Tasic V2, Ezgu F3

1Neonatology Department, University Children Hospital, Skopje, Macedonia

2Nephrology Department, University Children Hospital, Skopje, Macedonia

3Department and Laboratory of Pediatric Metabolic Disorders, Gazi University, Ankara

Neonatal hyperlipidemia is a rare disease occurring in genetically related families and it affects about 1 out of 1 000 000 people, mostly due to genetic dyslipidemia. Patients with this disorder rarelypresent during the neonatal period. During infancy this patientshave increased risk of pancreatitis and they present with hepatosplenomegaly, lipemiaretinalis and eruptive xanthomas. Recurrent pancreatitis ultimately leads to pancreatic insufficiency, which is the major threat of this disease. Here we present a case of two weeks old newborn infant who presented to our neonatal ward with milky serum detected on routine blood checkup for febrile illness. This newborn infant is the first childcoming from a family from Turkish etnicity and with consanguinity.Given the potentialrisk of an extracorporeal technique such as plasmapheresis is such a small infant, the lack of evidence for exchange transfusion as a method of treatment, we decided to treat the baby administering aspecial low fat formula high in medium chain triglycerides. This turned out to be effective in keeping fasting plasma triglycerides level in approximately normal range.

Key wards: Newborn, hyperlipidemia, genetic, diet


Sy Zellweger- un unusual presentation Aluloska N.1, Palcevska S. 1, Papazovska A. 1, Zdraveska N. 1, Tasic V.2, Peter M.3

1 Neonatology Department, University Children Hospital, Skopje, Macedonia 2 Nephrology Department, University Children Hospital, Skopje, Macedonia 3 Screening-Laboratory, Hannover, Germany

Abstract

Peroxisomal disorders are a group of genetically heterogeneous metabolic diseases that share dysfunction of peroxisomes. The Zellweger spectrum disorders (ZSDs) are characterized by a generalized loss of peroxisomal functions caused by deficient peroxisomal assembly. Zellweger syndrome (ZWS) is the most common peroxisomal disorder to manifest itself in early infancy. Its incidence has been estimated to be 1 in 50,000-100,000. Clinical presentation and survival are heterogeneous.

Here we present a 2 week old male newborn affected by Zellweger syndrome with an unusual presentation. During the regular fetal ultrasonography large polycystic kidneys were noted and postnatal nephrologic workout was suggested. The baby was admitted to Neonatal Department suspected to have polycystic kidney disease. Clinical features of severe hypotonia, facial appearance, redundant neck skin, ventricular septal defect, talipies equinovarus, renal cystic disease suggested the possibility of Zellweger syndrome. The diagnosis was confirmed by biochemical/metabolic assessment – increased concentration of cytosomal bile acids precursors di- and three-hydroxychoolestanoic acid.

In conclusion – prenatal renal ultrasound pattern may mimic polycystic kidney diseases in fetuses with Zellweger spectrum disorders. Significant extrarenal signs and metabolic investigation were clue to correct diagnosis in our case.

Key wards: Zellweger, newborn, polycystic kidney disease


Molecular diagnosis of the macedonian patients with nonclassical 21-hydroxylase deficiency

Violeta Anastasovska and Mirjana Kocova

Genetic Laboratory, Department of Endocrinology and Genetics, University Children’s Clinic, Skopje, Republic of Macedonia

Background: The mild nonclassic form of steroid 21-hydroxylase deficiency is one of the most common autosomal recessive disorders causing premature adrenarche and pubarche, virilization in young women and variable symptoms in young men. It is commonly associated with the missense P30L and V281L mutations in CYP21A2 gene for 21-hydroxylase, that produce 20-60% of normal enzyme activity.

Methods: Using the PCR/ACRS method, we have studied six common CYP21A2 point mutations (P30L, IVS2, I172N, V281L, Q318X and R356W) in 25 Macedonian patients with clinical and laboratory signs of nonclassical 21-hydroxylase deficiency evaluated at Department of Endocrinology and Genetics, University Children’s Clinic, Skopje, Republic of Macedonia.

Results: Four different mutations were detected in 24/50 (48%) alleles. The most prevalent mutation was P30L, present in 15 alleles (30%), followed by the Q318X in 6 (12%), V281L in 2 (4%) and IVS2 in 1 allele (2%). In 24% (6/25) of the patients complete genotype was revealed with good correlation with phenotype. Of them, 5 were homozygous with P30L/P30L genotype and 1 was compound heterozygous with P30L/Q318X genotype. Twelve (48%) patients were heterozygotes for one mutation: 5 for Q318X, 4 for P30L, 2 for V281L, and 1 for IVS2 mutation. 7/25 (28%) patients harboured none of the tested mutations.

Conclusion: Our finding support a role of the P30L mutation in nonclassic 21-hydroxylase deficiency. We observed a high frequency of the severe nonsense Q318X mutation in the heterozygous state. Mild mutation present on the second allele might be explanation for the mild clinical manifestation in thеse patients.

Keywords: nonclassical 21-hydroxylase deficiency; CYP21A2 gene; point mutations


Incidence of congenital hypothyroidism in the Macedonian nurseries obtain by the neonatal thyroid screening, 2002-2014

Violeta Anastasovska, Milica Pesevska, Elizabeta Taseva and Mirjana Kocova

Genetic Laboratory, Department of Endocrinology and Genetics, University Children’s Clinic, Skopje, Republic of Macedonia

Background: Neonatal thyroid screening program allow early effective diagnosis and treatment of congenital hypothyroidism (CH), the most common preventable cause for mental retardation in children. The incidence of CH among neonates in the nurseries in the Republic of Macedonia has not been separately evaluated before.

Method: Newborns of all 31 nurseries in the country (227,661) have been screened for neonatal thyroid-stimulating hormone (TSH) from blood spots on filter paper (Whatman 903), obtained 48 hours after birth, using the fluoroimmunometric DELFIA assay, during the period 2002-2014.

Results: Ninety-six newborns with congenital hypothyroidism were detected in the 17/31 (54.8%) of the nurseries, with following distribution: 23 CH in the University Clinic for Gynaecology and Obstetrics – Skopje with incidence of 1:2462, 12 in Special Hospital for Gynaecology and Obstetrics ,,Mother Teresa’’- Skopje (1:3277), 11 in General Hospital Prilep (1:1134), 11 in General Hospital Tetovo (1:1667), 7 Kumanovo (1:1767), 7 Kavadarci (1:598), 5 Gostivar (1:1501), 4 CH in Strumica (1:2500) and 3 in Bitola (1:5096). Two CH were obtained in General Hospital Štip, Struga, Veles, Remedika - Skopje and Acibadem Sistina Hospital- Skopje, each. Only one newborn with CH was obtained in the each of the General Hospital Gevgelija, Ohrid and Kriva Palanka. In the rest 14 nurseries, newborns with CH were not detected.

Conclusion: The incidence of CH varied wide in the most of the nurseries compared to the incidence reported for the whole country (1:2486). Assessment of the iodine status in the regions with high incidence of CH, especially in the Kavadarci region, is necessary.

Keywords: congenital hypothyroidism; neonatal thyroid screening program


Treatment for traumatic brain injury in children

Andonovska B, Kuzmanovska B, Kartalov A, Spirovska T, Andonovski A, Trajkovska Dj.V

Clinic for Traumatology, Orthopedics, Anesthesia, Resuscitation, Intensive Care and Emergency Center

ABSTRACT

Traumatic brain injury (TBI) is the leading cause of death in children and young adults. They are usually caused by falls from height and motor-vehicle–related accidents. Head trauma may be responsible for primary and secondary brain damage. The management of severe TBI includes prevention and treatment of intracranial hypertension and secondary brain insults, preservation of cerebral perfusion pressure (CPP), and optimization of cerebral oxygenation. Evidence-based guidelinesand management protocols are prepared to help the treatment of these patients with TBI and obtain a better outcome.

In this review, the critical care management of severe TBI will be discussed with focus on avoidance and minimization of secondary brain insults, and optimization of cerebral oxygenation and CPP.

Keywords: Review. Trauma. Brain injury. Children


Timed and functional testing as outcome measure in Duchenne muscular dystrophy patients

N. Angelkova*, V Sabolic*, F. Duma*, T. Useini**, E. Kocova*, A. Stamatova** *University Children’s Hospital Skopje, **Clinical Hospital Bitola

Abstract Progressive muscular dystrophy (Duchenne) is а devastating X linked disease of the body muscles with clinical appearance of progressive loss of ambulation and wheelchair bonding during early adolescence. According to standards of care, steroid treatment is followed by continuous physiotherapy, use of orthotic and orthopedic equipment. To accomplish the follow up guidelines and measure the treatment efficacy, we performed functional tests at the time of diagnosis, before the steroid treatment and yearly during follow up. Goal To introduce the functional testing at newly diagnosed patients with Duchenne muscular dystrophy using standardized 6 minutes walking test and North Star Ambulatory Assessment. To assess and correlate the type of the mutational defect in dystrophin gene with creatine kinase blood level and functional test score. Method We report the results of clinical examination, CK blood level, dystrophin gene MLPA analysis, 6 minutes walking test and North Star Ambulatory Assessment in six newly diagnosed patients with Duchenne muscular dystrophy. The boys were steroid naïve at the moment of diagnosis and received treatment after molecular confirmation of the disease. Results 10m walking test lasted 6-10 sec. Timed Gowers sign was done in 9-11 seconds. 6 minutes walking test completed 295 to 375 meter distance. NSAA score is 18/34 to 28/34. There was positive correlation between timed testing and functional testing in all participants. Conclusion The functional and timed testing is noninvasive, clinically relevant and accepted form of outcome measure in Duchenne muscular dystrophy patients. Initially obtained scores in patients from Macedonia are as expected and reported in the literature. Key words: outcome measures, Duchenne muscular dystrophy


Тумор во десна комора приказ на случај

Митрев Ж., Анѓушева Т, Милев И, Христов Н, Стоицоски Е, Алексовски Е

Специјална Болница по хируршки болести Филип Втори, Скопје, Македонија

Миксомот е тумор кој во 75% од случаевите се јавува во лева преткомора и најчесто во адултна

возраст. Само 12 случаи во светот се опишани на присуство на овој тумор во десна комора и

тогач најчесто е во склоп на синдромот Карнеј.

13 годишно момче примено за хируршки третман на тумор во десната комора со димензии

3,5х2,5цм. Детето , 40 дена со фебрилност до 40,0 оЦ, замор, потење, болки во зглобовите и

губиток на телесна тежина. Физикалниот наод позитивен за левострана пнеумонија,

дијастолен шум над пулмоналната артерија, голем А-бран на југуларната

вена.Ехокардиографски е верифицирана голема хетерогена маса, суспектна за миксом

фиксирана за мембранозниот дел на ИВС, која продира кон десната преткомора, и флотира на

десниот РВОТ. Туморот ебеше со сесилна околна маза од налепени тромби ЦТ скенот покажа

миксом во десна комора, со пулмонални микроемболи ( потврдено со позитивни Д-димери).

Туморот комплетно беше отстранет, при што се отстрани и неговата база и се направи дирекна

сутура на мемебранозниот дел од ИВС.

Патохистолошкиот наод потврди дека ткивото е миксом, со околни сесилни тромби.

Поради настанување на АВ блок од втор степен (2/1) кај детето е поставен траен двокоморен

пејс мејкер со атријален сензинг и вентрикуларен пејсинг.

Во стабилна состојба детето е отпуштено дома.


Improvement of the health care for children with haemophilia in Republic of Macedonia

Zorica Trajkova Antevska, Sofijanka Glamocanin, Kata Martinova, Biljana Coneska, Svetlana Kocheva,Aleksandra Jovanovska

Introduction: HEMOPHILLIA is X-linked recessive bleeding disorder, caused by wide range of mutations in the factor VIII (F8) or IX (F9) gene. It occurs in about 1:5000 or 1:30 000 male births respectively.

Aim:The objective was to present the development of health care for children with hemophilia in Republic of Macedonia(RM)

Methods: University Children’s Clinic in Skopje is single institution where all children (0-18 years) with bleeding disorders in RM are diagnosed and controlled. Laboratory diagnosis was established and still is performed on Institute for Transfusion Medicine. There were prepared plasma and cryoprecipitate from own voluntary donations .It was only treatment for children with hemophilia until 2004.Factor concentrates(pd) were introduced in2004 in limited amounts. Prophylaxis for children with severe HA was introduced in 2007. Factor concentrate consumption rose from 1 IU to 3 IU per capita. Treatment with recombinant FVIII started 2010 in clinical study setting.

Results: Since 1965, 289 pts with hemophilia were diagnosed (193 MK, 96 from surrounding countries, HA 197 (133 MK) pts and HB 92 (60 MK) pts. One HA pt and one HB pt developed inhibitors and they were treated with rFVIIa. Prophylactic / home /self treatment was applied to 12 HA and 2 HB children. Short term secondary prophylaxis was performed for 10 children (8 HA and 2 HB), tonsillectomy for 3 , appendectomy for 1, synovectomy for1.

Conclusion: Diagnosis and treatment for children with hemophilia in RM dated from 1965 and great improvement in of the health care was achieved in the last 10 years. HTC Skopje was established and University Children’s Clinic –haematology department became functional part of it. HTC Skopje was recognized as Comprehensive HTC 2014.


How parents’ attitude can affect children’s rehydratation plan

Aurela Saliaj MD,PhD, Majlinda Zahaj MD,PhD, Alketa Hoxha MD,PhD, Sonila Nikaj RN,MSc, Prena Halilaj RN,MSc

Faculty of Public Health, University ‘Ismail Qemali’ Vlorë, Albania

Introduction: Oral rehydration (ORT) is the treatment of choice in the mild and moderated dehydration. A program launched by WHO, promoting the use of oral rehydration solutions to outpatient, reduced mortality rates from diarrhea by 74% from 1980 to 2008, worldwide. Yet despite recommendations, oral rehydration is not widely used in the clinic. 75% of health clinics all over the world have intravenously rehydration first line treatment of gastroenteritis. One of the factors thought to affect this practice is the attitude of the parents and the pressure exerted by them on the physicians.

Objective: This study aims to assess how the attitude and knowledge of parents affect their children’s rehydration plan.

Material and Methods: The study was conducted at the Regional Hospital of Vlora, in the beginning of 2015. The sample included 97 parents who had children admitted to the pediatric ward with clinical diagnosis: acute gastroenteritis. Parents were questioned on their information and experience about rehydration of children.

Results: During the study period in the pediatric ward, 63% of children, hospitalized with gastroenteritis, were subjected to parenteral rehydration, while 26% to oral rehydration. 47% of parents stated that they expected their child to get IV fluids, while 42% expected oral rehydration. Regarding the parents’ attitude toward the doctors, 34% of parents said they have put pressure on the doctor to decide on giving IV fluids to their child. Parents, who have given ORT or tea to their children prior to the hospitalization, mostly live in urban areas than in the rural ones (7 times more).

Questioned to whom they wanted to speak about gaining information on dehydration, most parents responded that they relayed more on health professionals. 73% of them would like to speak with a physician, 22% with a nurse and 3% with their mothers, as more reliable.

Conclusions: There is a statistically significant relationship between the child’s current rehydration plan and parents’ expectations for oral/IV dehydration’s treatment (Pearson correlation index = 0.3808). We can also say that the expectation of parents is connected with the pressure they put on physicians (Pearson correlation index = 0.4336). While the pressure of parents do not seem to have any strong connection with the physician’s decision about rehydration plan (Pearson correlation index = 0.2300). The study showed that there is a very strong relationship (Pearson correlation index = 0.5191) between the parent’s information on oral rehydration and the management of children’s dehydration at home, before sending them to the hospital.

Key words: dehydration, oral and intravenous rehydration, parental attitude.


Split Hand/Foot malformation associated with 7q21-q22.2 deletion: A Case Report

Anila Babameto-Laku, Dorina Roko, Donjeta Bali

Service of Medical Genetics, University Hospital Center ”Mother Teresa”, Tirana, Albania

Split hand/split foot malformation is a rare congenital deformity involving limb development. Also known as ectrodactyly is characterized by absence of digits, fusion of remaining digits, and a deep median cleft in the hands and feet. It is genetically heterogeneous involving several loci including 7q21-q22.1, Xq26, 10q24-q25, 2q31 and 3q27. Chromosomal rearrangements involving7q21-q22 is most commonly associated with isolated or syndromic ectrodactyly. We report a case of syndromic ectrodactyly , in a 4 months old male with bilateral limbs ectrodactyly, growth retardation and developmental delay. Chromosome analysis using high resolution banding technique showed an interstitial deletion of the band 7q21.3. Array CGH resolved the break to have occurred within the band 7q21.3. The deletion was de novo because the parental karyotypes were normal. Syndromic ectrodactyly is expressed as an autosomal dominant trait with reduced penetrance and variable expression. Several studies have pointed out the probable role of three genes present in this region – DLX5, DLX6 and DSS1 - in limb development. We present a case in which use of array CGH has provided an insight of genetic abnormality. Molecular characterization of more patients will ultimately lead to a further delineation of the critical regions and thus to the identification of candidate genes for these specific traits.


Does the monitoring exhaled nitric oxide (FeNO) levels can improve the management of childhood asthma ?

Prof. dr. Gordana Badzakova Macheska,Clinical Hospital “Acibadem Sistina”, Laboratory of lung function and allergy in children, Skopje, Macedonia

Introduction: asthma is chronic inflammatory disorder of the airways associated with airway hyperresponsiveness and recurrent episodes of reversible airway limitation that are accompanies by wheeze, shortness of breath chest, tightness and cough. Asthma is a disease of the entire respiratory tract that inflammation, obstruction and remodeling occurs the whole respiratory tract, including the small airways (peripheral membranous bronchioles < 2 mm diameter). The main focus of the asthma management in children are the achievement of daily control and prevention of long-term consequences of inadequate disease control. Fractional exhaled nitric oxide (FeNO), surrogate marker of eosinophilic inflammation, non-invasive method of measuring, give us the levels of eosinophilic inflammation in the lower airways and marker of airway smooth muscle tone. Aim of the study: we detected the variation of the FeNO levels and asthma control in children. Materials and Method: we evaluated 116 asthmatic patients with allergic asthma, for index period of 12 months, aged 7-18 years (mean age 12.5 years), the beginning of symptoms, first wheezing < 5 years. The atopic status with skin prick test, show us polysensitisation (Der.p + pollens-grass and / or tree). The daily doses of ICS are mainly doses of 200 – 400 mcg Fluticason (+ LABA or LTRs). Results: the clinical events are: 50 children (43%) with controlled asthma and 66 patients (57%) with partially / uncontrolled asthma (GINA quidelines, 2009). FeNO values in controlled asthmatic patients were 6-40 ppb (mean values 15.5 ppb) versus partially / uncontrolled asthma, FeNO were 20-120 ppb (mean 58 ppb).

Conclusion: FeNO levels below 25 ppb (cut-off) indicator for good asthma control.


Review of immunization for 2014 on the territory of the city of Skopje Baevska Vuckovic T, PHI Health Center Skopje- Skopje Introduction: According to the statistical data in the Republic of Macedonia, on annual level, 150.000 children are subject to regular immunization according to a calendar, 55.000 of which were born on the territory of the city of Skopje and the surrounding settlements. The annual plan and realization regarding proimmunization is for about 8000 newborns. Objective: Immunization review for 2014 on the territory of the city of Skopje according to the current immunization calendar. Material and method: In 2014, at the level of the Health Center – Skopje, the immunization was realized by 39 prevention teams (doctor, nurse), of which 16 teams in pre-school age. Results: planned/realized: BBCCGG п-8675 р-7642(98,19%); DDTTPP--11 пп--7750 р-7635(97,17%); DDTTPP--22 пп--7750 р-7458(96,23%); DDTTPP--33 пп--7750 р-7198(92,87%); PPOOLLIIOO--11 пп--7890 р-7751(98,23%); PPOOLLIIOO--22 пп--7750 р-7556(97,49%); PPOOLLIIOO--33 пп--7750 р-7237(93,38%); PPOOLLIIOO RRvv11 пп--7564 р-6944(91,80%); HHeeppBB--11 пп--8675 р-8395(96,77%); HHeeppBB--22 пп--8520 р-8092(94,97%); HHeeppBB--33 пп--7750 р-7374(95,14%); MMPPRR пп--7564 р-6708(88,68%); MMRRPP RRvv11 пп--7092 р-6685(94,26%); DDTTPP RRvv11 пп--7841 р-6863(87,52%); DDTTPP RRvv--22 пп--7088 р-6382(90,03%); HHiibb--11 пп--8250 р-7850(95,15 %); HHiibb--22 пп--7750 р-7352(94,86%); HHiibb--33 пп--7740 р-6786(87,67%); HHiibbRRvvrr п-6855 р-5454(79,56%); dT Rv-3 п-6643 р-5933(89,31%); dT Rv-4 п-6931 р-6022(86,88%); TT Rv-5 п-5438 р-877(16,12%); POLIO Rv2 п-6643 р-6414(96,55%); POLIO Rv3 п-6631 р 5655(85,28%); HPV-1 п-3050 р-635(20,81%); HPV-2 п-3180 р-957(30,09%); HPV-3 п-3180 р-705(22,16%); Conclusion: Immunization in 2014 is at a solid and satisfactory level with high 94%, with partial mild decline of MMRRPP RRvv ssccooppee,, which is mainly due to the anti-vaccination campaign related to the autism occurrence in the period after taking this vaccine, although it is not scientifically proved that there is a direct connection thereof, and although they were regularly invited to come to the immunization posts. HPV vaccine has a constant low scope, but there is better scope in the rural areas. Strategy – strengthening human resources. Education for introduction of new polyvalent vaccines. Public debates and discussions with arguments for the vaccine versus anti-vaccine movements.


Prokalcitonin impact method in detecting pediatric infections Sani Bajrami1*,Zlata Trimcevska1 ,Antigona Trenevska1,Flori Besimi1,Nagip Rufati1,Drita Tefiku1,Savica Markovic1

1Clinical Hospital Tetovo (Department of Neonatology, Department of Child), R of Macedonia 1Faculty of Medical Sciences Tetovo, R of Macedonia ABSTRACT Introduction Differentiating bacterial from viral infections and their early detection is important in practice pediatric. Procalcitonines level increase as a result of inflammation from bacterial origin, but no spondents from viral infections. PURPOSE: The purpose of this work is: To show the role of PCTand PCR early detection of bacterial respiratory tract infections. Report between Procalcitonin and CRP. The determination of the Sensitivity,Specificity,PPV,NPV of the Prokalçitonin (VIDAS quantitative method-B-R-A-H-M-S-PCT),CRP,Neutrophils.

Мaterial and methods:The study was prospective, randomized, performed in Clinical Hospital Tetovo(Department of neonatology, pediatrics,biochemical clinical laboratory), in the period November 2011-December 2012,was included 99 children . Males 56 or 56.56%, female 43 or 43.43%. The average age 2.47 years ± 3.5 months, average weight 12.05 ± 2.8 kg, the average length of 81.42 ± 12.4 cm, days average length of stay was 4.1 ± 1.3 days, , average use of antibiotics was 0.8 ± 5.25 days. To confirm the diagnosis is calculated PCT-cutt-off> 0.25 ng / ml, CRP-cutt-off> 40mg /L, morphological blood smear,x-ray. 1. study group includes 76 children - In the upper respiratory tract infections (tonsilopharingite): Of 35 the PCT growth projects have cases bacterial infection (28,57%). - With the lower respiratory tract infections (bronchopneumoni) by 41 pct growth projects in the cases bacterial infectin in (39,02% ), - In 10,52 % to cases PCT,CRP,Le,Ne was conducted twice, it was observed that 72 hours after receiving the therapy..Have a decrease 37,12% leukocytes in the value of the second in comparison with the first value. A decrease from 91% to procalcitonines, which reinforces the reliability of procalcitonin as early marker of respiratory infections.Well as a decrease of 67,55 % of the CRP

2. In the control group 23 cases, was healthy childrenIn 4 cases had slight growth of PCT and in three cases in the newborn, DISCUSSION AND CONCLUSION Based on the results seen in both diagnosis Tonsilopharingitis acuta respectively Bronchopneumoniae, PCT: 95 % sensitivity 80,36% specificity 75.5% PPV 97.82% NPV CRP: 74.41% sensitivity 78 % specificity 63.39% PPV 84.15% NPV, Neutrofils: 90.57% sensitivity 82.11% specificity 77.92% PPV 92.35% NPV. Results certify PCT high accuracy in comparison with CRP and Neutrophils in pediatric respiratory infections. Correlation between PCT and CRP its more pronounced in Bronchopneumoniae.

Although this method has difficulties because of the high cost are major benefits: • PCT may serve to reduce hospitalizations • Rational use of antibiotics.

Key words :Procalcitonin, bacterial infection.


Guidelines for the diagnosis and management of syncope in children and young teenagers

Ramush A. Bejiqi1, David M. Bush, R. Retkoceri1, N. Zeka1, A. Gerguri1, A. Vuçiterna1, H. Bejiqi3, A. Beha3, Sh. Borovci1, A. Batalli1, 1Pediatric Clinic, University Clinical Centre of Kosovo, Prishtina, Republic of Kosovo 2 University of Texas, San Antonio, Texas, USA 3 Mean Centre of Family Medicine, Prishtina, Republic of Kosovo

Introduction Syncope refers to a sudden partial or complete loss of consciousness often accompanied with collapse as a cause of a lack of blood or oxygen to the brain. When this happens, the brain attempts to increase blood flow to itself by diverting blood away from the rest of the body. This causes pale skin and a white face, rapid heart rate, rapid breathing and weakness of the limbs, particularly the legs. Eventually collapse occurs. There are a number of situations that can induce syncope as a little food or water, low blood sugar, intense physical exercise, and standing up too quickly can all cause an episode of fainting. Additionally, low blood pressure and an abnormal heart rhythm can also cause a lack of blood to the brain and induce syncope.Syncope can occur in anyone and recurrent episodes of syncope may be a sign of a more serious underlying condition and require further investigation. Aim of presentation is to present most common causes of syncope in children and teenagers and basic examination needed for diagnosis and eventual prevent or treated possible underlying cause of syncope trying to reduce the number of syncope episodes and possible injuries. PresentationThe word syncope derives from a Greek term meaning "to cut short" and may have been first described by Hippocrates.Syncope accounts for approximately 3% of emergency department visits and between 1 and 6% of acute hospital medical admissions, affecting 6 per 1000 people per year.Clinical assessment of syncope is challenging, owing to the heterogeneous nature of underlying causes, ranging from benign neuro-cardiogenic syncope to potentially fatal dysrhythmias and pulmonary embolism Syncope is a commonly encountered problem in the emergency department. Simple syncope episodes can be diagnosed fairly easily by a doctor without complicated tests. However, recurrent episodes may be a symptom of another condition and a specialist doctor may order some tests to investigate what could be the cause of syncope. Very often a specialist consultation with a 12 lead Electrocardiogram will point to a diagnosis, but other tests may be needed such as an ambulatory 24 hour electrocardiogram, electroencephalogram (EEG), computerised tomography scan (CT) or magnetic resonance imaging (MRI), Carotid Doppler ultrasound scan, blood pressure monitoring, and echocardiogram and a tilt table test are used to investigate recurrent episodes of syncope. Ultimately if no diagnosis can be made a small ECG recorder can be inserted under the skin (Implantable loop recorder Reveal Medtronic TM) so that ECGs can be recorded continuously for up to 3 years. Generally, syncope is treated at the time of fainting by lying down and raising the legs in order to improve blood flow to the brain. Treatment for an underlying cause may be necessary in order to stabilise and reduce the number of syncope episodes. Key words Syncope,arrhythmia, sudden death, diagnostic guidelines, emergency department


Inflamatory bowel disease in children after therapy with infliximab, series of cases

Bojadzieva S., Kostovski A, Nikcevska N, Grujovska S., Todorovski G.

University Children’s Hospital Skopje, Department of Gastroenterohaepatology

Background

Inflammatory bowel disease (IBD) is a group of inflammatory conditions of the colon and small intestine. It is an idiopathic disease caused by a dysregulated immune response to host intestinal microflora. The two major types of inflammatory bowel disease are ulcerative colitis (UC) limited to the colon, and Crohn disease (CD).

Presentation of cases

Five patients were treated with Infliximab. First one is male 15years, with CD, and numerous life threatening GIT bleedings. Colonoscopy showed ileocolonic form of CD. We started “top down“ therapy due to his condition. After the first dose, great improvement is noted. Second patient is 22 years female, CD, with fistula. After Infliximab, the fistula closed. Third patient is 8 years girl with ileocolonic form of CD. No improvement was seen with regular” top up therapy”. Wih Inliximab, there is an evident improvement. The fourth case is 14 years, girl, with UC, unresponsive to therapy. Great improvement is seen in the overall condition. The fifth case is 8 years girl, with diagnosis of Total colonic aganglionosis, and frequent episodes of endogenous sepsis and uncontrollable enterocolitis. Unfortunately, no great improvement is seen.

Conclusion

Infliximab is a good alternative treatment in patients with inflammatory bowel disease, especially in those that no improvement is seen with the standard “top up “ therapy.

Key words : Inflammatory Bowel Disease, Infliximab

https://en.wikipedia.org/wiki/Inflammation

https://en.wikipedia.org/wiki/Colon_(anatomy)

https://en.wikipedia.org/wiki/Small_intestine

https://en.wikipedia.org/wiki/Small_intestine


Recent Findings in the Diagnosis and Treatment of Inflammatory Bowel Disease in the Childhood Age

Sonja Bojadzieva

University Children’s Hospital, Skopje. R.Macedonia

Gastroenterohepatology Department

The Inflammatory bowel disease (IBD), which includes ulcerative colitis (UC) and Crohn's disease (CD) is an idiopathic chronic disease of the gastrointestinal tract. The Ulcerative colitis is a nonspecific chronic inflammation of the mucosa of the colon, while the Crohn's disease can affect the entire gastrointestinal tract. In our study we have examined 45 children with IBD (24 children had been diagnosed with the Crohn’s disease and 21 with ulcerative colitis). The diagnosis has been confirmed after the realisation of all the diagnostic protocols provided by the European Society for Pediatric Gastroenterohepatology and Nutrition (ESPGHAN). The immunological investigations include the investigation of the immunoelectrophoresis, proinflammatory cytokines, ASCA (IgA, IgG) and the p-ANCA (MPO, PR3). The therapeutic aspect of IBD includes the corticosteroid, the antibiotic, amynosalicilate, immunomodulators, the biological therapy and the surgical therapy.The IL-1, IL-6 and TNF-α values and ASCA (Anti-Saccharomyces cereviseae antibodies) IgA and IgM were increased in the patients with CD. Five patients were treated with the biological therapy with Infliximab and after the administration of the same there was an evident improvement. The overall, phenotypic, serological, immunological and genetic aspects are the key parameters and have an important role in the diagnosis and therapy in children with IBD. The effectiveness of the biological therapy with Anti-TNF -α agent (Infliximab), have been proven in children with heavy active luminal forms of CD and CD fistulas achieving clinical remision. The biological therapy is approved for the treatment of the fistulizing forms of the CD and the UC, and in the cases where the effect is not achieved with another form of therapy.

Key words: Inflammatory Bowel Disease, Crohn’s Disease, Ulcerative Colitis.


Display of cases in one family with Tyrozinemia type 1

Bojadzieva S., Kostovski A., Nikcevska N., Grujovska S., Todorovski G

University Childrens Clinic Skopje, Department of Gastroenterohaepatology

INTRODUCTION Tyrosinaemia type 1 is an autosomal recessive disease, causing enzyme FAH (15q23-q25) deficiency. Patients have a characteristic smell of cabbage, renal tubular dysfunction, amino aciduria, progressive hepatomegaly to cirrhosis, failure to grow, elevated alpha- fetal protein, and coagulation disorders. There is an increased risk of hepatocellular carcinoma.

CASE PRESENTATIONS

We present two cases of tyrosinemia type 1 in the same family. The first alive child, female, is 7 years , transplanted in 2011. Diagnosed by Succinylaceton in urine. Another girl diagnosed with tyrosinemia, and a boy are departed. The girl is currently relatively stabile.

The second alive child is boy, 8 month. Born 3600gr ,51 cm. Admitted for evaluation, for coagulation abnormalities, infection, and anemia. Physical examination showed hepatomegaly 4 cm, signs of rickets, pale skin. Analysis Hgb 88 , elevated liver enzymes (AST 938), elevated amino acid tyrosine (464micromol / L) in plasma, and of all amino acids in urine. Alfa-feto protein extremely elevated (>60500,0). Ultrasound showed hepatomegaly, clearly demarked formation (20x 26 mm). CT of abdomen revealed hepatomegaly, right lobe discreetly hyper dense round formation (23x20 mm), left kidney 72 mm, 71.8mm. Coagulation :PT 20 sec, aPTT 66 sec, TT 24sec. He is currently in the process of finding a suitable liver donor within the family. DISCUSION The incidence is 1 100 000. Mortality is very high. Nitosinone, together with low protein nutrition and milk free of tyrosine is the first line therapy. We present a family where 4 children out of 6 had tyrosinemia. Key words Tyrosinemia tip 1, hepatomegaly


Macedonian Strategy for Containment of Antimicrobial Resistance

Bosevska Golubinka1, Kakaraskoska Boceska Biljana1, Cekovska Zaklina2, Kaphtandzieva Ana2, Hristova Kristina4, Milevska Kostova Neda7, Kostovska Jovanka3, Manevska Suzana3, Velev Romel6, Spasenovska Margarita8, Chichevalieva Snezhana8,Kuzmanovska Gordana1, Coneva Elizabeta1, Sofijanova Aspazija5,Jankulovski Nikola2, Panovski Nikola2

1Institut for Public Health, 2Medical faculty, University "Saint Cyril and Methodius",Skopje, 3Ministry of Health, 4Insurance fund of RM, 5University clinic for child diseases, 6Veterinary faculty, 7Center for Regional Policy Research and Cooperation – Studiorum, 8WHO office in Skopje

The aim is to present Macedonian activities for rational use of antibiotics in order to decrease the development of antimicrobial resistance.

Antimicrobial resistance (AMR) is a real threat in R. Macedonia. Resistance of invasive strains in Macedonia is higher than in EU countries, but similar to South and South-East Europe. Following WHO and ECDC recommendations, Multisectorial committee for surveilllance of AMR is established in May 2009, and Macedona was one of the first countries, after Croatia and Bulgaria, with developed National Strategy with Action plan for control of AMR 2012-2106. Specific activities are: 1.Information and education of general public and health professionals on prudent use of antibiotics with organized AMR campaigns (2008 -2014), hand hygiene campaigns (2013-2014), organization of workshops and educational seminars such as on-line workshop ˮAntimicrobial Stewardship: Optimization of Antibiotic Practicesˮ developed by University of Stanford, USA 2. Straightening the system for surveillance and control of AMR with implementation of EUCAST system in microbiological labs, reporting AMR in the country and to the WHO according CAESAR methodology; 3. Straightening the system for surveillance and control of antimicrobial consumption with close collaboration with Insurance fund, hospital settings and My time project of the Ministry of Health. 4. Control and preventive measures for communicable diseases such as immunization programs, surveillance of antibiotic use in animals and agriculture etc.

Conclusion: Coordinated response is necessary to keep antibiotics working. ˮEveryone is responsible, everywhere”

Key words: antimicrobial resistance, strategy


Early detection and diagnostic assessment for children in risk of autistic spectrum disorder

Meri Boshovska, Lidija Spasova, Marina Pop-Lazarova, Marija Stojkoska Vasilevska

First General Hospital Re-Medika, Skopje

Introduction

Autistic spectrum disorder is a complex neurodevelopmental disorder, whose basic characteristis are presence of abnormal and … development that can be noticed before age of 3. Also there is specific way of pathological functioning in the social interaction and communication with the environment as well presence of stereotypical behavior. Pediatricians has the opportunity to be the first health workers in the process of diagnosis and clear orientation with whom parents get in contact because they are concerned about the development and the behavior of their child.

Objective

We point out the importance of early detection for children suspected of having autistic spectrum disorder, and their guidance towards referral health institutions for clinical assessment and diagnosis (by child psychiatrists) for eventual confirmation of the diagnosis.

Method

About early detection of children at risk of having autistic spectrum disorder at the age between 18 and 24 months, there is an instrument called CHAT – Cheklist For Autism In Toddlers, designed for pediatricians. If the pediatrician has the child who is suspected of having autistic spectrum disorder, he/she should be addressed to a child psychiatrist for assessment and diagnostic. Whole professional team is the part of the diagnostical procedure that include developmental and family history, focused observation of the child, cognitive and communicational assessment, physical examination and analyses, evaluation of the mental health and behavior as well the needs and strength the capacity of the family. The diagnostic of autism is directed to confirm the clinical picture of diagnostic criteria by ICD -10 and DSM IV. The following diagnostic instruments are in use: CARS- Childhood Autism Rating Scale II, ADI-R Autism Diagnostic Interview, WISC, Bayley Scales of infant and toddler Development .

Conclusion

The early diagnosis initiates preparation of Program for early intervention which goal is to overtake the maximal developmental potential of the child, that will influence his/hers adaptable functioning in perspective which will determinate the quality of life of the child and his whole family system. The diagnostical and the therapeutic procedures should be based on evidences and good clinical practice.


Значењето на quantiferon tb gold test за дијагноза на латентна туберкулозна

инфекција(лтби) во детската возраст - прикази на случај

Катерина Бошковска, Л. Петрушевска Колеќевска, М.Дилберовска, Д.Дацевски

Институт по белодробни заболувања кај децата-Козле, Скопје,Р.Македонија

Абстракт

Туберкулозата според светска здравствена организација е дефинирана како

инфективна бактериска болест предизвикана од Mycobacterium tuberculosis. Главен извор на

инфекција се болни од белодробна туберкулоза од чиј спутум се изолирани бацилите на

M.tuberculosis. Латентна туберкулозна инфекција претставува инфекција со бацилите на M.

tuberculosis, без знаци на болест, без рентгенолошки промени и без бактериолошка потврда за

туберкулоза. Заедничко за туберкулозата и ЛТБИ е позитивен туберкулински тест и Interferon-

Gamma Release Assays тестови. Туберкулинскиот кожен тест според методот на Манту не е

сигурен тест за откривање на латентната туберкулозна инфекција особено во земји каде

постои BCG вакцинација. Quantiferon TB gold тестот се базира на ослободување на IFN-γ од

страна на Т клетките стимулирани со специфични антигени добиени од Mycobacterium

tuberculosis. Јасно е документирано дека BCG вакцинацијата не влијае на вредностите на

ослободениот IFN-γ. Обработени се два случаи на деца со позитивен интрафамилијарен

контакт со болен од активна туберкулоза. Кај двете деца се реализирани in vivo и in vitro

тестови. Со реализација на Quantiferon TB gold тестот е исклучена латентната туберкулозна

инфекција, и е прекината медикаментозната превенција. Oдредувањето на IFN-γ придонесува

за подобра дијагноза на латентната туберкулозна инфекција со што се намалува непотребната

медикаментозна превенција.

Клучни зборови: деца, латентна туберкулозна инфекција, туберкулоза, туберкулински кожен

тест, Quantiferon TB gold


Промените на колкот кај пациенти со спастична форма на церебрална парализа

Божиновски З., Георгиева Д., Џолева Толевска Р., Кузевска Манева К* УК ТОАРИЛУЦ Клиника за ортопедски болести-Скопје, Р Македонија *Универзитетска Клиника за детски болести – Скопје, Р Македонија Цел

Да се утврдат промените на колкот кај пациенти со спастична форма на церебрална парализа и нивното влијание на дислокацијата на колкот.

Вовед

Дислокацијата на колкот кај пациенти со церебрална парализа настанува како резултат на повеке фактори, вклучувајки го асиметричниот мускулен спазам, валгус и ротациониот деформитет на проксималниот фемур и контрактурите на колкот.

Материјал и методи

45 пациенти со церебрална парализа со 50 дислоцирани колка. 32 пациенти беа без способност за одење, а 13 беа способни за самостојно одење или одење со помош на помагало или друго лице. 40 пациенти беа со унилатерална дислокација, а 5 со билатерална дислокација на колкот. Просечната возраст беше 6,4 години. Миграциониот процент на Реимерс беше користен за евалуација на миграцијата на колкот надвор од ацетабулумот.

Резултати

Кај оние пациенти со унилатерална дислокација 28 имаа денивелација на карлицата и сколиоза. Валгус деформитет на проксималниот фемур и антеверзија беше присатна кај сите пациенти. Обемот на движења на колкот беше мерен кај сите пациенти при што е најдена корелација со дислокацијата на колкот. Позитивна корелација е најдена меѓу миграциониот процент и дислокацијата на колкот. Денивелацијата на карлицата не корелира со дислокацијата на колкот. Таа е резултат на мускулниот дисбаланс и дислокацијата на колкот.

Заклучок

Базирани на овие резултати препорачуваме периодични рендгенграфии на карлица со употреба на миграциониот процент на Реимерс за спречување на дислокација на колкот и одредување на времето за оперативна интервенција.


Surgical Treatment on the Lower Extremities in Patients with Spastic Cerebral Palsy

Bozinovski Z, GeorgievaD, Dzoleva Tolevska R. Samardziski M. Kuzevka Maneva K * UK TOARILUC Clinic for orthopedic diseases in Skopje, Republic of Macedonia * University Clinic for Children's Diseases, Republic of Macedonia Purpose To analyze the most common surgery in the lower extremities in patients with cerebral palsy, recurring operating procedures and timing of surgery.

Patients and Methods Included 260 patients with cerebral palsy aged 3 to 16 who performed the surgery of the lower limbs in the period from 2008 to 2014. In 86 patients the surgery was repeated at different times of growth. Analyzed surgeries of the hip, knees and feet. Patients were divided into two groups capable of walking and walking with assistance, and patients who do not have ability to walk. Surgery were divided into soft tissue operations and the bone tissue operations.

Results

We analyzed 780 surgeries. Soft tissue surgical procedures are more prevalent in the hips, knees and feet. They are repeated more than bone operations. No difference in age compared to surgery of the hips, knees and feet. Bony operations are in the age group over 10 years. Surgery of the hips and feet are more common than knees operations.Most frequent operative procedures were adductor tenotomy of the hips and aponeurectomy of the gastrocnemius muscles . In terms of bone surgery varusderotational osteotomy of the proximal femur were most frequent.

Conclusion Based on the analysis of the operative procedures it can be concluded that the hips and feet are usually operated in both groups of patients.


Демографски карактеристики на децата со аутистичен спектар на Нарушувања третирани со хипербарна оксигено терапија во периодот 2009-2015

Брешковска Х. Туџарова Ѓоргова С. Џокиќ Ѓ. Етеми Ј. Нацевска Ѓорѓеска А. ЈЗУ Клиника за пластична и реконструктивна хирургија,Медицински факултет, Скопје Аутистичниот спектар на нарушувања претставува сериозно невроразвојно нарушување кое ја намалува способноста на децата да комуницираат и дејствуваат заедно со другите луѓе. Ова предизвикува значајно влошување во социјалните, работните и другите сфери на нивното дејствување. Аутистичниот спектар на нарушувања е дефиниран од Американската психијатриска асоцијација и Статистичкиот прирачник за ментални нарушувања како единствено нарушување кое вклучува: аутизам, Аспергер синдром, детско дезинтегративно нарушување, первазивно развојно нарушување неспецифицирано. Бројот на деца со дијагностициран аутизам е во постојан пораст во светски рамки и во Република Македонија како резултат на зголемениот број и подобрата детекција на заболените. Раното поставување на дијагнозата има особено значење и директно влијае на резултатот од третманот. Хипербарната оксигено терапија како адјувантна терапија се употребува во третманот на деца со аутизам. Во прилог на ова се и резултатите од неколку студии кои потврдуваат клиничко подобрување кај децата по третманот. Во периодот 2009-2015 година во Хипербарната комора во склоп на Клиниката за пластична и реконструктивна хирургија беа третирани 64 деца со аутизам. Од нив 56 се од машки пол ,а 8 од женски пол. Според возраста 31 дете се под 7 годишна возраст,а 33 над 7 години. Во однос на местото на живеење доминираат оние кои живеат во Скопје - 40 деца, потоа Штип 5, Тетово 4, Битола 3, Кавадарци 3, Гевгелија 3, Охрид 2, Кочани 2, Неготино 1 и Куманово 1. Од децата на школска возраст 30 посетувале редовна настава, а 3 настава во училиште за деца со посебни потреби . Најголемиот дел дополнително работеле со дефектолог и примале одредена медикаментозна терапија. Овие податоци како и тие од литературата потврдуваат дека појавата на аутизам е скоро 5 пати почеста кај момчињата отколку кај девојчињата. Родителите кои имаат едно дете со аутизам имаат 2% -18% поголема шанса да имаат второ дете со аутизам. Нашите резултати не се во согласност со литературните кога се работи за возраста на родителите. Имено не потврдивме дека возраста на родителите мајка над 35 години и татко над 40 години преставува поголем ризик за појава на аутизам.


The role of blood cultures in the detection of septic condition Zaklina Cekovska, A. Kaftandzieva, M. Petrovska, N. Panovski Institute of Microbiology and Parasitology, Medical Faculty, Skopje Sepsis is a potentially life-threatening complication of an infection that requires urgent and comprehensive care. Mortality rate is approximately 15% in case of mild sepsis and 50% in case of severe sepsis and septic shock. Rapid diagnosis, detection of the etiologic agent and its antimicrobial susceptibility can be crucial in saving the patient’s life. The purpose of this study was to examine the role of blood culture in microbiological diagnosis of septic conditions. Following conditions should be achieved in obtaining valid diagnosis: taking a sample under aseptic conditions with minimized risk of contamination of the sample, real time sampling, the optimal amount of sample, a sufficient number of blood cultures from the same patient, a modern method of detection, appropriate media for cultivation, standard conditions of cultivation, proper reading and interpretation of the findings. The correct choice of the sample and its right collection depends on hospital staff, but the precise microbiological diagnosis is provided by microbiological staff in the microbiological laboratory. The Institute of Microbiology and Parasitology, has performed automated diagnostic blood cultures with Bact/Alert system (bioMerieux, France) for almost two decades. In recent years, the latest generation of BacT/Alert 3D system (bioMerieux, France) has been used. It brings the most advanced, innovative microbial growth and detection technology to our laboratory. BacT/Alert 3D media (PediBact medium, especially for children) provides unsurpassed performance, for detection of a wide variety of microorganisms including bacteria, fungi, and yeasts. This system offers immediate bottle recognition, control of the bottle loading and unloading and virtually eliminating bottle handling errors during microbial detection testing. The system's automatic, built-in quality control, along with a low false positive rate and a rapid response time of this system, means to do more in less time with greater accuracy. Blood cultures are incubating for 5 days and after the positive signal, Gram stain slide have been prepared. A phone contact is used to report the findings to the clinicians. After this, standard microbiological examination are performed, including in vitro susceptibility testing. This approach of microbiological treatment of blood cultures and collaboration with clinicians is a major imperative for rapid and successful therapy in treating septic conditions. Key words: sepsis, blood cultures, Bact/Alert 3D system


Toxic effects during therapy with high doses methotrexate in children with acute lymphoblastic leukemia

B.Choneska Jovanova, S.Glamochanin, K.Martinova, Z.Antevska Trajkova, S.Kocheva, A.Acevska

University Clinic for children diseases, Department of Hematology and Oncology, Skopje, Macedonia

Purpose/objective Intensification of systemic chemotherapy with inclusion of high doses methotrexate( MTX) has contributed to the improvement of event free survival in children acute lymphoblastic leukemia (ALL) and has helped to reduce cranial radiation for mostly patient. Despite this benefit, this agent might cause serious toxicity, even life treating events during the treatment. The aim of our study was to evaluate the incidence of toxic effects of chemotherapy with high doses MTX (5g/m2) and to compare them with toxicity during application of lower doses MTX(2g/m2)

Materials and methods Retrospective record review was done in 77 children with standard risk ALL treated in our department. Forty five of them were treated with 5g/m2 and 32 of them were treated with 2g/m2( historic group)

Results Toxicity of high doses MTX was predominant in the group treated with 5g/m2. Most significant toxic effects were hepatotoxicity 77% versus 25%( p=0.000013), oral mucositis 35.56% versus 18.75% (p=0.023) and myelosupresion. Anemia gradus 3 was present in 37.78% versus 6.25%, trombopenia gr 3 in 28.8% versus 12.5% and patient of the study group have experienced more episodes of neutropenia, 99 episodes of neutropenia versus 32. Bacterial and viral infections were predominant in the study group due to severe myelosupresion.

Conclusiones In our study toxic effects were more common in the study group due to application of higher doses MTX. Variationes in toxicity between the patients in the study group are probably due to the genetic differences in the drug metabolism.


Use of oral propranolol for treatment of hemangiomas in childhood

B.Choneska Jovanova1, S.Glamochanin1, K.Martinova1, R.Kacarska2, Z.Trajkova Antevska1, K.Maneva2, S.Kocheva1, S.Bojagieva3, A.Jovanovska1

1Department for Hematology and Oncology 2 Department for Cardiology 3 Department for Gastroenterohepatology University clinic for Children diseases, Department of Hematology and Oncology, Skopje, Macedonia Introduction: Hemangiomas are the most common benign vascular tumors in infancy with an incidence of 5-10% at the end of the first year. Traditionally, corticosteroids were the drug of choice when treatment was indicated. In recent years, beta blockers, most specifically propranolol, have been shown to be an effective pharmacological treatment.

Purpose: To report our preliminary results of treatment of pediatric hemangiomas with oral propranolol.

Methods: Two children, infant with hemangioma localized on the left hand and left part of the chest, in proliferative phase, and four years old child with localized hemangioma on the neck were treated with oral propranolol. Patients were admitted for three days for laboratory tests, abdominal ultrasound, cardiovascular evaluation and for monitoring their vital signs and blood sugar after starting the therapy. Lesion size and evolution were assessed ambulatory during the follow - up period.

Results: Significant improvement was noted in the infant with hemangioma in the first two months of treatment. Minor improvement was registered in the other patient. No serious complications were observed.

Conclusion: Our preliminary results indicated that propranolol is more efficient when administered in the proliferative phase of hemangioma evolution. It is necessary to have follow up observation of the patients.


Is quality of life in children with asthma dependent on the child’s sex?

Cvejoska Cholakovska V¹, Vlaski E¹, Velik Stefanovska V²

University Children’s Hospital, Skopje, Macedonia¹

Institute of Epidemiology and biostatistics, Skopje, Macedonia²

One of the aims of asthma management is to better the quality of life in asthmatic children. It is necessary to make direct measurement of the health in relation to the quality of life. In response to this need, we used Juniper’s Pediatric Asthma Quality of Life Questionnaire (PAQLQ), the Macedonian version. The PAQLQ consists of 23 items that in children with asthma have been identified as troublesome in their daily lives. The study design consisted of a 24 month single cohort study. Patients participating in the study were 64 children, 7-17 years of age, with a wide range of asthma severity. For each patient a PAQLQ was completed. One week before a visit patients recorded morning peak flow rates, medication use and symptoms in a diary. After completing physical examination, for determining the asthma severity, spirometry was performed. The questionnaires after statically analysis showed good levels of both longitudinal and cross sectional correlations with the conventional asthma indices and with general quality of life. We found that consistently QOL in males was more disturbed than in females, a good relevancy between severity of asthma and QOL scores in male asthmatic patients. We could not find any significant relevancy between FEV1 percentage of predicted and overall scores of QOL. Smaller airways, and higher airway resistance and more activity of males than females may explain why boys have more disturbed life styles than girls.

Key words: Asthma; Children; Quality of Life


Туберкулоза во детската возраст според нејзините ентитети во изминатите триесет и осум години

Институт по белодробни заболувања кај децата - Kозле, Скопје

Др. Дацевски Д., Др. Дилберовска М., Др. Зафировски О., Др. Бужаров Ј.,

Детската туберкулоза обилува со свои клинички ентитети и во ниеден случај не можеме едноставно да ја пресликаме состојбата од таа кај возрасните. Низ годините што поминале, почнувајќи го приказот од далечната 1976 год, па досега, се имаме соочено со бројни предизвици во дијагностиката и терапијата.

Целта на овој труд е да се запознаеме со бројот на деца лекувани при нашата болница на одделот за туберкулоза во одредени временски периоди, од ентитетите кои поминале, дијагностичките процедури и терапискиот пристап. Од приложениот материјал ја следиме и динамиката на болеста.

Првиот период кој го прикажуваме е од 1976 до 1982 год, кога било лекувано 1521 дете, во кој период доминираат комплицирани форми на белодробна туберкулоза. Овде би спомнале дека милијарниот облик на туберкулоза е застапен со 88 или 5,78% пациенти. Во периодот од 1986 до 1990 год, лекувани се само 13 пациенти или 2,42% од истиот облик. Во следниот период од седум години ( 1995 до 2001 год, ) следи значително намалување на вкупниот број на лекувани деца. Драстичен пад на бројот на заболени деца кои се лекувани во болницата бележи последниот период, од 2007 – 2014 год, вкупно 248 деца. Но за жал во овој период има остеоартикуларни случаи.

Со развој на здравствениот систем, сеопфатноста на населението во него, добрата едукација, пораст на стандардот, се разбира и ангажираноста на лекарите, можеме да бидеме задоволни што Република Македонија бележи успех во превенцијата и лекувањето на оваа болест.


Специфичен (туберкулозен) остеомиелитис на тибиа во педијатриска популација: приказ на случај.

Аида Далипи, Мирјана Дилберовска, Драган Дацевски, Љуљијета Мустафа.

Институт по белодробни заболувања кај децата-Козле, Скопје

Вовед: Екстрапулмоналната туберкулоза е ретка форма. Најчесто се манифестира со остеартикуларно инволвирање и претставува проблем во детската и адолесцентната популација.

Случај: Ви презентираме случај на 2 годишно женско дете, со болка и оток во дисталниот дел на десната потколеница во тек на 2 месеци, тригерирана од повреда на истата и краткотрајна фебрилност. Хоспитализацирана на Клиника за детски болести каде се реализирани иследувања и на Клиника за ортопедија каде е реализирана пункциона биопсија на мекоткивниот оток и отворена биопсија на дисталниот дел на десна потколеница со цитолошки и хистопатолошки наод суспектни за специфичен процес. Поради тоа е упатена до нашата институција. На прием афебрилна, хипотрофична, со уреден аускултаторен белодробен наод, со лонгета на десната потколеница, присутна болка и оток на истата.Негираат можен контакт со болен од туберкулоза.Од лабораториските испитувања: со забрзана седиментација на еритроцитите, пораст на ц-реактивен протеин и анемија; Манту тест (PPD5 ) со индурација 15мм. Земен гастричен лават за микробиолошки испитувања, истиот на директен препарат и културелно негативен. Земен брис од рана-негативен. Квантиферон тест- негативен. GeneXpert- позитивен за M.tuberculosis. Радиографија на граден кош и ехосонографија на абдомен со уреден наод. Радиографија и КТ на десна потколеница со промени во прилог на остеомиелитис.Започнато е лекување со антитуберкулозна терапија. Во третманот се вклучени и редовни преврски на раната и консултација со ортопед.

Заклучок: Дијагонстицирање на специфичен туберкулозен остеомиелитис кај децата е комплициран процес кој вклучува мултидисциплинарен пристап. Kај пациенти со хроничен тек на коскена лезија секогаш треба да се бара присуство на специфична (туберкулозна) етиологија за да се превенираат можните компликации од задоцнето лекување.


Endoscopic Retrograde Cholangiopancreatography (ERCP) in Children

L. Dall’Oglio Digestive Endoscopy and Surgery Unit Bambino Gesù Children Hospital Rome ERCP is an endoscopic procedure that allows the contrast injection of contrast medium in the biliary and pancreatic ducts.,with a thin cannula inserted in to the operative channel of a side view jejunoscope.

Through this channel it is also possible to pass different accessories for therapeutic procedures. The therapeutic ERCP is now more important than before; in children too.

Modern and effective diagnostic tools, like magnetic resonance cholangiopancreatography (MRCP) and ultrasound, allow full morphology of biliary and pancreatic tree.

In uncollaborative children, the difficulty to obtain a breath free MRCP pictures, the diagnostic ERCP role remains s very important. In recurrent acute pancreatitis the presence of a common biliopancreatic duct needs to be ruled out for its possible role. A correct surgical strategy is very important in choledochal cysts. The common channel length needs to be correctly evaluated to avoid its incomplete resection in the pancreas head. The persistence of a choledochal stump may allow for acute recurrent pancreatitis.

Common therapeutic biliary ERCP in children are stones removal, choledochal stricture dilations and stenting, biliary tree post traumatic or post surgical leak, tumor suspicion. Sphincterotomy may represent an important therapeutic option in acute biliary pancreatitis or acute cholangitis.

In chronic pancreatitis, that is growing up in frequency in children, the therapeutic ERCP allows pancreatic sphincterotomy, proteic sludge removal, Wirsung dilation and stenting. Minor papilla sphincterotomy can represent the best therapeutic option in pancreatitis due to pancreas divisum.

Pancreatic traumatic duct leak can be treated with sphincterotomy and stenting, avoiding major surgery.


Brain injury in premature infants Darja Paro-Panjan Department of Neonatology University Children's Hospital, University Medical Centre Ljubljana

Advances in the treatment of premature infants have led to increased survival rates of these children, but the incidence of motor and cognitive developmental delay, which is the result of the brain injury, remains the same. The most common brain diseases of the premature infants are intraventricular hemorrhage, posthemorrhagic hydrocephalus, white matter injury and cerebellar injury. Most recent advances concentrate on the white matter injury; it is associated with preoligodendrocyte vulnerability and damage by excitotoxic and free radicals effects during the process of inflammation or ischemia. Periventricular encephalopathy is consequently also followed by neuronal and axonal damage. Clinical signs of the brain injury are often subtle or even absent, therefore some new non-invasive methods are used to identify and estimate central nervous system dysfunction. They provide insight into hemodynamics, electrical activity and structure of the brain. Knowing and understanding the causes and the mechanisms of the brain injury yield to a number of more or less successful neuroprotective approaches that reduce the negative influences on the developing brain. Since the brain injury and consequentlly its long term neurodevelopmental outcome remains the most important complication of the premature birth, a regular and comprehensive follow up of motor, cognitive and behavioral development of these children is essential, as it provides early detection of potential delays and intervention.

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Хеморагична болест кај новородени – наши искуства

В. Деловска – Стојкова, Ј. Гулева, Б. Михајловиќ, М. Зип, Б. Поцеста, Б. Исмаили

Вовед – Хеморагична болест кај новородените е релативно честа појава во новороденечкиот период. Во литературата се среќава со различни термини како: HBN, MHN, VKDB, HDN. TOWNSEND – 1864 прв ја опишал група на доенчиња кои развиле хеморагична болест “ особено изразени крварења од гастро интестинален тракт”. Во 1960 – откриена е етиологијата на хеморагичната болест кај новородените. Потврден е дефицит на К-VITAMIN зависните фактори на коагулација II, VII, IX, X. Зошто кај новороденото има дефицит на К-VITAMIN зависните фактори?

1. Ниско ниво на K-VIT во мајчиното млеко 2. Оскудно минување низ постелката 3. Намалена продукција во интестинумот на новородените

Инциденца во користената литература се движи од 1 : 200 до 1 : 400. Во однос на време на појавување може да бидат : рани, класични и касни, а во поглед на клиничката слика оскудни до профузни крварења во : CNS, GIT, КОЖА, ПАПЧЕ. Дијагноза: намалени вредности на фактор II, VII, IX, и X со нормален фибриноген и тромбоцити. Терапија : VITAMIN К мускулно трансфузија на Er и Тr терапија на основното заболување Превенција: препорачана од American Academy of Pediatrics - мајки – 10 мг интравенски VIT-K – 24 часа пред породување и тоа кај мајки што се антикоагулатна и антиепелептична терапија - новородени

- недоносени 0,5 мг кога имаат тм < 1000 gr - матурните 1 мг веднаш после рагање

Новородените од мајки со антиконвулзивна и антикоагулатна терапија треба да примат 1 мг веднаш после раѓање мускулно и 1 мг по првите 24 часа. Резултатите од анализата покажаа дека инциденцата во периодот 1985-2000 и 2000-2015 година, рапидното се намалуваат по секојдневната превентивна употреба на VITAMINOT K кај секое новородено 1985. – MHN застапен 0,6 % 2000 - МHN застапен 0,4 % 2015 – MHN застепен 0,3 %


Common Problems in Adolescence Prof. Dr. Orhan Derman Hacettepe University Medical School Department of Pediatrics Adolescent Unit- Ankara 06100 TURKEY Introduction Adolescence is a transition period between childhood and adulthood. According to WHO (World Health Organization), 10-19 years old are adolescence, 15-24 years old are the youth and 10-24 years old are the young people. Adolescence has contained three different steps for maturation. In order these are; physical growth, sexual development and psychosocial maturation. All steps do not start and complete at the same time among adolescence. The adolescence girls start puberty two years earlier than boys. The most common problems in adolescence are related to growth and the development of sexual and psychosocial. Material and methods: Common problems in adolescence related epidemiological data from the Adolescent Medicine Unit of Hacettepe University was obtained and analyzed. Results: Each year 7000 new adolescence cases are expected to be evaluated in the Adolescent Medicine Unit of Hacettepe Univesity . According to our data, all problems in adolescence are related to growth and development of sexual and psychosocial. In addition, childhood illnesses that continue into adolescence; mental health disorders; the consequences of risky or illegal behaviors, including injury, legal consequences, pregnancy, and infectious diseases. Unintentional injuries resulting from motor vehicle crashes and injuries resulting from interpersonal violence are leading causes of death and disability among adolescents. Common problems are seen in Adolescent Unit of Hacettepe University such as, Growth Retardation, Short statue (%12 of applying to the outpatient clinic each year) and long statue (very rare, approximately 5-10 patients each year), Pubertal problems (We are referring to the Pediatric Endocrinology Department according to the modified Rosenfield score), Hypertrikosis, hirsutism (We are evaluated the patients related to the Ferriman and Gallwey scoring for hirsutism and androgenic hormon levels) , Breast disorders (%1 of our patients have breast disorders problems) , Gynecomastia (if we accept the size of glanduller tissue of breast more than 0.5 cm, two out of three adolescents have gynecomastia problems during adolescence), Adolescence Gynecologic Problems (Menstrual problems are seen the most common adolescent gynecologic problems among girls in Turkey, dysmenorrhea are seen %85 adolescent girls during adolescence), Scortal disorders (We are referring 15-20 adolescent boys to the Department of Pediatric Surgery due to having third grade varicocel. Eating disorders (Approximately 25-35 new cases are followed with the department of Child Psychiatry each year), Obesity (if we add the overweight adolescents to the investigation group, %20 adolescence have the obesity problems), Orthopedic problems (Posture problems are seen the most common then apophysial lesions are proceeding), Dermatologic problems (%95 of dermatologic problems are acne), Psychosocial problems (Relationship problems with parents, peers and siblings and/or school failure) may develop or first become apparent during adolescence, leading to a risk of adolescence health. Conclusion Eating disorders, such as anorexia nervosa and bulimia nervosa are particularly common among adolescent girls. Breast disorders in girls are the most commonly seen developmental anomalies


(Asymmetric Breast Development, Accessory Breast Tissue, Absence of Breast Tissue ( Amastia ve Athelia ), Tuberous Breast Deformity, Macromastia) and Benign Breast Disease; Physiologic Swelling and Tenderness, Mastalgia ( Severe pain, either cyclic or noncyclic), Proliferative Breast Changes (nodularity, fibrocystic changes), Fibroadenomas (common, giant, juvenile, phyllodes). Fibroadenom is the most common problems among the adolescence girls. Menstrual problems are the most common problems among adolescence girls in Turkey. In order theaes are:Amenorrhea, Dysmenorrhea, Dysfunctional uterine bleeding, Polycystic over syndrome and Vaginal discharge. Gynecomastia are seen the most common problems among adolescence boys which is glanduler enlargement of the male breast and a transient or permanent disturbance in streoid hormone physiology. Scrotal evaluation are ignored during the adolescence examination. We have to keep in mind varicocel which are seen the same percentage of the adult men. Varicocel is the most common problems for the male infertility.


Важноста на превенцијата и контролата на туберкулозата во детската возраст

Дилберовска.М , Дацевски Д., Бошковска К. , Далипи А. Институт по белодробни заболувања кај децата-Козле, Скопје,Р.Македонија Цел: Да се прикаже големата важност на правилно и навремено спроведување на превенција на туберкулозата(ТБ) во детската возраст, а со тоа истата да биде ставена под контрола.

Материјал: БеСеЖе вакцинацијата е наједноставна и најефтина метода во превенцијата на ТБ , но не и најефикасна. Посебно важна е процедурата за идентифицирање на лица, особено деца, кои биле во контакт со болен од активна форма на ТБ, испитување на латентна ТБ инфекција(ЛТБИ) и овозможување на адекватно лекување на ТБ и ЛТБИ.

Резултати: Една третина од светската популација е инфицирана со M.tuberculosis. Без дијагностицирање и лекување на инфекцијата околу 5-10% ќе се разболат од ТБ. ЛТБИ е инфекција со M. tuberculosis без постоење на знаци или симптоми на болест, ниту пак бактериолошка или рентгенолошка потврда на ТБ. Се испитуваат лица ( деца) од близок контакт со болен од ТБ, неодамнешни конвектори на туберкулинскиот кожен тест, особено деца под 5 годишна возраст, лица кои се врзани со одреден ризик како: ХИВ инфицирани, корисници на дрога, потхранети, и специфични други медицински услови. За дијагноза налчесто се користи туберкулински кожен тест, радиограм на бели дробови, и квантиферон( гама интерферон) тест. Третманот на ЛТБИ се спроведува медикаментозно со табл.изониазид во тек на 6-9 месеци.

Заклучок: Важноста на скрининг инвестигациите кај деца кои оствариле контакт со болен од активна ТБ е голема, се со цел да се превенира појава на туберкулозна болест кај децата.


Хронична кашлица

Дејан Докиќ

Универзитетска клиника за пулмологијаи алергологија.

Некои најчести причини на хронична кашлица се астмата, алергискиот ринитис, инфекции на

параназалните синуси, езофагеална рефлуксна болест и др. Во поретки случаи хроничната

кашлица може да се јави при инхалација на страно тело (особено кај децата). Заради

горенаведеното секогаш се препорачува на пациентите со хронична кашлица да се упатат на

доктор кој заради хроничноста на симптомите честопати ординира РТГ на белите дробови.

Најчестите причини за хронична кашлица се:

1. Пушењето на цигари коешто претставува најчеста причина за кашлица.

2. Астмата претставува болест на бронхијалното стебло како резултат на инфламацијата на

бронхијалната мукоза којашто води до бронхијална преосетливост и симптоми на свирење во

градите, тешкотии при дишењето, диспнеја и абнормални спирометриски тестови.Некои

пациенти со астма имаат само еден единствен симптом, а тоа е хронхична кашлица и

нормални функционални тестови. Ваквата форма на астма се означува како cough-variant

asthma. Симптомите на астма може да се влошат при изложување на пациентите на

аерозагадувачи, полен, чад од цигари, ладен сув воздух, јаки мириси (парфеми) и др. Кај

пациентите со астма, горно респираторните вирусни инфекции честопати резултираат со

пролонгирана кашлица којашто перзистира и по исчезнување на инфекцијата.

3. Гастро-езофагеална рефлуксна болест (GERD) е болест при којашто киселата содржина од

желудникот се враќа во езофагусот. Ова резултира со рефлексен спазам на дишните патишта

којшто клинички се манифестира како тешкотии во дишењето и кашлица. Понекогаш кога

рефлуксот на гастричната содржина е многу изразен може да дојде до аспирација (инхалација)

на желудечна киселина при што се оштетуваат белите дробови. Кај некои пациенти отсуствива

симптомот на печење во градите (карактеристичен симптом за рефлукс), а како единствена

клиничка манифестација се јавува кашлицата.

4. Воспаление на синусите и пост назален исцедок можат да предизвикаат хронична кашлица.

Понекогаш е навистина тешко да се дијагностицира ваквата состојба па е потребно

изведување на КТ на параназалните синуси. Пациентите честопати се жалат на јадеж во грлото

и прават маневар на прочистување на истото.


Esophageal atresia with distal tracheoesophageal fistula (case report)

Doneva L, Shpritova E, Breslieva J

Clinical hospital – Shtip, Macedonia

Background: Esophageal atresia with tracheoesophageal fistula is a birth defect with an incidence of 1:4000 newborns. It was first described in 1670 and the first surgical correction was made in 1920. The first long term survival after a successful surgical correction was reported in 1939.

Goal: Description of a newborn with esophageal atresia with distal tracheoesophageal fistula that was diagnosed within 24 hours after birth.

Material and methods: Clinical observation, blood tests, babygram, esophagogram. Results: Male newborn that was born naturally in the 40th gestational week with birth weight of

3350g and Apgar score 7/9. The amniotic fluid was with signs of meconium. The vital signs were normal on birth. Four to five hours after birth the newborn started vomiting and the vomited material was with signs of meconium. The breathing was diminished vesicular with crackles bilaterally. The respiratory rate was 30-50 per minute. There was 10% drop of the body weight compared to the birth weight. WBC: 25,36x109/l, GRA: 68,8% , RBC: 5,2x1012/l, HGB: 186 g/l, PLT: 131x109/l, HCT: 51,2%. The babygram showed normal heart contours and no signs of consolidation in the lungs. The newborn was treated with intravenous rehydration solutions and a third generation cephalosporin. The vomiting continued, now without any signs of meconium in the vomited material. Hypersalivation was also noted. There was a resistance when trying to insert a nasogastric tube. An esophagography was performed through the placed tube using a nonionic contrast. On the esophagogram it was clearly visible that the proximal segment of the esophagus was dilated and atretic, thus not allowing the contrast to pass to the more distal parts of the esophagus. There was air in the stomach which is an indirect sign of a tracheoesophageal fistula. The newborn was sent to the Clinic for child surgery in Skopje for a definite treatment.

Conclusion: In a newborn with a severe and continuous vomiting and hypersalivation esophageal atresia

should always be considered as a possible diagnosis. The positive outcome is a fruit of a devoted team work.


Pediatric paliativ care a big challenge of pc: our experience

Donjeta Alia (Bali)1, G.Cekani2, Y.Voka3, M.Xhafa1, E.Nastas1, A.Godo1. 1PediatricOncoematologist 2Social Worker 3Psychologist -OncoematologicServise, Pediatric Department -University Hospital Center “Mother Theresa”-Tirana, Albania Introduction The pediatric palliative care as the active total care of body, mind and spirit of the child and includes the active support to the family (Cancer Pain Relief and Palliative Care in Children, WHO - IASP, 1998 defines); An infant or child will benefit from palliative care when no treatment has been shown to alter substantially the expected progression toward death. The number of children eligible for palliative care is increasing: at least 10 out of 10.000 children aged 0-19 years suffer from life-threatening conditions. Too many of them die in hospitals, often in intensive care facilities; only a small percentage die at home, even if this is what most of them would prefer. Many families are left on their own to cope. In fact, they often suffer unnecessary pain and distress due to poor symptom management and prolonged hospitalizations. Instead, palliative care can make an enormous difference to the quality of their life and to that of their family members. The causes of death in children are substantially different from the causes of death in adults; thus, palliative care guidelines that are appropriate for adults are often inappropriate for children. The goal of pediatric palliative care is the achievement of the best quality of life for patients and their families, consistent with their values, regardless of the location of the patient. Purpose To give a panorama of PPC in general, our experience and challenges in promotion of this “new” discipline in our country with low resource. Results Few and limited in scope and research (specific groups of disease) are the research related to the medical and social (family and non-family) of incurable pediatric disease. Conclusion Palliative care and respite programs need to be developed and widely available to provide intensive symptom management and promote the welfare of children living with life-threatening or terminal conditions.It is important to offer an integrated model of palliative care that continues throughout the course of illness, regardless of the outcome.All medical staff needs to become familiar and comfortable with the provision of palliative care to children.Our scholar curricula should include topics such as palliative medicine, communication skills, grief and loss, managing prognostic uncertainty, and alternative medicine. Key words Pediatric Palliative care, experience, challenges, low resourses.


Тactile kinesthetic stimulation and development in very low-birth-weight infants

Valentina Dukovska1, Filip Duma1, Vesna Sabolik1, Natalija Angelkova1

1University Clinic for Children's Diseases, department of neurology

Introduction: Early stimulation program, approach to sensory enrichment for very young children, particularly those who are ill or who are otherwise deprived of typical sensory experiences. Infant stimulation is a process of providing supplemental sensory stimulation in any or all of the sensory modalities (visual, auditory, tactile, vestibular, olfactory, gustatory) to an infant as a therapeutic intervention. The intervention uses supplemental stimulation to compensate for the lack of normal or typical environmental sensory stimulation or the presence of abnormal or atypical environmental sensory stimulation. For example, premature infants are exposed to high levels of intense and aversive sensory stimulation related to necessary medical care. Furthermore, these infants do not receive the same caregiver stimulation and interaction that healthy full-term infants.

Aim: The primary aim of this research is to determine the impact of the tactile kinesthetic stimulation on the general developmental outcomes in preterm infants, or in other words, its influence on the motor, social and cognitive development, including the parents too.

Population and sample: The sample consists of 50 very low-birth-weight infants, who have been exposed to tactile kinesthetic stimulation and 50 very low-birth-weight infants not exposed to stimulation. Griffiths developmental scales were used to assess developmental abilities.

Results: The analysis of the results confirmed that the very low-birth-weight infants who have been exposed to tactile kinesthetic stimulation demonstrate an improved developmental outcome in relation to the control group at the end of the 24-month Gestation-Adjusted Age, particularly concerning the motor, social and cognitive development.

Conclusion: The obtained results undoubtedly confirm that early sensorimotor stimulation positively affects the developmental outcomes in very low-birth-weight infants.

Key words: very low-birth-weight infants, tactile kinesthetic stimulation, early intervention,


Diagnosis and treatment of Osgood-Schlater disease

Dzoleva-Tolevska R1, Georgieva D1, Samardziski M1, Bozinovski Z1, Maneva K2, Georgiev A3. 1University clinic for orthopaedic surgery, 2University Children Hospital, Clinical Campus “Mother Theresa”, 3PHO Cardiology-Prima, MIT University, Skopje, R. Macedonia. Abstract

Introduction: Osgood-Schlater disease is frequent cause of knee pain in children and adolescents, especially in those with extended sports activity. This disease is in group of juvenile osteochondrosis and it is a result of repetitive quadriceps contraction through the patellar tendon at its insertion upon the skeletally immature tibial tubercle.

Aim: The aim of this article is to analyze the accuracy of the patients treated conservatively versus patient treated operatively.

Material and methods: We analyzed 70 patients with Osgood-Schlater disease, between 9 and 15 years old. We used method of clinical examination and radiographic method for establishing diagnosis. The radiographic method was used for dividing patients in three groups. First group: patients with normal radiographic findings. Second group: patients with elongated and fragmented tibial tubercle on RTG. Third group: patients with separated tibial tubercle on RTG.

Results: Male: Female ratio was 3:1. In the first group there were 43(61,42%) patients with normal radiographic findings. In the second group there were 25(35,71%) patients with elongated and fragmented tibial tubercle on RTG and in third droup there were 2(2,85%) patients with separated tibial tubercle on RTG. From all analyzed 70 patients with Osgood-Schlater disease 97,13% were treated conservatively, and only 2,85% were treated operatively.

Conclusion: In this article ratio between conservative: operative treatment of Osgood-Schlater disease was 34:1. Early diagnosis of this disease and conservative treatment gives us better results. Operative treatment is very rare in Osgood-Schlater disease.

Key words: Osgood-Schlater disease, Knee


Meniscal lesions in children

Dzoleva-Tolevska R1, Temelkovski Z1, Samardziski M1, Georgieva D1, Nanceva J2, Jovanovski-Srceva M2. 1University clinic for orthopedic surgery,2University clinic for anesthesiology, reanimation and intensive care, Clinical campus “Mother Theresa”,Skopje, Republic of Macedonia Abstract Introduction: Meniscal lesions of the knee in children are diagnosed and treated by same methods asin adults.

Aim: The aim of this paper is to present the importance of the clinical examination in the process of diagnosis meniscal lesionsin children, as well as to establish the percentage of the clinical diagnosis accuracy in regard to the arthroscopic diagnosis.

Material and methods: In the University Clinic for Orthopedic surgery in Skopje, we diagnosed and treated 20 patients (children under 16 years old) with meniscal lesions. The method of well taken anamnesis and clinical examination has been applied for establishing clinical diagnosis. We also usedradiographic method as: standard radiographic images of the knee and MRI for some of the patients. Arthroscopy was used as diagnostic and operative method.

Results: The results obtained with the clinically established diagnosis were compared to the results obtained with the arthroscopic examination. The accuracy of clinical diagnosis was 80% in comparison to the diagnosis established with arthroscopy. Arthroscopy was used as gold standard. We found 16(80%) medial meniscal lesions, 3(15%) lateral meniscal lesions and 1(5%) lateral discoidal meniscus.

Conclusion: Clinical signs and tests, as well as the well taken anamnesis, are an excellent foundation for establishing the clinical diagnosis of meniscal lesions. Arthroscopy gives us direct visualization of the knee, and because of that it is most informative and most affirmative tool for diagnosis knee injuries and changes in the joint.

Key words: Meniscal lesions, Knee, Arthroscopy.


The data on short-term and long-term complications of the infection caused by the pharyngotonsillitisof the Streptococcusgroup Ain clinical pediatrics

Prof.asc.dr Edmont LAHO1, Prof .drGjeorgjina Kuli-Lito2

1Elbasan Regional Hospital, University "A.Xhuvani" Elbasan, Albania 2University Hospital Centre "Nënë Tereza", Tirana, Albania

Introduction Among the most frequent pathologies in pediatric age are pharyngotonsillitis where about 20 % are caused by Streptococcus group A which is resistant and important to follow and be treated effectively.Pharyngotonsillitis caused by Streptococcus Group A are dangerous to the health of children and young adults.Streptococcus Group Acause short and long term complications such as acute rheumatic and glomerulonephritis, which are threatening the health of children and adults. Aim of study Evaluation of the performance of short-term complications caused by Streptococcuspharyngotonsillitis of Group A.The assessment of the progress and more detailed data on complications caused by Streptococcus Group A. Materials and methods This is a prospective study of extended time period 2004-2008. From all cases raised we have followed and studied all pharyngotonsillitiscases caused by StreptococcusGroupA(SGr.A). Criterias of study group were among others, clinical forms, throatculture, performance, complications, age, place of residence, etc. Results From all cases studied and hospitalized at our hospital have resulted 785pharyngotonsillitis and 173 of those caused by Streptococcus Group A.Data from over the years resulted : short-term complications are frequent otitis by 17% , with 10 % sinusitis etc. As for long term complications is concluded rheumatoid acute polyarthritis in 4 cases at a frequency of 2.3% and acute glomerulonephritis in 3 cases with a 1.7% frequency. More affected ages by long-tem complications is 7-11 years and about 75 % are from rural areas. Conclusions The data clearly showed that pharyngotonsillitiscaused by SGr.Abesides short -term complications cause also and long-term complications.Although there are great decreased of cases over the years, they continue to be a cause of acute complications like rheumatoid polyarthritis with glomerulonephritis prevalence 2.3% and 1.7 % .While foreign authors describe in developing countries these complicationsranged in 2-5 %. Keywords :pharyngotonsillitis, Streptococcus , Group A , polyarthritis , glomerulonephritis.


Surgery inInflammatory Bowel Disease (IBD)

G. Federici di Abriola

Digestive Endoscopy and Surgery Unit Bambino Gesù Children Hospital Rome

Inflammatory bowel disease (IBD) are chronic disease that may affect different part of bowel.

Commonly they are classified as Ulcerative colitis (UC), Crohn’s disease (CD) and Indeterminate

colitis (IC). Pediatric onset of IBD is charachterized by aggressive course and worse outcome.

Frequently UC has an extensive localization, with pancolitis onset and severe course. Crohn’s disease

may affect all segment of bowel, also perianal region with abscesses, fistula, skin tags; complications

of CD are abdominal abscesses, intestinal fistula, strictures.

IBD in children may cause failure to thrive, puberal delay and malnutrition. So it’s important to

identify and treat these pathologies especially when with early onset.

Diagnosis of IBD derives from collection of clinical and biochemical data, imaging (abdominal

ultrasonography, abdominal CT scan or Magnetic Resonance, endoscopic and istological

examination).

Treatment are represented by steroids, immunosuppressants (thiopurines, methotrexate), biological

drugs (infliximab, adalimumab), and they may have side effects on bone mineral density, on growth,

on relationships.

Surgery is a therapeutic approach, curative in ulcerative colitis butreserved to complications in

Crohn’s disease.

Indication to surgery are relapsing-refractory disease, or acute severe ulcerative colitis.

Colectomy with proctectomy and ileo-anal-pouch anastomosis is the preferential approach in

surgery for ulcerative colitis.

Surgery for complicated Crohn’s disease is represented by perianal curettage on abscesses and

fistula andintestinal resection or strictureplasty for bowel stricture.

Elective surgery,when possible, must be the first choice for patients with ulcerative colitis or Crohn’s

disease.


New pharmacological approaches in treatment of cystic fibrosis: achievements and challenges

Stojka Fustikj

Cystic fibrosis (CF) is an autosomal recessive disease which is caused by a mutation in each of the 2 CFTR genes. Almost 2000 different mutations have been reported, in that F508del is by far the most common mutation. It is found in about 70% of CF chromosomes worldwide and is present in ~85% of patients on at least one allele. CF is a multisystem life shortening condition affecting organs and tissues where CFTR is expressed. The common clinical manifestations are related to impact of the defect gene on the airways, gastrointestinal tract including the biliary system and the reproductive tract. About 85% of patients with CF have pancreatic insufficiency associated with malabsorption and often under-nutrition. Therapies are complex and involve pancreatic enzyme supplementation, fat soluble vitamins, mucolytic (dornase-alpha) and hydrator therapies (hypertonic saline, mannitol), airway clearance, and frequent and often repeated courses of antibiotics. The vast majority of morbidity and mortality results from pulmonary disease associated with chronic bronchial infection and bronchiectasis. Lung disease remains a progressive condition and the burden of therapy is very significant for the patient, his family and the health care system.

Since the discovery of the CFTR gene in 1989, the search for therapies to improve the basic defects of CF commenced. The understanding of the complex biology of CFTR protein function has advanced significantly and allowed prospects of developments of therapies specifically designed to address the basic defects of CF. The elucidation of the molecular and cellular effects of mutations provide the scientific basis for development of targeted compounds for mutation-specific correction. CFTR mutation classes

CFTR mutations have been classified according to their functional defect, as follows : - Class I: mutations interfering with protein synthesis. They result in the introduction of a premature signal of termination of translation (stop codon) in the mRNA: nonsense, frameshift or severe splicing mutations. The truncated proteins are unstable, rapidly degraded, so, the net effect is no protein at the apical membrane (e.g. G542X, 711+1G>T, 621+1G>T). - Class II: mutations affecting the protein maturation. These lead to the production of a protein that cannot be correctly folded and trafficked to its site of function on the apical membrane, retention of a misfolded protein at the ER, and subsequent degradation in the proteasome (including the most prevalent, F508del). - Class III: mutations altering the channel regulation through impaired gating. The mutated protein is properly trafficked and localized to the plasma membrane but cannot be activated or function as a chloride channel (missense mutations located within the NBD e.g. G551D). - Class IV: mutations affecting chloride conductance. The CFTR protein is correctly trafficked to the cell membrane but generates reduced Cl ̄ flow (most are missense mutations located within the membrane-spanning domain). - Class V: mutations reducing the level of normally functioning CFTR at the apical membrane (cause significant reduction in mRNA and/or protein levels often through causing alternative splicing) - Class VI: mutations decreasing stability of CFTR present or affecting the regulation of other channels.

Mutations belonging to classes I, II, III and VI confer little or no functional CFTR at the apical membrane and are considered as "severe" and usually lead to a classic CF phenotype with pancreatic insufficiency (CF-PI) although the severity of lung disease may be variable. Mutations belonging to classes IV and V retain some residual CFTR activity and confer a milder phenotype. In patients with at least one "mild" CF allele, CFTR function is usually sufficient for digestion (CF-PS), and the lung disease is less severe.


CFTR modulators (Mutation-specific therapies)

The major virtue of the CFTR mutations classification lies in adapting strategies of drug development to the specific defects caused by groups of mutations. Several CFTR modulator therapies including ˝potentiators˝ (to improve channel gating, for class III mutations), premature termination codon ˝read-through˝ therapies (relevant for patients with premature stop codons, most class I mutations) and ˝correctors˝ (to improve abnormal CFTR protein folding and trafficking, for class II mutations) are investigated at present in late phase clinical trials and some of them are approved for use.

- CFTR potentiators - CFTR channel activators, which are termed potentiators such as VX-770 (ivacaftor), improves CFTR channel gating and Cl− current. After several successful clinical trials, the potentiator - ivacaftor is now licenced (by both FDA and EMA) for use in adults and children (> 6 years), with CF having at least one G551D CFTR mutation and FDA licence was recently expanded to include 8 additional class III gating mutations.

- CFTR correctors - Chemical and molecular chaperones can potentially promote protein folding, allowing the mutant protein to escape ER degradation and reach the cell surface. These compounds have been termed correctors. The corrector VX-809 (Lumacaftor) which showed great success in vitro, as corrector monotherapy demonstrated only modest results for F508del/F508del patients. It became clear that full correction of F508del-CFTR in CF patients will require double or even triple combination therapy. Lumacaftor in combination with ivacaftor provided a benefit (improved lung function and reduced the rate of pulmonary exacerbations) for patients with CF homozygous for the F508del CFTR mutation. Therefore, combination of a CFTR corrector and potentiator, designed to address the underlying cause of CF by targeting CFTR, can benefit patients who are homozygous for the F508del CFTR mutation and represents a treatment milestone for the 45% of patients with cystic CF who are homozygous for this mutation. In July, 2015 FDA approved Orkambi (lumacaftor/ivacaftor), as medicine to treat the underlying cause of CF, in people ages 12 and older with two copies of the F508del mutation.

- Premature termination codon ‘read-through’ therapies - CFTR modulators also include treatments directed towards premature termination codons (class I mutations), thereby permitting translation to continue to the normal termination of the transcript. Ataluren (PTC124) was studied in two open label phase 2 trials with modest results in terms of efficacy. Further development of small molecules enhancing read-through of class I (stop) mutations is anticipated. Alternative therapies (non-CFTR based therapeutic approaches) such as gene therapy and pharmacological modulation of other ion channels, may be advantageous because they are mutation-class independent. The major virtue of these therapies is that they apply equally to all patients with CF. However, the progress in these researches is less well advanced. Impact and challenges of new treatments in CF community The potential for CFTR modulator therapy and other strategies that attack the basic CF defect has been a tremendous boost to the CF community. Despite this, there are challenges beyond those posed by the complexity of CFTR dysfunction. The cost of CFTR modulator ( ivacaftor or lumacaftor/ivacaftor) is extremely high compared with other therapies in CF and would far exceed the current total budget for CF care in many countries. There are significant challenges to deliver these new therapies to all CF patients who will benefit from therapy. Other CFTR modulator drugs have been identified and are currently being evaluated in pre-clinical studies and early phase clinical trials. There is much excitement in the CF community for therapies which have the potential to alter the natural history of CF. To obtain the ultimate goal of full CFTR correction in all patients, basic biology research efforts have to be continue.


Cystic fibrosis related diabetes: the prevalence and impact on clinical status of patients

S. Fustik, T.Jakovska, L.Spirevska, A. Stamatova University Children’s Clinic, Skopje, R.Macedonia

Objectives: It has been suggested that cystic fibrosis-related diabetes (CFRD) may be associated with faster decline in pulmonary function and in clinical status of CF patients. In this study we investigated the prevalence of CFRD in group of patients older than 10 years and the clinical and genetic characteristics of these patients.

Methods: An annual oral glucose tolerance test is performed in all CF patients older than 10 years (N=65). Patients were diagnosed as having CFRD if 2 hours blood glucose was ≥ 11.1 mmol/L. Associations with the following parameters were studied: age, gender, genotype, chronic lung infections, liver disease, BMI, FEV1 and FVC. Parameters of clinical status were collected one year before and one year after onset of CFRD and insulin therapy.

Results:11 patients developed CFRD (6 male, 5 female). An overall prevalence of CFRD in study population was 16.9%. Mean age at diagnosis was 18.6 (10-27) years. All patients were PI, all had chronic pseudomonas lung infection, and 8 (73%) were with chronic liver disease (cirrhosis). Genetic analysis showed high frequency of F508del mutation in the group with CFRD (86.3%). Increase in BMI (18.7±2.9..19.6±2.8; p=0.09), whereas decrease in FEV1% predicted (80.3±12.5..73.5±18.6; p=0.055) and FVC% predicted (89.6±10.3..86.1±14.6; p=0.2) were observed within one year of insulin therapy, but the differences did not reach statistical significance. Two patients in diabetic group had remarkable decline in pulmonary functions and increase rate of hospitalization.

Conclusion: CFRD is the common comorbidity in CF and may be associated with progressive clinical deterioration in some but not in all diabetic patients.


Дентално здравје во раното детство

Гацов Д.

Апстракт

Денталниот кариес е хронично, прогресивно заболување на тврдите забни ткива со комплексна и мултикаузална етиологија, при што кариогениот дентален плак предизвикува деминерализација на забните ткива. Кариесот во последните години се дефинира и како најраспостранетото инфектвно заболување кај човекот.

Цел на истражувањето: Да се испита влијанието на социјалната средина кај децата од предучилишната возраст; да се направи проценка на преваленцијата на денталниот кариес; да се направи проценка на преваленцијата и тежината на кариесот во раното детство (КРД).

Материјал на истражувањето: Истражувањето беше изведено како аналитичка студија на пресекот на 30% примерок на 979 деца од 10 предучилишни установи (градинки) на територијата на Општина Карпош во Скопје, кај деца од семејства со различен социоекономски статус.

Резултати: Од испитаните деца, без кариес беа 173 испитаника (17,67%), со кариес 465 (47,5%), со КРД 207 (21.14%) и со санирани заби 134 (13.69%).Кај децата без кариес, 105 (61,11%) од нивните татковци имаа високо образование, 68 (38,89%) имаа татковци со средно или основно образование. Кај децата со кариес, 251 (53,98%) од татковците имаа високо образование, 214 (46,02%) имаа таковци со средно или основно образование. Кај децата со барем еден вработен родител, процентот на деца без кариес беше 29%, а кај деца со невработени и двата родители истиот процент на деца без кариес беше 23%.

Испитувањето покажа дека постои голема поврзаност помеѓу степенот на деца со КЕП (кариес, екстракција или пломба) или со КРД и спиењето со цуцла и шише. Исто така се покажа дека постои голема поврзаност помеѓу пиењето на зашеќерени пијалоци и конзумирање на слатки и развојот на кариес или КРД во раното детство.

Заклучок: При комплексната интеракција на предиспонирачки фактори на социјалната средина како што се: социоекономскиот статус, здравствената едукација, градската и животна средина, се покажа дека овие фактори имаат значително влијание врз преваленцијата на кариесот и тежината на КРД кај децата од предучилишна возраст.


Is postoperative pediatric emergence delirium influenced by the type of anesthesia in hernia repair?

Gavrilovska-Brzanov A, Donev Lj, Leshi A, Kartalov A, Kuzmanovska B, Srceva-Jovanovski M, Mojsova-Mijovska M, Simeonov R, Brzanov N

1University Clinic for Anesthesiology, Reanimation and Intensive Care Medicine Clinical Center Mother Theresa, Skopje Background and objectives: Emergence delirium (ED) is a transient state of marked irritation and disassociation after the discontinuation of anesthesia in some patients which does not respond to consoling measures. Various causes of ED have been proposed, including rapid emergence after the use of short-acting volatile anesthetics and the intrinsic characteristics of such agents. The objective of this study is to evaluate the incidence of ED in different anesthesia administration techniques. Material and methods: Retrospective analysis was done on the files from January to August 2015. We analyzed and recorded anesthesia administration techniques, recovery in post anesthesia care unit (PACU) and every emergency call from the PACU nurses was also recorded as well. Results: 877 children underwent surgical intervention in period of nine months. 177 children went unilateral inguinal hernia repair (aged 0 to 15 years, American Society of Anesthesiologist physical status I-II, male - female ratio / 129:48). According to Watcha scale, ED was present in 24,5% in the group from 0 to 5 years of age, 12,5% in the group from 5 to 10 years of age and neither one child 0% had ED in the group from 10 to 15 years of age. Maintenance with inhalation anesthesia was present in 63,7% of the youngest group, only 21, 9 % of the middle group and neither one child was maintained with inhalational anesthesia in the oldest group. Conclusion: Emergence delirium is much more likely to occur in pediatric patients between 2 and 5 years of age undergoing relatively painful procedures under inhalation anesthesia. Key wards: emergence delirium, inguinal hernia repair, inhalational anaesthesia.


Pediatric orthopedics – basic scientific grounds

Georgieva D. (1), Samardziski M. (1), Bozinovski Z. (1), Dzoleva-Tolevska R. (1), Georgiev A. (2), Kuzevska-Maneva K. (3), Gjurkova - Angelovska B. (3)

(1) University Clinic for Orthopedic Surgery, Clinical Center Mother Teresa- Skopje,UKIM - Skopje

(2) MIT University , Skopje, PHO Cardiology - Prima (3) University Clinic for Children’s hospital, Clinical Center Mother Teresa- Skopje, UKIM - Skopje

Orthopedic diseases are the leading cause for the exceptionally high invalidity, morbidity and mortality in the world. Little is known about the genetic origin of orthopedic diseases. In the world today there is a trend to establish bridges of cooperation for the “gap” which exists between the modern medical practice and modern science, hoping that by better understanding of the basic principles we can move towards more successful solutions of the problems in several diseases with orthopedic issues.

The aim is to present the genetic relation with the appearance of several orthopedic diseases. Polymorphisms and mutations in certain genes can be found in the greates number of orthopedic cases: Achondroplasia is connected with mutation in FGF Receptor 3; Osteogenesis imperfecta is autosomal dominant genetic disease with poor quality of type 1 collagen; Spondyloepiphyseal dysplasia congenita with COL2A1 gene mutation responsible for type II Collagen; Dystrophyo musculorum Duchenne with mutation in Dystrophin gene; Osteopetrosis with Carbonic anhydrase II deficiency syndrome; Marfan’s Syndrome connection with Fibrillin mutation. Friedrich’s ataxia is an autosomal recessive disorder that is accompanied by muscle weakness, cardiomyopathy and diabetes.

Conclusion: Identification of same gene polymorphism and mutations contribute to enlighten the pathophysiological phenomena of the orthopedic diseases. The development of PCR based methods and other sophisticated techniques guarantee that, in near future, the research of the defined genetic polymorphism and mutations will be used for early orthopedics disease diagnoses, more adequate therapy choice and their prognosis.

Key words: orthopedic diseases, genetics basic


Treatment of juvenile hallux valgus deformity

Georgieva D. (1), Samardziski M. (1), Bozinovski Z. (1), Dzoleva Tolevska R. (1), Georgiev A. (2), Kuzevska Maneva K. (3), Gjurkova - Angelovska B. (3)

(1) University Clinic for Orthopedic Surgery, Clinical Center Mother Teresa- Skopje,UKIM – Skopje

(2) MIT University , Skopje, PHO Cardiology - Prima (3) University Clinic for Children’s hospital, Clinical Center Mother Teresa- Skopje, UKIM - Skopje

Juvenile hallux valgus represents a complex progressive deformity of the front part of the foot, with the most distinguished malformation as lateral deviation of the toe. The prevention of deformity worsening can be done with conservative treatment, but it can’t be removed. The treatment of this deformity is surgical.

The aim of this study is to evaluate the choice of operative procedures for correction of juvenile hallux valgus deformity, in addition to the grade of deformity.

Material and methods: in the analysis there were included 27 patients: 35 feet clinically and radiological evaluated preoperatively.

Results: In 8 feet McBride operation was performed, 22 feet were treated with single ablation of the exostosis of the first metatarsal bone. Mitchell osteotomy was performed in 5 feet with distal soft tissue procedure.

Conclusion: within the right estimation of the deformity grade, the correct choice of the operative procedure for correction of juvenile hallux valgus was revealed.

Key words: juvenile hallux valgus, surgical treatment


Третман на пациенти со церебрална парализа

1 Георгиева Д., 1Темелковски З., 1Божиновски Ж., 1Самарџиски М. 1Џолева-Толевска Р., 2Георгиев А., 3Кузевска – Манева К.

1 ЈЗУ Универзитетска Клиника за Ортопедски болести, Клинички центар Мајка Тереза – Скопје, УКИМ – Скопје, Р. Македонија 2 ПЗУ Кардиологија Прима - Скопје, МИТ Универзитет, Р. Македонија 3 Универзитетска Клиника за детски болести, Клинички центар Мајка Тереза – Скопје, УКИМ – Скопје, Р. Македонија

Вовед: Церебралната парализа или детската церебрална одземеност означува состојба на мозочна дисфункција. Таа е хронична невролошка состојба која ја карактеризира непрогресивно нарушување на моторната функција, тонусот, положбата, координацијата и чувството за рамнотежа.

Cel: Целта на овој труд е да се прикаже важноста на ортопедијата во превенцијата, лекувањето и рехабилитацијата на лицата заболени од церебрална парализа.

Материјал и методи: Трудот се работеше при Универзитетската клиника за ортопедски болести, Медицински факултет, „Св. Кирил и Методиј“ во Скопје. Беа анализирани 92 пациенти со церебрална парализа. Пациентите беа лекувани со конзервативен и оперативен третман. Кај повеќето беа направени дополнителни предоперативни кардиолошки и неуролошки иследувања.

Резултати: Од вкупниот број анализирани (92) пациенти со церебрална парализа, 43 се лекуваа конзервативно, додека 49 пациенти се лекуваа со оперативен третман. Анализата на резултатите посочува дека оперативните зафати во комбинација со конзервативното лекување даваат надеж дека е можно дефинитивно или продолжено излекување со максимално можно сочувана функција на локомоторниот систем или во најтежок случај ублажување на симптоматологијата на оштетениот локомоторен систем.

Заклучок: Раната дијагноза и соодветната примена на konzervativen i оперативen tretman, овозможуваат подобро функционирање на pacientite со церебрална парализа преку максимално искористување на нивните преостанати способности.

Клучни зборови: церебрална парализа, ортопедија, третман.


Implementation of immunization schedule in albania.successes and pitfalls.

Gjeorgjina Kuli-Lito* *Professor in pediatric infectious diseases. UHC “Mother Teresa “ Tirana, Albania Background: Albania, involved in the program EPI of WHO since 1974, is among the countries with enriched immunization calendar, which is rigorously implemented in all health centers. Our national immunization program encompasses the vaccines of diphtheria, tetanus, pertussis, tuberculosis, poliomyelitis, haemophilusinfluenzae, hepatitis B, pneumococcus, measles, rubella and mumps. This study is being implemented in a generally favorable epidemiological situation: Hundreds of thousands of vaccines administered each year, decreasing morbidity of vaccine preventable infectious , large involvement of pediatric population into immunization system, enlargement and completion of vaccination calendar with new vaccines ,greater awareness of population regarding the effectivity of immunization, Increasing of technique and professional skills of health personnel. Despite the above mentioned success, problems or deficiencies regarding implementation of immunization calendar or eventually newly emerged epidemiological situations there are noticed in daily practice. Objectives: Assessment of current immunological profile in some pilot areas. Highlighting of firm and weak spots according to specifics of rural areas. Discussion and highlighting of problems, mistakes, encountered difficulties. Assessment of the level of knowledge and information of mothers and health staff regarding immunization. Materials and methods The study is a cross sectional prevalence study carried out in a time period of 6 months. There were included 3 pilot zones with different geographical distribution: Lezha, Berat and health center of Kamez. ( Tirana suburbs). Two pediatric age groups: 24-30 months and 7-8 years, were evaluated. Blood sample were collected and various data other than demographic ones were obtained through a specific questionnaire regarding immunization for doctors, nurses and mothers, which were filled in by respective interviewers. All the sera were tested for four vaccine antigens; tetanus, pertussis, measles and hepatitis B. Epidemiological indicators were also employed, as: Vaccine coverage, Antibodies’ positivity level, Antibodies’ negativity level. Laboratory kits ELISA test, were of “NOVA TEC. Imunodiagnostika GMBH” company were employed. Statistical analysis of the results was carried out through SPSS, “Windows” and method of T-test, nonparametric test for comparison between means: Mann Whitney-Wilcoxon, and Chi square were used. Results The vaccination coverage for the four types of antibodies, in total for each district, is shown as below

The vaccination coverage for the zone of Kamëz was found lower than other two districts. The protection to tetanus is 100% for the three districts. The number of children that should administer a booster dose of this vaccine is almost similar for Lezha and Berati (P=1. 0), while for the Health


Center of Kamëz, this number is larger (N=26), a difference also proved statistically (P=0. 013). The number of children that display a lasting immunity to pertussis is larger for the district of Berat, compared to children of district of Lezha and Kamëz (P=0. 036). The values of the coverage of the measles vaccine are lower for Kamëz (P=0. 036), than the other districts. The difference is not statistically significant regarding the vaccination coverage for Hepatitis B. A difference was also observed by the comparison of the values of antibodies titre for the three districts. By the comparison of the geometric mean was found that the titres of antibodies for the four vaccines are lower among the children ofKamëz(p<0.05) Conclusions The vaccination is an important domain of the public health which was evaluated and implemented in the districts included in the study.The basal vaccination is carried out rigorously, in all zones.The vaccination coverage for tetanus resulted 100% for the whole subgroup of the children tested, regardless age and zone.The positivity level of measles specific antibodies is found in satisfying values, 83% on average (R: 74-91. 5%)The vaccination coverage for Hepatitis B was over 71% (R: 62-87. 5%). Shortcomings regarding the correct implementation of the immunization scheme were noticed among the incoming children in the zones of Lezha and Kamëz.


Early neonatal sepsis in NICU

Bisera Gjorgjievska , Lidija Madzovska, Marija Jakimovska University Clinic for gynecology and obstetrics Neonatal intensive care unit (NICU) Objective: Neonatal sepsis is often and important reason for neonatal morbidity and mortality. Early neonatal sepsis have manifestation in the first 72 hours of birth and it is associated with maternal-fetal transmission: transplacental/haematogenous, ascendent, or aspiration/ingestion of bacteria from the amniotic fluid. Matherial and methods: The study included a group of 184 newborns with diagnosed early sepsis. We divided this birth cohort into term (n=19) who were transfered from Unit for transitory care to NICU, and preterm (n=165) admitted in NICU from delivery room. We analyzed maternal and neonatal risk factors, blood culture, laboratory investigations in the first 72 hours from delivery, and clinical signs. Results:

-Maternal risk factors included: Vaginal infection 25: GBS 7, Ureaplasma 6, Chlamydia 5, Enterococcus 3, Gardnerella vaginalis 3, Candida 3, E.coli 1, Mycoplasma 1. Urinary tract infection 16: Enterococcus 4, E.coli 4, GBS 1, bakteriurija 8 . Colpitis 25, Prolonged rupture of membranes >18h: 20, Blurred amniotic fluid : 25. Gemellar pregnancy 37 , IVF et ET 23. Uncontrolled or no evidence : others. - Neonatal risk factors : Preterm newborns 165 (89.6%) (<28gw)11, (28-31gw) 57, (32-33gw) 48, (34-36gw) 45. Term newborns 19 (10%). With + blood culture 45 (25%) - Pseudomonas 15, Staphylococcus 14, Klebsiella 12, GBS 2, Listeria 1. - Laboratory studies in the first 72 hours: Leucopenia (<5.000) 52 (28%), Leucocytosis (>25.000/48h->20,000) 45(24%), Neutropenia (<7,500) 44(23%), Neutrophilia (>14,500) 39 (21%), Thrombocytopenia (<150,000) 119 (65%), CRP (>5) 112 (61%), Anemia 72 (39%). There was a big percent of sensitivity and specificity of the Platelet and WBC count and high positive predictive value. CRP is not specific marker, but had high negative predictive value. - Other used laboratory parameters: ABS, electrolytes, bilirubin, glycemia - most often results were metabolic acidosis, direct hyperbilirubinemia, hypoglicemia, hypocalcemia. - Radiology proven Bronchopneumonia had 51 (27%) newborns. - Clinical signs: manifested on the skin, cardiovascular, gastrointestinal, central nervous system, respiratory, haematologic, renal system. - Among studied infants 60 (32,6%) had need of mechanical ventilation. Conclusion: Many risk factors were detected with regard to NICU admission. There was a big percent of uncontrolled pregnancies and pregnancies with infections which were associated with early neonatal sepsis. Effective interventions for these risk factors should be considered and implemented into the national health services to protect mothers and newborns from poor outcomes. In the diagnosis and treatment of early neonatal sepsis when blood culture was negative, laboratory studies had big significance and were effective guideline, but there was not enough specific markers in the decisions for antibiotic therapy independent of clinical signs.


Congenital heart disease ( Coarctation of the Aorta) Dr.J.Gjorgievska,dr.R.Kacarska,dr.K.Maneva,dr.B.Gjurkova,dr.V.Jovanovska,dr.M.Neskovska, dr.M.Xhaferi,dr.D.Nonkulovski,dr.I.Petkovska Ivanovska,dr.M.Kacarska,dr.G.Paskalov. Pediatric University Clinic – Skopje, Macedonija Department of Cardiology Background: The Coarctation of the aorta is a congenital condition with narrowing of the wall on the biggest blood vessel in the body (Aorta). Occurs two categories of coarctation depending on location and age of patients ( postductal-adults and preductal). The aim: The infant on 6 mounts old with congenital heart disease (Coarctation of the Aorta) Methods We were using those methods: -Anamnesis, Physical examination, EKG, Color-doppler echocardiography (2D). Case presentation: We are present infant 6 mounts old with dyspnea and cyanosis. First symptoms has been occurred one day before he came in hospital, manifesting with dyspnea. The day of hospitalization he was in severe general condition with tachydyspnea and cyanosis, requiring of medical help. After the hospitalization the patient was very suspicion for congenital heart disease due to we immediately made echocardiography.

Echocardiography examination showed dilatation of LA and LV but the kinetics was weakness without visible insufficiency. The arch of aorta with good lumen and SPG to 17mmHg in area on previous coarctation.

Picture N.1. EKG: sinus rhythm, tachycardia- HR 170/min, LV-hypertrophy. On the physical checking he was without femoral puls but with high blood pressure in the both legs, also was detecting low blood pressure on the arms. Results:The condition of patient was very emergency with heart insufficiency because of it was made surgical operation with used resection of the coarctation (FLAP). During the operation as wall as post operation period was not detected any complications. The patient left the department in better condition and he got recommendation and suggestion about life style and drugs (tbl. Alcadil, tbl. Dilacor, tbl Carvedilol, tbl. Lasix, tbl.Spirinolacton, cps. Omezol). In period of 6 mounts ago he has regular clinic monitoring with good generally condition. Control echocardiography showed: LV hypertrophy and good myocardial kinetics with regression of dilatation. Conclusion: For every patients with Coarctation of aorta we recommended diagnoses on the time because it is necessary about treatment. The patient with timely diagnosis have good prognosis which requiring of continuous cardiac monitoring.


Juvenile idiopathic arthritis modern therapeutical treatment

Beti Gjurkova Angelovska1, Rozana Kacarska1, Konstandina Kuzevska Maneva1, Valentina Jovanovska1, Marija Neshkovska Shumenkovska1, Daniela Georgieva2

(1)University clidren` s hospital, Skopje, Macedonia (2)University clinic for orthopedic surgery, Skopje, Macedonia ABSTRACT Juvenile idiopathic arthritis (JIA), a broad term that describes a clinically heterogeneous group of arthritides of unknown cause, begins before 16 year of age. Managment of the disease has greathly improved in recent years due to advances in pharmacologic treatment options, especially with new biologic agent.

In our review are presented four different clinical types of JIA: extensive oligoarthicularis,arthritis, enthesitis-related arthritis, systemic form of disease and oligoarthicular form with chronic inflamation of anterior eye segment.

For included 5 patients we described natural history of the disease, the number of affected joints, sistemical signs of disease and serological findings. For the patient with enthesitis-related arthritis was done HLA typing and HLA B27 gene was found, that is tipical result. For patients with systemic form and oligoarthicular form with eye affection were found high titers of antinuclear antibodies.

All patients were treated with protocols that are in giudelines of ILAR (International League of Association for Rheumatology). Patient with extensive oligoarthicular form and arthitis with enthesitis are on therapy with biological agents. The patients follow up in 14/ 18 month showed very good response (ACR Pedi 70 – 90 %).

The goal of the review is to show our therapeutic approach for different clinical types of JIA and first experience with new treatments using biological agents. Treatment of the children with JIA is challenging and complex.

Key words: juvenile idiopathic arthritis, biologic agents, treatment, tocilizumab


Pulmonary artery variations and associated cardiac defects in Tetralogy of Fallot

B.Gjurkova Angelovska1, R.Kacarska1, K.Kuzevska Maneva1, V.Jovanovska1, M.Neshkovska Shumenkovska1, V.Chadikovski2, M.Kacarska1, M.Dzaferi1, D.Nonkulovski1, Gj.Paskalov3, R.Muratovska1, A.Sofijanova1, I. Petkovska1, D. Georgieva4

(1)University clidren’ s hospital, Skopje, Macedonia (2)University hospital of pediatric surgery, Skopje, Macedonia (3)PZU “Paskalov”, Skopje, Macedonia (4)University clinic for orthopedic surgery, Skopje, Macedonia Aim: To determine pulmonary artery variations and other associated cardiac defects in patients with Tetralogy of Fallot (TOF). All patients with TOF were hospitalized in the University Clidren`s Hospital and University Hospital of Pediatric Surgery, Skopje, Macedonia from January 2012 to September 2015. Method and Materials: All 14 patients with TOF, who underwent two-dimensional and Doppler echocardiography were included. Two-dimensional and Doppler echocardiography can provide information about pulmonary artery abnormalities and associated cardiac defects in patients with TOF.

Results: A total 14 patients with TOF were examined. Pulmonary artery abnormalities were present in 6 (42.8%) patients. The commonest abnormality was isolated hypoplasia of main pulmonary artrery-2 patients, followed by isolated left pulmonary artery stenosis-1 patient, and supra-valvular stenosis in 1 patient. One patient had absent right pulmonary artery with segmental branch pulmonary arteries originating directly from main pulmonary artery and were underwent cardiac catheterisation and 64-clice CT. One patient had absent pulmonary valve and aneurismatic dilatation on the left branch. Associated cardiac lesions included right aortic arch in 2, patent ductus arteriosus in 4, and major aortopulmonary collateral arteries (MAPCA) in 2. Significant coronary artery abnormality was present in 1 patient.

Conclusion: Pulmonary artery abnormalities were present in 6 (42.8%) patients with TOF. Two-dimensional and Doppler echocardiography can provide information about pulmonary artery abnormalities and associated cardiac defects in patients with TOF.


Frequency of respiratory infections and use of antibiotics in their tretment in “pedijatrika-bambi”Strumica Makedonija

M.Gligorova,paediatrician PEDIJATRIKA-BAMBI

M.VitanovA PEDIJATRIKA-BAMBI

INTRODUCTION: Respiratory diseases are the most frequent reason for children visiting the pediatric dispensary. Irrational prescription of antibiotics in acute respiratory infections is not without effect but also leads to development of bacterial resistance to antibiotics, which is one of the growing problems wordwide.

OBJECTIVE: Indentify the frequency of respiratory infections and estimate how much antibiotics are prescribed in practice for their treatment.

METOD AND MATERIAL: Retrospective rewiew of the data from daily records in paediatric ordination Pedijatrika-Bambi in November 2014.

RESULTS: In the reviewed period a total of1272 children were examined. There were 642 first examinations (50.4%), 335 control examinations (26,3%), 98 short visits (7,7%) while there were 190 preventive examinations (14,9%). Out of the total number of examined children ,there were 535 (42,05%) children with respiratory disease. Out of total number of the first examination, 339 (63.3%) chldren had respiratory disease and out of a total number of control examinations, there were 196 (36,6%) children with respiratory disease. Distribution of the respiratory diseases according to the diagnoses is as follows:J00 : 102(19,0%); J01 : 1(0,18%); J02 : 80(14,9%); J03 : 105(19,5%) ; J04 : 59(11,0%); J20 : 125(23,3%); J18 : 10(1,8%); J21 : 12(2,24); J45 : 5(0,9%); J44.8 : 22(4,1%); J30.4 : 14(2,61%).

With regard to the age, the majority of children were 0-3 years of age : 242 (45,2%), followed by 4-7years of age : 198 (37,0%) ; 8-11years of age : 60 (11,2%); age 12-15 years : 23 (4,2%) and age 16-19 years : 12 (2,24%).

Out of the total number, antibiotics were prescribed to 160 (29,9%) children. Distrbution according to the age of children who were prescribed antibiotics was: 0-3 : 60children (37,5%; age4-7 : 53children (33,1%); age 8- 11 : 17children (10,6%); age 12-15 : 19 children (11,8%) and age 16-19 : 4 children (2,5%). With regard to the type of antibiotics : Amoxicillin to 90 children (56,2%); Cephalexin to 30 children (18,7%); Cefuroxime to 22 children (13,7%); Azytromicin to 10 children(6,2%) and Cefixime to 8 children (5%) .

CONCLUSION: Almost half of the children in the frst examination were children with acute respiratory infection and more than half of them in control examinations. Antibiotics were prescribed to 29,9% of children,equally at age 0-3 and 4-7. Antibiotics were mainly prescribed for acute tonsillitis and the most frequently prescribed antibiotics was Amoxicillin.


Cow s milk protein allergy

Stefanka Grujovska Cow's milk protein allergy (CMPA) affects from 2 to 4% of children, with the highest prevalence during the first year of age .About 50% of children have been shown to resolve CMPA within the first year of age, 80-90% within their fifth year CMP persist in only minority of children. Parents often put their children on unnecessary diet without an adequate medical and dietary supervision. These inappropriate dietary restrictions may provoke nutritional unbalances. CMPA can developed in exlusively and partially breast fed infants and when CMP is introduced into the feeding regimen. Therefore, an accurate diagnosis of CMPA is important in order to avoid not only the risk of rickets, ,anaemia, poor growth and hypoalbuminemia, but also that of immediate clinical reactions or severe chronic gastroenteropathy leading to malabsorption.CMPA may be imunoglobulinE or A positive atopic familiar history is common in children with suspected CMPA The diagnosis of CMPA is based on a detailed history of symptoms The risk of atopy increase if a parent or sibling has atopic disease (20-40% and 25-35% respectively). Exclusive breast feeding during the first 4–6 months of life reduces the risk for CMPA and most severe allergic manifestations during early infancy. The distinction between breast-fed and formula-fed infants reflects the importance of ensuring an adequate duration of breast feeding. The management of breast-fed infants depends on reducing the maternal allergen load and avoidance of CMP in supplementary feeding. Symptoms of CMPA occur within the first weeks after the introduction of CMP. Children with CMPA develop symptoms in at least two of the following systems: gastrointestinal (50–60%), skin (50–60%) and respiratory tract (20–30%). The symptoms can range from mild to moderate to severe. There are different algorithms for the diagnosis and management of CMPA in exclusively breastfed infants and in formula fed infants.


Spondilocostal Dysostosis and Congenital Scoliosis;

A Rare Disease Gives Insight in the Genetics of the Frequent One

Zoran Gucev, Natasa Cuckova, Velibor Tasic University Pediatric Clinic, Medical Faculty Skopje, Macedonia Background: The autosomal recessive forms of spondylocostal dysostosis SCD) are caused by mutations in the DLL3, HES7, MESP2 or LFNG genes. Patients, Methods, Results: The proband is a 12 years old boy with growth deficiency (>3 percentile). The trunk is shortened, the legs and arms are normal. Pectus carinatus and scoliosis are prominent. The intelligence is normal, corneas are not cloudy and sight and hearing are normal. X-rays showed multiple, generalised, ‘mild’ segmentation abnormalities throughout the spine. However, the ribs appear almost normal, perhaps slightly thicker, and there is possibly a point of fusion high on the left and posteriorly, but not at the origin of the ribs. Similar X-ray features are present in the father (44 years) and the uncle (46 years). The grandfather had the same clinical characteristics. Gene sequencing excluded the DLL3, HES7, MESP2, LFNG mutation in the proband. Whole exome sequencing, filtering, SNP analysis implicated a causative effect of a member of the Notch signalling pathway the TBX3 gene/protein. Functional analysis has further demonstrated lower enzymatic activity. It was thus demonstrated that the TBX3 gene alterations are causative in autosomal dominant SCD.

In addition, genetic analyses was performed in 161 sporadic congenital scoliosis (CS) patients, 166 controls, and two 16p11.2 deletion pedigrees using comparative genomic hybridization, quantitative PCR, and DNA sequencing. An additional 76 CS patients from PUMCH and a multi-center cohort of 42 subjects with 16p11.2 deletions were studied for replication.

A TBX6 compound inheritance model (one rare null mutation, often a deletion, plus one common hypomorphic allele) accounts for 7.9%-10.6% of CS in the studied populations, representing a significant CS locus. This provides mechanistic insights into CS and other complex traits, and facilitate molecular diagnosis of CS and deletion-associated genomic disorders.

Conclusions: Rare diseases are models in which not only their own molecular mechanism can be elucidated, but they also offer an outstanding opportunity to have a novel insight in the underlying mechanisms of frequent diseases. Those experiments of nature are a glimpse into “a riddle wrapped in mystery, inside an enigma”.


Incedence of congenital cardiopathy on our 10-years material

Guleva J., Ismaili B, Delovska-Stojkova V., Mihajlović-Dimovska B., Kalajdzieva-Zip M..,Islami- Pocesta B.,Muaremovska Sh.

Special hospital for Gynecology and Obstetrics “Mother Tereza” – Cair,Skopje,R Macedonia

Congenital cardiopathy are problem with whom the contemporary medicine is faced. Incidence of congenital cardiopathy is 5-8 on 1000 live births.

Target: showing the incidence of congenital cardiopathy diagnosed on our material in a period of last 10 years.(2005-2014)

Methodology: used are data from the DATA base from the Neonatology Department from Special hospital for gynecology and obstetrics “Mother Tereza” – Cair and data from echo-cardiac examinations performed on newborns in whom during their stay on the neonatology department the cardiac systolic murmur was present.

Results: additional echo-cardiac examinations on 3354 newborns were performed from total number of 30430 live births newborns in a period of 10 years due to present of systolic murmur. With echo examination were diagnosed congenital cardiopathy in 235 or 7 on 1000 live births newborns. VSD is present in 132(56,17%) newborns, and in combination with ASD is present in 21(8,94%), 12(5,10%) newborns whit ASD, CAV in 16(6,81%), Tetralogia Fallot in 11(4,68%) newborns, combinated cardiopathy are present in 10(4,25%), DAP in 8(3,40%), the both TGV and aortic stenosis are diagnosed in 6(2,55%)cases, the both stenosis AP and hypoplasia left ventricle are diagnosed in 4(1,70%) cases, Single ventricle, DORV, Truncus communis, Monoatrium and pulmonary venous anomalies, all of them are present in only 1(0,43%) case.

Discussion and conclusion: With an analysis of the performed echo examination we have found that the incidence of congenital cardiophaty on our material is 7‰ The incidence of cardiophaty is most large in VSD diagnosed in 56,17%. This data is important due to the fact that there is a possibility for early detection of the congenital cardiac defects, regular control and their surgical treatment. Congenital cardiopathy diagnosed on our material are present in percentiles which are matched with the literature data, fact which shows good healthcare on the newborns on our department.


D - Vitamini Guner Karetekin

Vitamin D -t he sunshine vitamin- is essential for optimal health from conception to death for human being . About 3 percent of the human/mouse genome is under the control of vitamin D. This show that Vitamin D plays a crucial role for optimal health throughout life. A severe vitamin D deficiency [25(OH)D<10 ng/mL] results in rickets in children and osteomalacia in adults. Also vitamin D deficiency exacerbate osteopenia and osteoporosis in adults, which has root in infancy. In addition to its role in calcium and bone homeostasis, vitamin D potentially regulates many other cellular functions (cell differentiation, immunomodulation, and cell growth). Epidemiological studies shown that poor vitamin D status has been associated with several common diseases, including rickets and other bone diseases, diabetes, cardiovascular diseases, autoimmune diseases, infectious diseases, tuberculosis, and cancer. Maintaining optimal concentrations decreases risks and severities of autoimmune diseases, cardiovascular disease, many types of cancer, dementia, types 1 and 2 diabetes mellitus, and respiratory tract infections. Other benefits include improved dental and oral health and improved physical performance. Epidemiologic studies highlight the increasing prevalence of vitamin D deficiency and insufficiency and its association with an increased risk of autoimmune diseases and poor respiratory function


Expressions of oxidative injury markers in preterm infants during the first postnatal week

Liljana Guržanova-Durnev1, Elizabeta Zisovska2, Dragica Zendelovska3, Jasmina Dimitrova-Shumkovska4

1University Children's Clinic, State Hospital, Skopje, R. Macedonia

2University Clinic for Gynecology and Obstetrics, State Hospital, Skopje, R. Macedonia 3Institute of Preclinical and Clinical Pharmacology and Toxicology, Faculty of Medicine,

"Ss. Cyril and Methodius" University - Skopje, R. Macedonia 4Institute of Biology, Faculty of Natural Sciences and Mathematics, "Ss. Cyril and Methodius"

University, Skopje, R. Macedonia

Background: Oxygen free radical injury is implicated as a major factor in the pathogenesis of the long term complications seen in premature infant. The purpose of this study was to determine whether protein and lipid oxidation products can serves as useful markers for assessing oxidative stress in preterm babies who subsequently developed chronic lung disease, with immature lungs being particularly sensitive to the injurious effect of oxygen and mechanical ventilation

Material and Methods: Мarkers of potential oxidative risk: protein carbonyls and malondialdehyde (MDA) and antioxidant activity of glutathione peroxidase (GPx) were measured in blood samples, obtained at 1, 3 and 7 days from 104 preterm babies divided in: control group (healthy preterm babies, n=59) and CLD group (preterm babies who subsequently developed CLD, n=45).

Results: Evaluation of the results, during the first 7 days of life, showed permanent rise in serum concentrations of MDA (p<0.001) and protein carbonyls (days 1–3, p=0.044 and 4–7, p<0.001) accompanied by a simultaneous significant decrease in the erythrocyte activity of GPx (days 1–3, p=0.028 and 4–7, p<0.001). CLD group had significantly higher MDA concentrations than controls at day 3 (93%, p<0.001) with highest registered level at day 7 (4.55±0.92 nmol/ml, p<0.001) and significantly higher protein carbonyls at any time (p<0.001). GPx activity was significantly lower in CLD group at day 7 (54 %, p<0,001) compared with controls. A strong negative correlation was found between MDA and protein carbonyls with GPx activity (r=0.727, p<0.001 and r=0.736, p<0.001, respectively) in the CLD group.

Conclusion: Oxidatively modified molecules are appropriate for assessing oxidative stress in vivo. The presence of oxidant/antioxidant imbalance during the first days of life provides evidence that increased oxidative stress plays a role in the development of chronic lung disease (CLD).

Key words: Oxidative stress, Glutathione peroxidase, Malondialdehyde, Protein carbonyls


Interleukin-6andinterleukin-8 as predictors of chronic lung disease in premature infant

Liljana Guržanova-Durnev1, Elizabeta Zisovska2, Dragica Zendelovska3

1University Children's Clinic, State Hospital, Skopje, R. Macedonia 2University Clinic for Gynecology and Obstetrics, State Hospital, Skopje, R. Macedonia

3Institute of Preclinical and Clinical Pharmacology and Toxicology, Faculty of Medicine, "Ss. Cyril and Methodius" University - Skopje, R. Macedonia

Background:Improved survival from advances in neonatal care has resulted inan increased number of infants at risk for chronic lung disease (CLD). Pro-inflammatory cytokines such asinterleukins (IL)-6 and (IL)-8 play an importantrole in the inflammatory response to neonatal airway injury.In order to predict the late-development of CLD, cytokines in the blood samples were assessed in this study.

Materials and Methods:IL6 and IL8 were measured in 45infants developing CLD in blood

samples obtainedat 24 hours, 72 hoursand 1 week ofage. The normal range was established in blood samples of 59 consecutivehealthy pre-term infants. Cytokineconcentrations were quantified in duplicate using commerciallyavailable sandwich ELISA kits.

Results:High initial levels of IL6 and IL8(first 24 h postnatal age) obtained in

infantsdeveloping CLDindicate that this cytokines are not only markers but also initiators of inflammatory reaction. A persistent significant elevation of IL8 concentration in CLDgroup results with double increased levels at the end of the first week of life (799.2±122.8pg/ml). The obtained valuesshowed significantnegative correlation with gestational ageofthe newborns (IL6, r= -0.751 and IL8, r= -0.659; p<0.001) andsignificant positive correlation to duration of mechanical ventilation(IL6, r=0.837and IL8, r=0.704; p<0.001).

Conclusion:The determination of pro-inflammatory cytokines in blood can be usefulin

identifying babies at high risk for CLD and in devising newstrategies to ameliorate perinatal outcome.

Key words: Chronic lung disease, Interleukin-6, Interleukin-8


Angelie EIS System - Electrical Impedance Segmentography

Herbert Murlasits Vienna. Austria The Angelie EIS System is a great supporting tool for respiratory management, specially designed for Clinical use in Intensive Care. It’s a noninvasively and radiation free technology which provides real time monitoring of conditions of lung. With the 5 ECG electrodes in front and in the back we are getting the impedance which is in indirect relation to air flow in the 4 quadrants of the lungs. Angelie calibrates automatically the needed power for different type of patient,s for this reason you can use for Neonates – Paediatric and Adult. Distribution of Tidal Volume in ml, Compliance, right PEEP setting, appropriate suctioning and right ET positioning are getting shown in colours and easily understandable. This new method and device is in complete correlation to x-Ray and CT scans. You can compare different modes of ventilation and advantage of pulsatile BiLevel ventilation. The easy way of handling and communication with PC and storage on USB stick makes it an outstanding tool for all ventilated patients.


Epidemiological features of bronchiolitis in the Pediatric Department within General Hospital Kumanovo

Aleksandra Hristova, Gabriela Tavcioska General Hospital Kumanovo, General Hospital Prilep Abstract Objective: To present the Epidemiological features of bronchiolitis in a one-year period in patients of Pediatric Department within General Hospital Kumanovo Introduction: bronchiolitis is the most common respiratory infection in early infant age and one of the most common reasons for hospitalization of children under the age of 6 months. The disease is characterized by seasonal occurrence with prevalence in the winter season November – March. Patients and Methods: The history of the disease was analyzed within 58 patients treated because of bronchiolitis throughout the interval February 2013 till February 2014 Findings: Most of the hospitalized patients were under the age of six months. As for the monthly prevalence of the disease, highest incidence was registered in January and February. Approximately 50% of the patients were with risk factor for occurrence of bronchiolitis, with the following distribution: almost 51.7% were with risk factor artificial diet; 1 patient with congenital heart anomaly and twins with birth weight lower than 2500 grams. One patient required mechanical ventilation and his treatment was forwarded at IBZ Kozle. 25.8% of the patients were serologically positive on respiratory syncytial virus (RSV) Conslusion: Bronchiolitis is the most common respiratory infection in early infant age and one of the most common reasons for hospitalization of children under the age of 6 months with peak incidence in January and February. Key words: bronchiolitis, infant, epidemiology


Diagnostic indicators in neonates suspected for early neonatal infection

B.Islami-Pocesta B.Ismaili V.DelovskaJ.GulevaM.TrajchevskiB.Mihajlovic

M.Zip M.Jegeni I. Zulfiu I. Shurdhani M .Nedzhipi A. Islami Muaremovska Sh.

SHGO “ Mother Teresa” Skopje

Early diagnosis and treatment of neonates suspected of early infection is essential in preventing serious life threatening complications.

Objective: We have analyzed the risky clinical and laboratory parameters (tot.nr. of LE, CRP, PCT) in neonates suspected of early infection and / or sepsis compared with a control group of healthy neonates.

Materials and Methods:We used the data from the database of the department of neonatology in SHGOMother Teresa Skopje. It is a retrospective analysis, the respondents were selected according to a pre-established inclusion and exclusion criteria according to the recommendations of the CDC and Prevention.InSHGO “ Mother Teresa” Skopje in year 2014god. There were 3241 live born neonates, from which 3083 (95.12%) were in term, 351 (10.8%) were SGA and 158 We analyzed 111 neonates with suspected connate infection with g.w. 38,1 ± 1,3 (x ± SD), withbirth weight of 3100 ± 320 (X ± SD)Apgar Score -8,1 ± 1,3 in 1min (x ± SD) and Apgar Score -8,7 ± 1,3 in 5 min x_SD. Spontaneous deliveries were 78 (70.27%) with Caesarian section 33 (29.72%).Premature ruptured membranes 18 (16.2%). From the analysed cases 79 respondents (71.1%) were with clinical signs of early infection and according to theprevious criteria, were evaluated and clinically observed .The average value of CRP waswas 18,2 ± 11,7 ( 5,2 ± 43,1) in the first 24 h, and subsequently.The analysis of the predictive parameters in the infected compared to the healthy control group neonates showed high significance of differences.Predictive parameters for early neonatal infection were in direct correlation with the presented clinical features.In our study, 31( 27.9%)neonates were asymptomatic at birth, but with maternal intrapartal risk factors. Of these, 11 (9.90%) developed symptoms suggesting infection in the first 24h.

Conclusion:The evaluation and conduct of symptomatic neonates is not controversial and requires an early treatment.The asymptomatic neonates of mothers with intrapartal risk factors are a problem for early decision on diagnostic evaluation and treatment of infection. However, in unclear casesif the earlyinfection is sub diagnosed and a decision for early treatment has not been made, the neonates suffer.


Is there a place for ctx with cystic fibrosis

Tatjana Jakovska1, Jasmina Mecevska-Jovcevska2, Stojka Fustik1, Tatjana Zorcec1, L.Spireska1

1University Children’s Hospital, Medical Faculty, Ss. Cyril and Methodius University, Skopje, Macedonia 2University Clinic for clinical biochemistry, Skopje, Macedonia

Background: Imbalance between bone formation and degradation in cystic fibrosis (CF) has become an important issue for developing osteopenia. Bone turnover markers in CF patients indicate that they suffer from hyperresoption and an inadequate compensation in bone formation even when clinically stable. Bone turnover during growth may be of value in the identification of individuals who may be at risk for osteoporosis later in life. High plasma levels of C-terminal telopeptide of type 1collagen (CTX) reflects raised osteoclast activity. This study analyzed CTX levels alongside BMD in patients with CF.

Method: CTX levels ng/ml (0, 3 - 0, 5) were assessed at annual screening. BMD was measured by dual energy x-ray absorptiometry (DXA) scans with spinal scores recorded. Reduced BMD scores were categorized as osteopenic and osteoporotic values.

Results: The study included 80 CF patients who were divided in 3 groups depending Z or T score of bone mineral density (BMD). 55CF patients had normal BMD (NBMD), 17osteopenic and 8 osteoporotic values. Mean values for CTX (ng/ml) were for NBMD 1.24±0.74, for osteopenic 1.24±0.84 and for osteoporotic 1.05±0.48 . Raised CTX levels were seen amongst 34 (62%) with NBMD, 10 (59%) with osteopenia and 4 osteoporotic (50%) respectively and greatest with osteoporotic compared with NBMD and osteopenic groups.

Conclusions: The majority of patients (60%) had raised CTX values, suggesting raised osteoclastic activity. Bisphosphonates may have good effect on bone status. Bone turnover markers such as CTX may be valuable in selecting and monitoring efficacy of bone treatments.


Vitamin D influence on respiratory exacerbations in cystic fibrosis patients

Tatjana Jakovska

University Pediatric Clinic, CF Center, Skopje, R.Macedonia

Objective: Cystic fibrosis (CF) is highly associated with vitamin D deficiency. In 2012 at guidelines- committee of the CF Foundation published new guidelines for screening, diagnosis, management and treatment of vitamin D deficiency in individuals with CF. The objective of the study was to assess the efficacy of the new guidelines, as well as to test the correlations between vitamin D levels and pulmonary function and exacerbations.

Methods: The study included 80 clinically stable CF patients (range 5-36 y), who regularly attended CF center at the Pediatric Clinic in Skopje, Macedonia. Serum osteocalcin (OC), β cross laps, 25OHD and PTH were determined by ELISA assays. BMD was measured via dual energy-ray absorptiometry (DEXA) scans with spinal scores recorded.

Results: 50 % of the CF patients with PI had serum vitamin D >20 ng (range 10-45ng/ml) with no difference of age. Osteopenia was determined in 35 % of patients. High plasma β cross laps values reflects raised osteoclast activity in 50% of patients with osteopenia. We found 5 CF patients (16.6%) with 25OHD <10 ng /ml. CF patients with vitamin D insufficiency presented significant correlation with T/V(r=0.9); Schwachman score (r=0.95) and FEV1(r=0.91).

Conclusion: The levels of vitamin D in our CF patients are lower than optimal levels despite daily supplementation with 800 IU. The new guidelines for management of vitamin D deficiency appear to improve vitamin D levels in individuals with CF. Imroprovement in in vitamin D levels in individuals with CF appears to decrease both respiratory exacerbations and number of days of hospitalization per year.


Hemophagocytic lymphohistiocytosis

Janic D

Hemophagocytic lymphohistiocytosis (HLH) is a disorder characterized by excessive activation of macrophages that secrete large amounts of pro-inflammatory cytokines. The main features of HLH are fever, hepatosplenomegaly and bi- or pancytopenia. Hemophagocytosis itself may or may not be present in the bone marrow or lymph nodes. HLH can occur as a primary (genetic) disorder or as secondary to infections, autoimmune diseases, and malignancies. Primary HLH may be familial (associated with mutations in PRF1, UNC13D, STX11 or STXBP2 gene) or part of certain syndromes (X-linked lymphoproliferative syndrome, Griscelli syndrome type 2, Chediak-Higashi syndrome). The basis of HLH appears to be a defect of cellular cytotoxicity (impaired degranulation of CD8+ T lymphocytes and/or natural killer cells). Combined with an appropriate trigger, this causes prolonged activation of the immune system, ultimately resuling in the cytokine storm effected by macrophages. A fairly reliable marker of this condition is the plasma level of soluble interleukin-2 receptor (sIL-2R). According to the HLH-2004 Protocol devised by the Histiocytic Society, the diagnosis of HLH is established either by a known gene defect or the fulfillment of 5 of 8 criteria (fever, hepatosplenomegaly, bi/pancytopenia, hemophagocytosis hypertriglyceridemia and/or hypofibrinogenemia, hyperferritinemia, impaired cytotoxic cell degranulation, elevated sIL-2R). Treatment is immunosuppression based on dexamethasone, cyclosporin and etoposide. For primary HLH, curative treatment consists of hematopoetic stem cell transplantation. Since HLH is an acute life-threatening disorder, early diagnosis is esssential. We hereby present a total of 31 patients treated of HLH in our hospital in the period 2005-August 2015.


Biological aspects of asd

Jotovska Olga, Institute for mental health of children and youth, Zdr. Dom, Skopje

Introduction: Autism is a high variable disorder during neurological development which is characterized with damage of social interaction, verbal and nonverbal communication, weak interest for surroundings and restrictive, repetitive and stereotypic behavior.

Aim of the presentation: to show the recent investigations and their results about possible reasons of ASD.

Methods and materials: Many articles and books with recent information about this topic are selected processed and analyzed. The literatures mainly belong to American, English, French, Holland and Scandinavian resources. Most of them are neuropathologic, neuroimaging, neurophysiologic studies, genetic analyses with high-throughput-sequencing, biopsies of digestive system, comparisons between children who were vaccinated with vaccines with timerosal and vaccines without timerosal. There are also studies about mitochondrial dysfunction, oxidative stress and immunologic involvement ASD.

Results : investigations suggest at first genetic, neurodevelopmental than immunologic, gastrointestinal origin of ASD. The role of environmental factor nowadays is very controversial, although many researches consider that these factors contribute to occurrence of ASD. Many debates and reports about the influence of vaccines are also presented.

Conclusions : Autism is complex disorder with number forms of expressions and potentially many types of causes which are not completely understood.

Important questions about autism are still enigma. Understanding of autism biology is crucial for development of better ways for it’s diagnosis, as well as treatment.


Prediction of Outcome by Prednisone Response in Childhood Acute Lymphoblastic Leukemia

Aleksandra Jovanovska, Sofijanka Glamocanin, Kata Martinova, Svetlana Kocheva, Biljana Coneska-Jovanova, Zorica Antevska-Trajkova

Department of Hematology and Oncology, University Pediatric Clinic, Skopje, Republic of Macedonia

Introduction: In the ALL-BFM studies for treatment of acute lymphoblastic leukemia, reduction of leukemic blasts in peripheral blood after a one week prednisone pre-phase – the so called prednisone response – has been used for risk stratification since 1980s and has been one of the most relevant factors for identification of high risk patients.

Materials and Methods: We retrospectively evaluated prednisone response and analyzed its prognostic value in comparison to other established prognostic factors in 88 pediatric patients with newly diagnosed acute lymphoblastic leukemia, treated at the University Pediatric Clinic, Department of Hematology and Oncology in Skopje from 2003 to 2011.

Results: Prednisone good response (PGR) (<1000 blasts/mm3 in peripheral blood) was detected in 79 (89,8%) patients, and prednisone poor response (PPR) (>1000 blasts/mm3 in peripheral blood) in 9 (10,2%) patients. We found that PGR at day 8 of Induction therapy is an independent, clinically significant predictor of superior event – free survival (EFS) and overall survival (OS). Patients with PGR had an excellent outcome with 5 year EFS of 92,3% ± 0,03 versus 33,3% ± 0,157 for patients with PPR (HR 10,8 P<0,001). Also patients with PGR had superior 5 year OS of 93,5% ± 0,03 versus 44,4% ± 0,17 (HR 10,3 P<0,001). In multivariate analysis, the prednisone response remained an independent prognostic factor for EFS (HR 9,3 P=0,003) and OS (HR 7,2 P=0,015) after adjusting for age and initial white blood cell count.

Conclusion: These data suggest that prednisone response is still one of the most effective risk factor to predict survival in pediatric acute lymphoblastic leukemia.


Childhood Acute Lymphoblastic Leukemia in Macedonia: Prognostic Factors and Treatment Outcome

Aleksandra Jovanovska, Kata Martinova, Svetlana Kocheva, Biljana Coneska-Jovanova, Zorica Antevska-Trajkova

Department of Hematology and Oncology, University Pediatric Clinic, Skopje, Republic of Macedonia

The presenting features and treatment outcome for 96 Macedonian children, aged 1-14 years with newly diagnosed acute lymphoblastic leukemia, admitted at University Pediatric Clinic (Department of Hematology and Oncology), in Skopje, Macedonia between 2003 and 2011 were evaluated to determine their prognostic significance. The relationship of each presenting feature to treatment outcome was analyzed by the Fisher's exact test or chi-square test. The median age was 6 years, and the male to female ratio was 1:1. 15% of cases presented with high initial WBC (>50x109/L). Central nervous system involvement was detected in 6 patients (6.2%) and 14 patients (14.5%) presented with mediastinal mass. Immunophenotype was performed in 73 children. Precursor B cell ALL was confirmed in 62 (85%), and T cell ALL in 11 (15%) patients. The most of the patients achieved complete remission (94.8%). There were 10 remission deaths (10.41%). Mean follow-up was 66.4 months. Relapse was recorded in 9.37% (9 cases). 76 patients still live in continuous complete remission. For the entire cohort 5 years EFS was 83.9%±0,04. The prognostic factors adversely affecting treatment outcome were age (P<0,03), initial WBC (P<0,01), CNS involvement (P<0,001) and mediastinal mass (P<0,001). Sex and immunophenotype didn’t show any significant effect on treatment outcome. Among all variables a worse prognosis was associated with older age, WBC counts greater than 50,000/ml at presentation, CNS involvement and mediastinal mass. Our analysis of Macedonian children is similar to that on previous studies in other populations.


Interventional cardiology in congenital heart defects: Our experience

Jovanovska V.¹, Morgan G.², Kacarska R¹, Kuzevska Maneva K.¹, Spirovski I.³, Gjurkova Angelovska B.¹, Neshkovska Shumenkovska M.¹, Nonkulovski D.¹

¹University Children’s Hospital, Skopje

²Evelina London Children’s Hospital

³University Clinic of Cardiology, Skopje

The role of the interventional cardiology is crucial, whether in diagnostic, follow up after some surgical repairs or for treatment of the congenital heart defects. With the development of the techniques, technologies, equipments and the personal skills, many heart conditions previously treated surgically can now be solved with interventional procedures.

We are presenting our experience within the National project for the development of the neonatal and pediatric cardiac surgery.

Aim and methods: In our 2 year’s experience so far, 17 children underwent cardiac catheterization. They were diagnosed by anamnesis, clinical presentation, examination and echocardiography. 7 underwent diagnostic procedure before surgical repair (TCPC, Glen anastomosis, full diagnostic in some complex cardiopathies), 9 children underwent interventional procedures: ASD II closure in 5, PDA closure in 1, balloon dilatation in 3 neonates with critical PV stenosis and closure of the multiple bilateral pulmonary arteriovenous fistula in 1 child.

Results: 16 of the children had no complications, 1 needed surgery because of the ASD device displacement during procedure. 8 children are cured after procedure, they only need follow-up. 2 children with ASD needed surgery. All 7 children with diagnostic catheterization were adequately prepared for further surgical treatment.

Conclusion: cardiac catheterization is necessary for diagnostic, in decision making for cardiac surgery and in treatment for many congenital heart defects. With further development many of the children who previously needed open heart surgery will be treated with the minimal invasive interventional procedures.


Incidence of the congital malformation (cm) in the uro-genital system, compared to the incidence in other systems

Sh Jusufi1, M Kalajdzieva-Zip1, J Guleva1, B Mihajlovic1, V Delovska1, T Pensovska1, B Islami-Pocesta1.

1Department of Neonatology, Special Hospital for Gynecology and Obstetrics “Mother Teresa” - Cair, Skopje, Macedonia Background and Aims: Congenital malformations (CM) are defined as abnormal structure of the organism resulting from disrupted embryogenesis. Many factors influence the appearance of CM. Regarding different criteria and authors, the incidence of CM at newborns is between 2 – 7%. The aim of this paper was to determine the incidence of CM at newborns delivered at SHGO “Mother Teresa” - Cair, Skopje, during the five years period (2005-2009). Also, the distribution among organ system had been analyzed. The purpose of this paper is to present the incidence of CM uro-gonital sistem, compared with the incidence in other system.

Methods: A retrospective analysis of 15293 newborns delivered at SHGO Cair, during 2005-2009, has been performed. Database (Access 2005) from Neonatal Unit has been used. The incidence and percentage of CM among different systems have been determined.

Results: Among a total of 15293 newborns, 573 or 3, 75% have had CM. the incidence between different years was: 4, 02% in 2005, 3, 42%-2006, 3, 22%- 2007, 4, 23%-2008 and 3, 85% in 2009. Regarding different organ systems the distribution was: 40,31% of all CM were the anomalies of musculoskeletal system, 21,81 % - of cardiovascular system, 20,07% - anomalies of uro-genital system, 12,04% -CM of gastro –intestinal system, 5,76% of CNS, etc.

Conclusions: During the five years period, the incidence of CM is 3, 75%, which is in accordance with data from literature. Among years, the incidence varies from 3, 2 to 4, 3%. The anomalies of musculoskeletal system are the most frequent, followed by those from cardiovascular, uro-genital, gastro-intestinal and central nervous system. CM Incidence of uro-genital system , compared with other systems presenting occupies third place. This presents a serious problem, that requires a commitment as a team in prenatal and postnatal period. Congenital malformations remains an important medical and social demands serious commitment throughout the country, both in the medical, socio-economic, ecological, etc.


Critical Aortic Valve Stenosis in Neonates - Case report

M. Kacarska, R.Kacarska, K.Kuzevska - Maneva, B. Gjurkova, V.Jovanovska, M. Neshkovska - Shumenkovska, M. Xhaferi, D. Nonkulovski, J. Gjorgievska, I. Ivanovska - Petkovska,

Department of Cardiology- University Children`s Hospital, Skopje, Macedonia

Background and aims: Aortic valve stenosis refers to a condition that causes obstruction to blood flow between the left ventricle and the aorta. A decreased valvular area causes increased pressure gradient, and these parameters are used to classify and grade the aortic stenosis as mild, moderate or severe.

Methods: We present a case of a one month - old male patient who was admitted to Pediatric Clinic in Skopje from Struga with heart failure and systolic ejection murmur after second day of birth, for detailed cardiac investigation. Echo examinations was established following diagnoses: Ductus arteriosus persistens, Dysplastic Bicuspid aortic valve with pressure gradient across the valve of 115 mm /Hg (Mean gradient 75 mmHg), aortic ring - 7 mm, descending part with stenosis of 4 mm and turbulent blood flow, EF - 71%, left ventricle hypertrophy and endocardial fibroelastosis. CT-scan result showed aortic valve stenosis and post stenotic dilatation.

Results: According to the diagnoses patient was transported and surgical intervened in Sofia – R. Bulgaria with this interventions: Aortic Valve Commissurotomy and PDA ligation. After commissurotmy pressure gradient across the aortic valve was 35 mm /Hg (Mean gradient 15 mmHg).

Conclusions: Open commissurotomy for critical aortic stenosis in neonates carries a low operative risk and provides lengthy freedom from recurrent stenosis or regurgitation. Reoperations are inevitable, but aortic valve replacement can be delayed until the implantation of an adult-sized prosthesis is possible.

Keywords: Aortic valve stenosis; Aortic Valve Commissurotomy;


Tetralogy of Fallot With Absent Pulmonary Valve

R.Kacarska, K.Kuzevska - Maneva, B. Gjurkova, V.Jovanovska, M. Neshkovska - Shumenkovska, M. Kacarska, M. Xhaferi, D. Nonkulovski, J. Gjorgievska, I. Ivanovska - Petkovska, Department of Cardiology- University Children`s Hospital, Skopje, Macedonia

Background and aims: Tetralogy of Fallot (TOF) with absent pulmonary valve is a rare congenital anomaly characterized by features of tetralogy of Fallot with either rudimentary ridges or the complete absence of pulmonic valve tissue. Congenital absence of the pulmonary valve with an intact ventricular septum occurs, but this is much less common. The absence of mature pulmonary valve tissue leads to severe pulmonary regurgitation, which is often associated with massive dilatation of the pulmonary arteries and which is characteristic of this syndrome.

Material and methods: In our study we report a two cases which wear diagnosed prenatal which present in association with a ventricular septal defect, tetralogy of Fallot, respiratory problems and cyanosis. We therefore performed detailed echocardiography, cardiac catheterization, CT angiography.

Discussion and results: The patients have a grave prognosis as there was absence of the ductus arteriosus and a large VSD, which had resulted in pressure changes in both the ventricles with severe pulmonary arterial dilatation and early cardiac failure.

Conclusion: The TOF with absent pulmonary valve is a severe malformation, the prognosis is poor and highly dependent on associated malformations.


Агресивноста и Хиперактивноста кај деца со аутизам

Автор: проф. д-р Мирјана Ќаева-Пејковска

Завод за ментално здравје на деца и младинци „Младост“ Скопје

Вовед: Агресивноста и хиперкинетскиот синдром или растројство поради нарушено внимание (АДХД) се јавува во сите возрасти во развојниот период. Во трудот се обработуваат хиперактивноста и агресивноста кај деца со аутистичен спектар (АС) и се посочуваат искуства од примена на можни третмани.

Цели: Да се посочат причините, поврзаноста и третманот на пациенти со АС кај кои се изразува синдормот АДХД.

Материјал и методи: Испитувани се 124 пациенти на возраст до 26 години, кои во еден подолг период се третирани во Заводот за ментално здравје на деца и младинци „Младост“-Скопје. Со примена на повеќе методи на инвестигација, се согледани карактеристиките на проучуваната појава, нејзиното изразување и видовите растројства кај деца со аутизам. Се систематизираат причинителите за хиперактивноста и агресивноста кај децата и се претставуваат симптомите и факторите кои можат да го предиспонираат дете со аутизам да го развие ова нарушување.

Резултати: Повеќе од половина од децата со АДХД имаат АС. Хиперактивитетот може да биде симптоматска состојба на множество синдроми со генетска и негенетска етиологија. Семејно фамилијаните истражувања, како и проучувањата на близнаци, упатуваат на ризик за повторно појавување кај роднини од прво колено во сигнификантен обем. Се посочуваат и други причини и ризик фактори: мозочни структури, неуротрансмитери, медицински состојби, нарушувања во учењето, девијантни нарушувања и друго.

Заклучок: Со класификацијата на факторите се оформуваат сознанијата за причинителите на хиперактивноста и агресивноста кај деца со аутизам. Додека основните компоненти на АДХД и АС се различни, има некои кои се преклопуваат во симптомите. Најважно за диференцирање на двете состојби е да се утврди кои функции или развојни компоненти се блокирани или исчезнати, а со тоа се предизвикуваат овие симптоми. Од тоа зависат видовите третман на овие деца и последиците кои се изразуваат доколку отсуствува третманот. Се упатуваат препораки до родителите и наставниците за препознавање на проблемот, негово разбирање, приспособување, третман и тераписки постапки со овие деца.

Клучни зборови: хиперактивност, агресивност, деца со аутизам, третман


Vitamin K1 Deficiency

Kanevce-Paskali B., Papazovska A., Aluloska N., Paskali F. JZU General Hospital, department od Gynecology and Obstetrics, Ohrid Abstract

Vitamin K1 deficiency can be find in infants, children and adults. Can be find in first days of life (because of insufficient synthesis in bowel), disturbed intestinal intake (obstruction of the biliary system ), therapeutic or nutritional deficiency. Bacteria from the bowel synthesize significant portion of vitamin K needs, because of inadequate colonization of the bacteria in the first days (5-7 days) can be risk for Vitamin K Deficiency.

Introduction

Vitamin K1 Deficiency without bleeding can be find in 50% of infants less than 5 days and in 0,25-1,7% infants with classic hemorrhagic disease. Because of that Committee on Nutrition of the American Academy of Pediatrics recommended to be

Administered 0,5-1mg Vitamin K of all newborns immediately after birth.

Material and methods

Case report: Second child from the second row risk pregnancy, controlled. Born in 37 g.w. with section caesarea. Birthweight 3190gr, birth length 51cm, Apgar score 8/8. Cried immediately and it was not cyanotic. Lactation is not established in the maternity hospital. No manifested physiological icterus. After 12 hours old because of two heavy faeces colored cherry who were positive for benzedin sample, the infant was transported to Children's Clinic Department Neonatology. There were made pallet of laboratory and imidzhig investigations. Consulted a pediatric surgeon which exclude acute surgical abdominal zaboluvanje.Realiziran screening hemostasis with finding in addition to vitamin K deficiency therefore the newborn receives plasma and vitamin K. After this terapy an improvement in the general situation without new bloody faeces.

Conclusion

Because of this appearance of Vitamin K1 Deficiency in first 5-7 days, for all newborns are administered 1 mg vitamin K immediately after birth and recommended consumption of mother milk.


Respiratory syncytial virus infections and prevention with Palivizumab Kareva L, Mironska K, Stavrik K, HasaniA Clinic for childrens deseases,Skopje, Macedonia Department of immunology Respiratory syncytial virus (RSV) is a single-stranded RNA virus of the family Paramyxoviridae, subfamily Pneumovirinae. Its name comes from the fact that F proteins on the surface of the virus cause the cell membranes on nearby cells to merge, forming syncytia.RSV can cause upper respiratory infections and lower respiratory tract infections such as bronchiolitis and pneumonia. In children younger than 1 year of age, RSV is the most common cause of bronchiolitis and pneumonia.

Almost all children will have had an RSV infection by their second birthday. When infants and children are exposed to RSV for the first time, 25 to 40 out of 100 of them have signs or symptoms of bronchiolitis or pneumonia, and 5 to 20 out of 1,000 will require hospitalization. Most children hospitalized for RSV infection are younger than 6 months of age.People of any age can get another RSV infection, but infections later in life are generally less severe. Premature infants, children younger than 2 years of age with congenital heart or chronic lung disease, and children with compromised immune systems due to a medical condition or medical treatment are at highest risk for severe disease.RSV infections generally occur during fall, winter, and spring.

Drug contains monoclonal antibody against RSV ,called Palivizumab is available to prevent severe RSV illness in certain infants and children who are at high risk. The drug can help prevent development of serious RSV disease, but it cannot help cure or treat children already suffering from serious RSV disease and it cannot prevent infection with RSV. Palivizumab is administred ones a month via intramuscular injection to high risk infants and young children during RSV season.

American academy of Pediatrics has developed guidelines for prevention of RSV infections in high risk infants and little children with Palivizumab.In this article the most important areas of prevention with Palivizumab are higlighted.


Treatment of chylothorax in ICU following cardiosurgery in CHD

Kimovska M., Sofijanova A., Kojik Lj., Naunova S., Muratovska R., Voinovska T., Mandzukovska H., Neskova S.

Intensive Care Unit, University Children’s Hospital, Skopje, Macedonia

Abstract

Aim: The purpose of this article is to review our modest experience with the management of chylothorax following congenital heart surgery.

Background: Chylothorax after congenital heart surgery (CHD) is a rare, but potentially challenging complication. Although hospitalization can be prolonged, conservative therapy is effective in most cases, while surgical treatment can be useful in the refractory cases.

Materials and Methods: We reviewed the management of 5 patients with chylothorax (CT) between March 2013 and September 2015 in the ICU in our institution, (2 females, 3 male; 4 -8 months old) following 145 cardiothoracic procedures (incidence of 3,4%). Four patients were excluded because of incomplete data or incorrect diagnosis. Surgical procedures included tetralogy of Fallot repair in two patients, ventricular septal defect closure (one), DORV repair (one), Fontan procedure (one). The following parameters were analyzed: the triglyceride and cholesterol levels in the pleural fluid, LDH level,pleural fluid versus serum total protein/albumin, total cell number, and lymphocyte percentage; amount of pleural effusion on day of diagnosis, day 5, and day 14; and total time of pleural effusion. All patients followed a therapeutic protocol including complete drainage of chyle collection and controlled nutrition .

Results: Four of the patients (80.0%) responded to conservative therapy. Lymph leak ranged from 2,1 to 11,1 mL/kg per day for 5 to 35 days. One of the patients was treated with intravenous antibiotic therapy, due to the findings that correlate with pleural effusion (empyema) following CT. None of the patients with persistent drainage required thoracotomy, there was no necessity to attempt chemical pleurodesis. There was no deaths.

Conclusions: The majority of CT following surgery for CHD could be safely treated without surgical interventions, by diet manipulations with acceptable inherent morbidity. Patients with high-central venous pressure should be managed early with TPN and bowel rest. Enteral low-fat diet used in appropriately selected patients appeared to be as effective in controlling lymph leak as TPN. Monitoring closely for eventually development of infection of the pleural effusion (CT), differentiation between exudate or transudate, diagnosis and appropriate treatment for this condition, could be life saving.

Keywords: Chylothorax, Cardiosurgery, Congenital Heart Disease, ICU treatment, Pleural effusion, Conservative therapy


Femoral osteomyelitis associated with intravenous drug use in childhood

1Kirovski I, 1Sazdovski A, 1Micevska V, 2Georgiev Lj, 1Cvejovska-Colakovska V, 1Miskova S, 1Seckova L.

1University Children’s Hospital, Skopje, Macedonia

2PZO Dr. Ljupco, Kriva Palanka, Macedonia

Intravenous drug users can be at risk of many negative consequences as a result of their behavior. One possible complication is osteomyelitis. A 13-year-old boy with a history of intravenous drug abuse presented to the University Children’s Hospital with acute abdominal pain and pain in the left leg. After surgical consultation appendectomy was performed and uninflamed appendix was found. A surgical wound was healthy slowly. After a while he complained to left thigh and leg pain and dramatic swelling (Figure 1). A chest x-ray of the leg was performed. Radiography of the left thigh bone show cortical thickening with osteolitic and sclerotic changes including periostal reaction and epiphisiolis of the head of the femur (Figure 2). CT scan confirm the diagnosis of femoral osteomielytis-progressive inflammatory destruction and new apposition of bone of the femoral head/neck and intensive periostal reaction (Figure 3). Pus culture was positive for Staphylococcus aureus. Extensive antibiotic treatment, anticoagulation was performed for osteomyelitis. Surgical procedure was not undertaken. We present a case of femoral osteomyelitis associated with intravenous drug abuse in childhood. Aggressive management with early antibiotic administration, anticoagulation and surgical debridement can be life saving. Successful therapy requires a team approach including the pediatrician and consultants from orthopedic surgery, infectious diseases and substance abuse counselors.


Allogeneic Hematopoietic Stem Cell Transplantation (HSCT) in Pediatric Acute Leukemia

Svetlana Kocheva, Kata Martinova, Zorica Antevska –Trajkova,

Biljana Coneska-Jovanova, Aleksandra Acevska

University Children’s Hospital, Medical Faculty Skopje, Ss Cyril and Methodius University Skopje, Macedonia

Allogeneic hematopoietic stem cell transplantation (HSCT) is considered a treatment

modality for patients with acute leukemia who are predicted to respond poorly to intensive chemotherapy. Nowadays allogeneic HSCT is a routine procedure for the treatment of children with hematological, oncological and immunological diseases. In allogeneic HSCT recipients hematopoietic tissue is replaced by that of the donor. A source of hematopoietic stem cells for allogeneic transplantation can be bone marrow, mobilized peripheral blood cells and cord blood. Standardization and improvement of transplant approaches has led to equivalent survival outcomes with matched sibling and well-matched unrelated donors. Survival using mismatched and haploidentical donors in recent years by using new transplant protocols are becoming safer and more efficacious and are increasingly being used. Patients defined, as high risk by new methods will benefit from HSCT requires careful testing. In general, HCT typically offers benefit only to children at high risk of relapse with standard chemotherapy approaches. Accordingly, treatment schemas that accurately identify these high-risk patients and offer HCT and HLA-matched donors are available. HSCT have come to be the preferred approach.

In this review the authors outlines indication for allogeneic HSCT in children with acute leukemia, as well as the most common complication after HSCT. Key words: Pediatric allergenic transplantation, Hematopoietic stem cells, Acute leukemia, indication, Complication.


Immunization status of newborns in general hospital Prilep during the period 2008-2014

Kochoska B1, Tavchioska G 2, Koviloska R1, Hristoska L1

1 General Hospital Prilep, department of neonatology 2 General Hospital Prilep, department of pediatrics

Aim: to present the immunization coverage of newborns during the period of seven years.

Material and Methods: data were collected from the annual reports of the department of neonatology.

Results: In 2008 out of 1051 newborns, vaccinated with BCG were 85.25% and 98,85% against Hepatitis B. 91.92% of newborns were vaccinated with BCG in 2012 and 97.84% against Hepatitis B. Out of 978 born in 2014, 90.01% were covered by BCG, a 97,4% with Heapatitis B vaccine.

Conclusion: despite anti-vaccine movement, the immunization status of newborns in our hospital is on satisfactory level. Medical contraindications for immunization were the only reason for not implementing the immunization. There was not recorded a request by a parent for refusing the vaccination during the period 2008-2014.


Rate of premature newborns in General Hospital Prilep during the period 2008-2014

Kochoska B1, Tavchioska G2, Koviloska R1, Hristoska L1

1 General Hospital Prilep, department of neonatology 2 General Hospital Prilep, department of pediatrics

Aims: to present the trend of preterm born newborns during the period of seven years (2008-2914) in General Hospital Prilep, to present the gestational age of premature newborns.

Material and methods: for this retrospective study data were collected from the annual reports of the department and medical histories of newborns. Statistical analysis includes estimation of rate of prematurity for each year of the investigated period, creation of linear trend and selection of newborns according the gestational age.

Results: In 2008, out of 1051 newborns, 70 (6.6%) were premature. In 2009, out of 1149 newborns, 64 (5.57%) were premature. In 2014, out of 978 newborns, 56 (5.7%) were born before the full 37 weeks. Newborns are grouped according to gestational age into three groups (from 26.0 to 27.6 GW from 28.0 to 33.6 GW and 34.0 GW to 36.6)

Conclusion: there is a trend of discreetly decreasing of the rate of premature newborns, mostly in the group of newborns with gestational age from 34,0 to 36,6 GW which is consistent with the level of health care providing in the General Hospital Prilep.


Rare variant of Cohen syndrome, or a novel syndrome?

M. Kocova, E. Sukarova-Angelovska

Guidelines for GH treatment in children provide therapy only for several syndromes such as Turner syndrome, Noonnan syndrome, and Prader Willi syndrome. However, these guidelines allow therapy for GH deficiency in general. Several syndromes associated with GH deficiency are described:Toni-Debre-Fancony syndrome, VCF (De George) syndrome (del 22q11.2, Costello Syndrome,Sturge-Weber Syndrome, and Crouzon Syndrome.

We present two sisters with unusual set of pathologic features: complete GH deficiency, Cohen like dysmorphic features and absent ovaries. Clinical presentation, was of different severity. The older girl had hypotonia, seizures at 40 days of age, documented hypoglycemia, and impaired vision-severe myopia. She had normal development, and completed academic education. The younger girl had moderate hypotonia, hypoglycemia, frequent respiratory infections in infancy, repeated febrile seizures, severe myopia, neutropenia;she was significantly delayed in reaching motor and cognitive milestones. Facial and body features were concordant with Cohen syndrome in both girls : short philtrum, down slantedpalpebrae, thick hair, truncal non-progressive obesity.

Molecular analysis showed splicing COH1 mutation, however in heterozygous form.No mutation was found in genes that might cause growth deficiency (PROP and Pit).

Testing for GH confirmed severe isolated GH deficiency in both girls, and GH treatment helped to reach normal height in both girls. Vision was corrected with glasses.Only adrenal puberty developed. Thus induction with estrogen patches, and contraceptives followed. Hypergonadotrophic hypogonadism remains unexplained.

We did not find similar patients in the literature. Thus, it might be a variant of Cohen syndrome presenting unusual features, or a new syndrome.


State of the art therapy of Diabetes mellitus in childhood: Challenges in achieving optimal metabolic control

M. Kocova

Diabetes mellitus is among the most frequent endocrine diseases in childhood. Achieving metabolic control during childhood is a difficult task and international studies confirm that optimal metabolic control is missing even in the developed countries. Significant improvement in the therapeutic approach during the last decades was provided by the intensive insulin protocols, new insulin analogues, introduction of insulin pumps and improvement of the education of patients and families.

Introduction of insulin pumps as a standard of care especially for young children under 6 years of age and routine application of continuous glucose measurements significantly contribute to the improvement of the overall control.

We present the overall metabolic control in diabetic children based on analysis of 150 patients treated with intensive insulin therapy. Major factors contributing to metabolic control such as regular measurement of HbA1c at 3-4 months intervals, frequency of outpatient visits, glycemia records ((4 measurements per day) and frequency of hypoglycemia are presented. Local data are compared with the data of the International Hvidoere study group for study of childhood and adolescent diabetes. Data of Macedonian patients are comparable with the data of patients from 17 countries form Europe, USA and Japan.

Indications for use of insulin pumps, as well as the advantages and problems with use of insulin pumps are presented through analysis of 20 children on pumps during several months to 13 years.

New research in the field of insulin analogues and closed loop systems will be reviewed.


Thyroid neonatal screening program in the capital city of the Republic of Macedonia, 2002-2014

Mirjana Kocova and Violeta Anastasovska Genetic Laboratory, Department of Endocrinology and Genetics, University Clinic for Childrens’ Diseases, Skopje, Republic of Macedonia Background: Neonatal thyroid screening was introduced as a pilot study in Skopje, the capital city of the Republic of Macedonia, from April 2002 and established as nationwide screening program, after five years.

Methods: Blood spot TSH was measured by time-resolved fluoroimmunoassay (DELFIA, Wallac) in 111,276 newborns from all nurseries in Skopje during the 2002-2014. The TSH cut-off level was 10 mU/L.

Results: Out of 116,308 liveborn neonates, 111,276 have been screened (95.67%), with male to female ratio of 1.074. Among screened neonates 50% were Macedonians, 35.1% Albanians, 9.8% Gypsies, 1.4% Turkish and 3.7% other nationalities. A 2.69% newborns with TSH levels above 5mU/L indicate iodine sufficiency in Skopje. Thirty-four newborns with CH were detected with prevalence of 1:3273 and female to male ratio 1.43:1. Of the CH cases, 19 were Macedonians with prevalence of 1:2928 and 15 were Albanians or Gypsies with prevalence of 1:3331. The thyroid gland was undetectable on ultrasound in 18 (52.9%) newborns with CH, thyroid hypoplasia was confirmed in 4 (11.8%) and 7 (20.6%) had a normal size and structure of the thyroid gland. In 2 newborns (5.9%) the thyroid gland was slightly enlarged and in three (8.8%) agenesis of one lobe was confirmed. The median age at starting treatment with levothyroxin was 10.2 days, after birth.

Conclusion: The CH prevalence in Skopje was lower than assessed for the whole country (1:2486), however, it was comparable with reported in most of European countries. Higher coverage of the newborns in the Skopje region is warranted.

Keywords: congenital hypothyroidism; neonatal thyroid screening program


Respiratory resistance with interrupter technique - rint

Slavica Kostadinova

The application of lung function testing to preschool children is difficult due to limited cooperation that can be obtained in this age group.

Measurement of Rint has several practical advantages - it is measured during tidal breathing using portable equipment and is a non invasive, effort independent technique which requires minimal subject cooperation.

The principle of Rint is that during a sudden airflow interruption at the mouth, alveolar pressure and mouth pressure will rapidly equilibrate. Rint is defined as this pressure divided by airflow measured immediately before interruption. Occlusions should be made with a valve closing in less than 10 milliseconds and lasting for 100 milliseconds. They should be triggered by a flow set to coincide with the PEF and made during expiration. More occlusion should be recorded with an aim of retaining a minimum of five acceptable manevars. The median of all technically acceptable occlusions should be reported. During tidal breathing Rint reflect resistance of the respiratory system which includes conducting airways, lung tissue and the chest wall.

The interrupter technique is able to measure the magnitude of changes in airway caliber after inhalation of bronchodilator. A bronchodilation test should be considered clinically significant when the decrease in Rint after bronchodilator exceeds within occasion repeatability between two sets of measurements established in 30 to 50 subjects for each individual laboratory.

Assessment of pulmonary function in preschool children will help diagnosis, assist prognosis, monitor disease progress and measure the effect of therapeutic intervention.


Recommendations for diagnostic gastrointestinal endoscopy in children Kostovski A. University Children’s Hospital, Faculty of Medicine, Skopje, Macedonia Gastrointestinal endoscopy has revolutionized the diagnosis and management of many gastrointestinal diseases and is now an integral part of the evaluation and treatment of gastrointestinal diseases in children and adolescents. Endoscopic gastrointestinal procedures are now common in most major pediatric centres and they can be safely performed in small infants, including newborns. Over the past few years, experts in committees and scientific societies have been working to establish indications for performing gastrointestinal endoscopy in children. With the development of pediatric gastroenterology new diseases emerged and indications for performing endoscopies had changed. The most recent data on the indications for performing diagnostic endoscopy in the most common pediatric gastrointestinal disorders (celiac disease, GERD, eosinophilic esophagitis, H.pylori infection, cow’s milk protein allergy etc.) will be summarized and presented. Also, the results of the multicentre European study, in which participated Macedonia, considering the pediatricians compliance to the newest ESPGHAN guideline for diagnosing and management of GER will be presented. It is important to implement in practice the evidence based recommendations issued by relevant associations when indicating diagnostic endoscopy in children.


Асфиксија кај новороденчињата во породилиштето во прилепската болница

Автор: Роза Ковилоска, педијатар во Неонатолошко одделение при општа болница ,,Борка

Талески”, Прилеп

Клучни зборови: новороденче , асфиксија, превенција

ВОВЕД По дефиниција асфиксија е состојба непосредно по раѓањето каде при очувана срцева акција, имаме намалени или ретки површни респораторни движења или истите ги нема. Асфиксијата може да биде бледа или модра. ПРИЧИНИ: Матернални, плацентарни, фетусни ЦЕЛ Да се прикаже процентуалната застапеност на асфиксијата во породилиштето во Прилеп како и причините кои довеле до неа МАТЕРИЈАЛ И МЕТОДИ Земени се податоци од картотеката и евиденционите листи кои се водат во Неонатолошкото одделение во Прилеп. РЕЗУЛТАТИ Обработени се податоци за период од 2005 до 2010 година 2005 година- вкупно родени 1185 ; доносени 1078 (91%) ; недоносени 107 (9%) ; спонтано родени 878 (74%) ; S.C 209 (17,6%) ; асфиктични 107 (9,2%) 2006 - вкупно родени 1176 ; доносени 1106 (94%) ; недоносени 70 (5,9%) ; спонтано родени 825 ; S.C 191 (16,2%) ; асфиктични 132 (11,2%) 2007 - вкупно родени 1105 ; доносени 1036 (93,7%) ; недоносени 69 (6,25%) ; спонтано родени 828 (75%) ; S.C 172 (15,5%) ; асфиктични 102 (9,8%) 2008 - вкупно родени 1051 ; доносени 981 (93,3%) ; недоносени 70 (6,6%) ; спонтано родени 815 (77,5%) ; S.C 161 (15,3%) ; асфиктични 63 (4,2%) 2009 - вкупно родени 1149 ; доносени 1085 (94,4%) ; недоносени 64 (5,5%) ; спонтано родени 884 (77%) ; S.C 190 (16,5%) ; асфиктични 90 (7,8%) 2010 - вкупно родени 861 ; доносени 813 ( 94,4%) ; недоносени 48 (5,5%) ; спонтано родени 650 (76%) ; S.C 174 (20%) ; асфиктични 68 (7,9%). Асфикциите се почести кај недоносените деца и деца родени со S.C. ЗАКЛУЧОК Со редовните контроли на бремените жени кај матичниот гинеколог и спроведување на неговите совети, како и контролирање на постоечките заболувања кај мајките , бројот на асфиктичните новородечнчиња би се сведил на минимум.


Retrospektivna analiza na vlijanieto na vakcinata vrz Epidemiolo{kite karakteristiki na morbili na podraчjeto na zdravstven dom - Вeles od

1961 do 2014 godina

P. Krstev1, I. Koleva1, B.Trajkova2 , T. Samarakova1, M. Janevska1 Zdravstven dom- Veles1, Centar za javno zdravje2-Veles, R. Makedonija CEL: Evaluacija na epidemiolo{kite karakteristiki, pojavata i {ireweto na bolesta vo period bez vakcina, potoa vlijanie na zadolìtelnata imunizacija protiv morbili vo period koga e vakcinirano edna{ i vo period koga e vovedena i revakcinacija na sedum godi{na vozrast na podra~jeto na Zdravstven dom-Veles od 1961 do 2014 godina.

MATERIJAL I METODI: Analizirani se realiziranata imunizaciona programa, vakcinalniot status na zabolenite lica od evidencijata na detskata preventivna zdravstvena za{tita so razvojno sovetuvali{te, u~ili{nata preventivna zdravstvena za{tita i prijavite za zarazni zaboluvawa od CJZ-Veles. Vo statisti~kite metodi koristen e deskriptivniot i analiti~kiot metod. Vozrasnite grupi, op{tiot i specifi~niot morbiditet po vozrast se prikaàni spored standardite i podatocite za naselenieto od oddelenieto za socijalna medicina pri CJZ-Veles.

REZULTATI: Vo analiziraniot period od 54 godini najmnogu zaboleni ima koga ne se vakcinira od 1961 do 1971 godina so 745(Mb 125,9 %oo) vo 1964 godina. Na edna godi{na vozrast se vakcinira od 1972 godina do 1986 godina, so najmnogu zaboleni vo 1975 godina 363(Mb 59,7%oo). Vo 1987 godina e vovedena i revakcinata na 7 godi{na vozrast, so najmnogu zaboleni vo 1993 godina 22(Mb 3,3 %oo) i vo 1996 godina 64 (Mb 9,7 %oo). Vo periodot od 1987 do 2014 godina vakcinirani se 22883 i revakcinirani 24043 lica, a zaboleni se 242 i toa 212 nevakcinirani i 30 vakcinirani, od koi so edna doza 25, a so dve dozi 5. Po vozrasni grupi dominiraat zaboleni koi se nevakcinirani od 1-4 godini 61 i nad 15 godini 93, od koi 39 se nad 20 godi{na vozrast.

Vo 2011 godina ima 21 zaboleno lice i od 29.03 - 23.08.2011 godina poradi proglasena epidemija e sprovedena vonredna vakcinacija kaj deca od 6 do 12 mese~na vozrast i kaj lica do napolneti 40 godini koi bile nepotpolno vakcinirani. Vo toj period vonredno se vakcinirani 589 deca pod edna godi{na vozrast i 59 lica nad 15 godi{na vozrast.

Od po~etokot na vakcinacijata do sega nema prijaveno seriozen slu~aj na postvakcinalna reakcija nitu dete so autisti~en sindrom povrzan so vakcinata.

ZAKLUÔK: Dobienite podatoci go potenciraat zna~eweto na vakcinacijata. Epide-miolo{kata prognoza za pojava i {irewe na morbili najmnogu zavisi od procentot na vakcinirani i prazninite vo grupniot imunitet. So vakcinirawe na propu{tenite lica i nad 95% opfatenost od podle`nata grupa, bi se spre~ila cirkulacijata na bolesta, pomestuvaweto kon povozrasnite grupi, a indirektno bi se za{titila i vozrasnata grupa pod edna godina, dodeka navremenoto vakcinirawe bi rezultiralo so eradikacija na bolesta.


Congenital complete atrioventricular block

Kuzevska-Maneva K.¹, Kacarska R.¹,Gjurkova-Angelovska B.¹, Jovanovska V. ¹,Neshkovska-Shumenkovska M.¹, Chadikovski V.², Popovska L.³ ¹University children’s hospital, Clinical center Mother Tereza, Skopje, Macedonia ²University hospital for children’s surgery, Clinical center Mother Tereza, Skopje, Macedonia ³Institute for cardiovascular diseases, Clinical center Mother Tereza, Skopje, Macedonia Congenital complete atrioventricular (AV) block is a very rare heart rhythm disturbance in which atria and ventricles beats independently one of each other, every with own frequency. It causes significant bradycardia. AIM. We represent three cases of AV total congenital block, the way of diagnosis and treatment. MATHERIAL AND METHODS. For the period of 2 years (2013-2014 y) we diagnosed three children with complete congenital AV block, aged from 1 day to 3 months at the time of diagnosis. As a methods we used: anamnesis, clinical sings and findings, 12 channel ECG and Holter 24 hour ECG monitoring. RESULTS. We diagnosed three children 1day, 1 month and 3 months old. Two were boys, and one girl. Two children had prenatal findings of abnormal cardiotocography. Two of the mother had illness of the collagen tissue - one Sjogren syndrome and the other had systemic lupus eritematosus. All children were asymptomatic. Clinically they had bradycardia (under 70 bpm), normal heart auscultation. On ECG we found AV total congenital block with frequency between 80-104 bpm, no evidence of ectopic beats. Two of children had normal echocardiographic finding, one had dilatation of left ventricle with mild hypokinesia. Holter 24 hour ECG was performed in all children, two of children had investigation in neonatal period, the other at the age of 3 months. Minimal cardiac frequency was 40..55..48, the main 78..75..85, and the maximal 120..104..108. One of children had PVC. No evidence of sinus pauses. Pacemaker was applied in one child at the age of 2 months, because of poor myocardial function. The other two children had not symptoms, good myocardial function and have regularly follow-up. CONCLUSION. AV total congenital block is a rare condition in childhood. It is more frequent in children from mother with illness of collagen tissue. Early application of pacemaker is necessary in small number of patients, but every pts had need of pacemaker later in their life. Most of them have normal expecting life surviving.


Diagnosis and management of urinary tract infection in children

Kuzmanosvka Dafina, St Cyril&Methodius University Pediatric Clinic

Urinary tract infection (UTI) is among the most commonly diagnosed bacterial infections of childhood. Although frequently encountered and well researched, diagnosis and management of UTI continue to be a controversial issue with many challenges for the clinician. For reasons that are not yet completely understood, a minority of UTIs in children progress to renal scarring, hypertension and renal insufficiency. Clinical presentation may be nonspecific. Prevalence studies have shown that UTI may often be missed on history and physical examination, and the decision to screen for UTI must balance the risk for missed infections with the cost and inconvenience of testing. Interpretation of rapid diagnostic tests and culture is complicated by issues of contamination, false test results, and asymptomatic colonization of the urinary tract with nonpathogenic bacteria. The appropriate treatment of UTI has been controversial and has become more complex with the emergence of resistance to commonly used antibiotics. Until recently, imaging work-up after first febrile UTI was tailored to uncover functional and structural abnormalities such as dysfunctional voiding, vesicoureteral reflux and obstructive uropathy. Aggressive work-up, including renal cortical scintigraphy, ultrasound and voiding cystourethrography, was recommended for patients at greater risk for pyelonephritis and renal scarring, including infants less than one year of age and all children who have systemic signs of infection concomitant with a UTI. Antibiotic prophylaxis was used in patients with reflux or recurrent UTI who are at greater risk for subsequent infections and complications. However this approach has been based on limited evidence, and newer studies question some of the tenets of prior recommendations. The goal of this review is to provide an up-to-date summary of the literature with particular attention to practical questions about diagnosis and management for the clinician.


Geographic variation in childhood asthma prevalence

Lawson Joshua

Medicine and Canadian Center for Health and Safety in Agriculture , University of Saskatchewan, Saskatoon, Canada

Childhood asthma is a global health problem. Over the past few decades, the prevalence of asthma has increased rapidly. However, there has also been geographic differences both in the frequency of asthma and in the rates at which it has risen. First, internationally, asthma prevalence appears to be higher in Westernized nations. Second, rural areas and farm dwelling have been shown to have inverse associations with asthma prevalence. The reasons for the observed variations in asthma prevalence are unknown but the following have been investigated: (1) diagnostic labeling, patient reporting, and health care access; (2) environmental differences; and (3) health behaviours. The objective of this presentation is to review the current knowledge regarding the geographic variation with a special emphasis on recent studies completed within Canada and Europe. As a part of this, we will discuss childhood asthma prevalence, incidence, and potential risk factors as we present evidence to help identify the major reasons for geographic variation in asthma prevalence.


Етиологија на конгенитален стридор кај дeца - приказ на случај Манасиевски Е. , Галбова С., Шаревска З., Petliчkovska S.,Ugrinoska Pandeva A. Институт за белодробни заболувања кај децата- Козле, Скопје Цел на трудот е докажување на улогата на флексибилната бронхоскопија при дијагностицирање на етиологијата на конгенитален стридор кај доенчиња со рецидивни респираторни тегоби. Трахеомалацијата претставува состојба на слабост на трахеалниот зид и потпорните картилагинозни прстени на трахеата. Обработено е 7 месечно доенче, со анамнестички податок за стридор од раѓање, чести епизоди на ларингеална кашлица, една епизода на бронхит и често повраќање по хранење. На прием бледо, со рахитични стигмати, со ларинготрахеална кашлица, груб инспираторно-експираторен стридор и аускултаторен наод во прилог на бронхиолит. Биохемиските иследувања со знаци за акутен инфект и анемија, рентгенолошкиот наод во прилог на воспалителна инфилтрација, реализирана флексибилна бронхоскопија со која се докажа трахеомалација. Флексибилната бронхоскопија е златен стандард за дијагностицирање или исклучување на посериозни причинители за стридор.


Oesophageal atresia - experience and management on department of intensive care and therapy

dr. H.Mandzukovska, dr.Lj.Kojik, dr.S.Naunova, dr.R.Muratovska, dr.T.Voinovska,

dr.S.Neshkova, dr.M.Kimovska, dr.A.Sofijanova,dr. S.Bojadzieva

University Pediatric Clinic-Skopje

Abstract

Oesophageal atresia with tracheoesophageal fistula is an anomaly of the esophagus that often appears in the neonatal age. Most often it occurs with other associated anomalies such as cardiomyopathy, followed by genital-urinary and gastrointestinal malformations.

The existence of oesophageal atresia in the prenatal period can be predicted by the presence of polyhydramnion. But usually it is confirmed within 24 hours after the birth, when clinical symptoms appear: hypersalivation, inability to swallow and easy respiratory distress. Placing a nasogastric tube is impossible simultaneously.

Diagnosis is established by an x-ray of the lungs and abdomen (babygram), with tracheobronchoscopy and contrast radiographs of the oesophagus (oesophagogram).

It is preferable that the surgery is made as early as possible to reduce the risk of postoperative complications. Oesophagus atresia which are not associated anomalies have better prognosis, and worst prognosis have atresia associated with cardiomyopathy.

There is a tendency to improve the postoperative treatment of these abnormalities in the last 20 years. Largely this is due to advancement in postoperative intensive treatment and care, mechanical ventilation as respiratory support, early surgical intervention, use of intravenous antibiotic therapy and so on.

In interdisciplinary teams of physicians-specialists who assist in establishing the quality of life of these children in recent times for the correct postoperative treatment are engaged to correct postoperative treatment .

Keywords: oesophageal atresia, traheoezofageal fistula oesophagogram, postoperative treatment


Pediatric oncologic emergencies

Kata Martinova, Svetlana Kocheva, Biljana Coneska-Jovanova, Zorica Trajkova-Antevska, Aleksandra Jovanovska,

University Children’s Clinic, Department of Hematology and Oncology, Skopje, Macedonia

Abstract

Cancer and cancer treatment may lead to a range of life-threatening conditions requiring urgent action. Oncologic emergency (OE) is any acute potentially morbid or life-threatening event related to cancer or cancer treatment. OE can be classified as metabolic, hematologic, structural, or chemotherapy side-effects. Metabolic emergencies include tumor lysis syndrome (TLS), hypercalcemia and syndrome of inappropriate antidiuretic hormone (SIADH).

Acute TLS is a group of metabolic complications associated with the treatment of rapid proliferation cancers caused by acute cell lysis. The release of intracellular products overwhelms the homeostasis mechanisms. The cardinal findings of acute TLS include hyperphosphatemia, hypocalcemia, hyperuricemia, hyperkalemia and acute renal failure. Hematologic emergencies include febrile neutropenia (FN) and hyperviscosity syndrome. FN is one of the most common complications, contributing to 50% of deaths associated with leukemia, lymphomas and solid tumors. Bacterial infections are common in patients with febrile neutropenia and fungal sources are increasingly prevalent. Symptoms include temperature above 38.5°C and absolute neutrophil count (ANC) less than 0,5X10/9/L. Antibiotic treatment for FN depends on the patient’s risk of life-threatening infection. Hyperviscosity syndrome due to hiperleukocytosis is most common in patients with leukemia who have markedly elevated white blood cell (WBC) count being at risk of end-organ damage. Structural emergencies include superior vena cava syndrome (SVCS), epidural spinal cord compression and malignant pericardial effusion. Side effects of chemotherapy agents include extravasations and allergies.

Conclusion: Recognising and instituting prompt and appropriate management in an oncologic emergency could make a major difference to a patient’s short-term prognosis and quality of life.


Thalassemia in republic of Macedonia – an overview

Kata Martinova¹ Svetlana Kocheva¹ Biljana Coneska-Jovanova¹, Aleksandra Jovanovska¹, Zorica Trajkova-Antevska¹, Oliver Karanfilski², Svetlana Stankovikj²

¹University Children’s Clinic, Department of Hematology and Oncology, Skopje, Macedonia

²University Clinic for Hematology, Skopje, Macedonia

Abstract

Thalassemias are a group of inherited disorders of Hemoglobin (Hb) synthesis caused by an imbalance in the production of alpha and beta globin chains. Thalassemia is considered the most common genetic disorder worldwide. Globally there are 269 million estimated carriers of Hb disorders and 150 million people carry Beta Thalassemia genes. In R. of Macedonia, roughly 3,8% of the population carry genetic abnormalities. Currently, no more than 15 cases with Thalassemia major (TM) are under care of the University Children’s Clinic, of which 2-3 over the age limit, and no more than 10 cases are under care of the University Clinic for Hematology. Most of the patients are homozygous for Beta thalassemia major, and others are double heterozygous. Most of the cases are diagnosed before the age of 6-12 months. In all patients, the disease is defined at a molecular level which allows for genetic counseling and prenatal diagnosis. Currently, treatments are limited to life-long regular blood transfusions and chelation therapy to prevent fatal iron overload. The alternative treatment is bone marrow transplantation (BMT)-an option open to small number of patients. Concerning outcome analysis, there are no fatalities among pediatric patients, but adult patients rarely exceed the age of 40 years. The main causes of death are cardiac lesions and hepatic insufficiency. Our efforts today are focused at maintaining the good condition of the patients, improved quality of life, extended lifespan up to possibilities of application of new therapeutic options (gene therapy).


Pneumonia – case report review

Micevska V., Vlaski E., Seckova L., Sazdovski A., Kirovski I., Colakovska V., Miskova S.

Department of pulmoallergology, University Childrens Hospital, Skopje, Macedonia

Introduction: Pneumonia and other low respiratory tract infection are the leading causes of mortality in children worldwide (annual incidence of approx.150 milion new cases). 10-40 % of them goes to atypic pneumonia, mostly in school children. Pneumonia is an inflammatory process of the lung tissue presented by consolidation of the affected part and inflammation inside the alveoli consisting of exudate, inflammatory cells and fibrin. Aim: case report review of pneumonia with atypic presentation in hospitalized children in winter 2014-2015. Results: In the study six cases of hospital patients, age 4 to 13 were analyzed. In all of them main symptoms of the disease were cough and fever at least 7 days before they were admitted to hospital. Auscultatory normal to diminished breath sounds were noted. Huge discrepancy between auscultatory and radiological findings was found in all of the cases. Radiological findings such as massive inflammatory infiltrates (segmental and lobar) were present. Laboratory showed normal level of white blood cells and negative CRP (C- reactive protein). In three of the patients positive IgM titres for Mycoplasma pneumonia , one for Chlamidia pneumonia and one for Legionella pneumophilla were detected. Microbiological findings of examined materials (tracheal aspirate and blood samples) were all found sterile. After the antibiotic treatment complete remission of the radiologic findings was achieved in all six of the patients. Conclusion: this case report review showed: a group of patients with atypic pneumonia characterized by a huge discrepancy between auscultatory and radiological findings; negative bacteriological findings and excellent response of the antibiotic treatment.


Hiatus hernia – case report

Micevska V1., Vlaski E1., Seckova L1., Sazdovski A1., Kirovski I1., Colakovska V1., Miskova S1., Simeonov R2., Abdiu B.3

1Department of pulmoallergology, University Childrens Hospital ,Skopje, Macedonia

2Pediatric Surgery Clinic, Skopje,Macedonia

3Pediatric department, Clinical Hospital Tetovo, Macedonia

Introduction: Hiatal hernia is a rare condition in childhood. Congenital paraoesophageal hernia is characterized by an anatomic defect of the gastric hiatus through which abdominal organs migrate in the thorax, while gastroesophageal circuit is in its normal position. The most common location is the right side of the thoracal oesophagus where a part of the gastric fundus herniates. It is common found in infants and toddlers. Most characteristic symptoms are respiratory (cough,dyspnea,respiratory distress), vomiting,regurgitation and anaemia. Diagnosis is made by chest x-ray and gastroduodenooesophagography and the treatment is surgical.

Results: a case of a 11 month old infant is reported, which is admitted to our hospital because of a cough that persisted during 7 months until the actual moment. Laboratory findings showed hyposideremic anaemia. The chest x-ray finding suspected a presence of hiatal hernia. Final diagnose was made by gastroduedenooesophagographic examination. The treatment of the hernia was surgical, performed by fundoplicatio Nissen. The successful operation was confirmed by the control finding of the contrast examination.

Conclusion: we reported a case of e rare condition in childhood, which was presented by cough as a dominant symptom. Timely made both diagnosis and treatment is very important for prevention of possible fatal complication of this condition, such as gastric volvulus, strangulation and perforation.


Newborn screening of Congenital Hypothyroidism at Special Hospital of Gynaecology and Obstetrics “Mother Theresa” , Skopje

B. Mihajlovic Dimovska1, V. Delovska Stojkova1, J. Guleva1, M. Kalajdzieva Zip1, B. Pocesta1, B. Ismaili1, M. Kocova2, V. Anastasovska2

1Department of Neonatology, Special Hospital of Gynaecology and Obstetrics “Mother Theresa” – Skopje, Republic of Macedonia

2Genetic Laboratory, Department of Endocrinology and Genetics, University Clinic for Childrens'

Diseases, Skopje, Republic of Macedonia

Congenital hypothyroidism(CH) is one of the most common preventable causes of mental retardation. Newborn screening, early diagnosis and thyroid therapy started within 2 weeks of age can normalize cognitive development. The overall incidence of CH ranges from 1: 3000 to 1:4000 newborn infants.

Aim of the study is to analyze the coverage of the screened newbornsat our hospital, as well as to present the incidence of CH.

Material and Methods: the study is retrospective analysis of 41 637 newborns in the period of April 2002 up to June 2015 at SHGO“Mother Theresa” , Skopje. The data from computer database and neonatal histories at Neonatal Department, as well as the data from Genetic Laboratoryof University Clinic for Childrens' Diseases, have been analyzed. The detection of CH consists of fluoroimmunometric assay (DELFIA,Wallac) for neonatal thyroid-stimulating hormone (TSH) from bloodspots on filter paper (Whathman903), obtained from newborns 48-72h after birth. Cut of value is 10mU/L.

Results:during the mention period out of 41 637 newborns, 40 851 (98,11%) newborns have been screened. Eighteen newborns(0,044%) with CH have been detected. The incidence is 1:2269. Covering analysis of screened newborns up to years shows in the first seven years it is 97,19%, while in the last seven years it is 99,43%. There is large difference in the incidence, too. The incidence in the last seven years is 7,6 times higher vs. first seven years (1:922 vs. 1:7076).

Conclusion:higher covering of screened newborns in the last several years is result of better organization of the realization of newborn screening(taking blood, filter papers transport, blood analysis). Longer period of screening is necessary to accurately assess the average incidence of CH at our hospital.


Inetrventional procedures for congenital heart disease

I.Milev , T.Anguseva, S.Idrizi, V.Ampova, , E.Stoicovski, V.Belostotckij, Z.Mitrev

Special Hospital for Surgery Fillip II, Skopje, Macedonia

OBJECTIVE: Interventional techniques available for use in treating congenital heart disease include balloon dilation of valves and vessels, stent placement and coil embolization of collaterals, patent ducts and other arterial fistulae. In addition, a variety of devices for closure of atrial and ventricular septal defects and patent ducts currently are under investigation. Radiofrequency ablation of arrhythmias also is applicable to the pediatric population

METHODS: During last 12 years 522 patients with congenital heart disease had been treated with interventional procedures in our hospital. All of them had been diagnosed by transthoracic and transoesophageal echocardiograpfy. All patients had one day hospitalization.

RESULTS: 268pts with atrial septal defects had been occluded with amplatzer septal occluder, 50pts had interventional closure of the persistent arterial channel between aorta and pulmonary artery by amplatzer AGA vascular plug et coil, 35 pts got ventricular septal defect occlusion by amplatzer septal occluder; balloon valvuloplasty of the congenital aortic valve stenosis was performed in 17 pts. and balloon valvuloplasty for pulmonary valve stenosis was performed in 120 pts.Aortic stent for treatment of the aortic coarctation was implanted in 29pts.With an anplatzer AGA vascular plug coronary AV fistula was occluded in 3 pts. No mortality and no complications were noted. Follow up period is up to 12 years.

CONCLUSION: interventional catheterization has become solidified as an integral component of the comprehensive management of patients with essentially all forms of congenital heart disease. Patients are getting permanent solution with minimum side-effects of their health.


Risk factors for depression as discriminating factors for clinical and subclinical depression in adolescence

Lence Miloseva, a, TatjanaVukosavljevic-Gvozden aFaculty of Medical Science, Dept. of Psychiatry and Clinical Psych., GoceDelcev University, Stip, R. Macedonia bFaculty of Philosophy, Dept. of Clinical Psych., University of Belgrade, R. Serbia Introduction: We believe that the identification of potential groups of differences and similarities between clinical and subclinical depression in adolescence, compared to the risk factors of cognitive vulnerability and psychosocial risk factors, contributes to shedding light on the etiological picture of depression.Prevention of subclinical depression is important not only for treatment, but also because subclinical adolescents are under risk of occurrence of Major Depressive Disorder. Objectives: To examine whether the clinical group with depression, the subclinical group and the control group differ significantly in relation to risk factors of cognitive vulnerability (dysfunctional attitudes, negative inferential style, ruminative response style) and psychosocial risk factors (negative life events and perceived social support). Methods: The final survey sample consists of 412 adolescents (61.7% female and 38.3% male) aged 13-17 years (M = 15.70, SD = 1.22) from Stip, Skopje and Bitola, R.Macedonia. Cognitive vulnerability factors for depression (dysfunctional attitudes, negative inferential style, ruminative response style), psychosocial risk factors (negative life events and perceived social support) were measured by a set of instruments. We applied: MINI kid Screen; Dysfunctional Attitude Scale; Adolescent's Cognitive Style Questionnaire; Ruminative Response Style Questionnaire; Adolescent Life Events Questionnaire; Multidimensional Scale of Perceived Social Support; and Beck Depression Inventory II.Using canonical discriminant analysis we tested the ability to predict group membership (clinical, subclinical and control) by means of all instruments used,RUM, DAS, ACSQ, ALEQ and MPSS. Results and discussion: The discriminant analysis showed that it was possible to statistically significantly distinguish groups on the basis of the mentioned predictors and it allocated two discriminant functions. The first discriminant function consists of all listed predictors, RUM, DAS, ACSQ, and ALEQ in a positive direction and MPSS in a negative direction, and in this function the highest scores are for the clinical and the lowest for the control subjects. Subclinical respondents were in-between. The overall test of the first discriminant function was significant (χ2 (10) = 1234,67, Wilks’ λ = .048, p < .001) indicating that scores discriminated between the three groups accounting for 99.3% (canonical R = .97) of the total variance of depression. Only RUM makes the second discriminant function and the subclinical sample has the highest scores, while the clinical sample has the lowest. The control group is in-between. The overall test of the second discriminant function was significant (χ2 (4) = 47.28, Wilks’ λ = .89, p < .001) indicating that scores discriminated between the three groups accounting for 0.7% (canonical R =.33) of the total variance of depression. The overall accuracy for classification using the two discriminant functions for the level of depression was 99.3%. Conclusion:We have confirmed our expectations that the clinical, subclinical group and control group differ significantly with respect to the factors of cognitive vulnerability (dysfunctional attitudes, negative inferential style,ruminative response style) and psychosocial risk factors (negative life events and perceived social support). We believe that this research has scientific and applicative value and that it has not only theoretical and empirical contributions and contributions to clinical practice, but that its contribution is also social and concerning public health. Key words: risk factors, discriminating factors, clinical depression, subclinical depression, adolescence.


Тумор некрозен фактор алфа-биомаркер кај асматска болест во детската возраст Мирчевска В.,Лазаревска И.,Младенов М.,Мирчевска С. ЈЗУ.Институт по белодробни заболувања кај деца, Скопје; ПМФ.Институт за Биологија, Скопје, Р.Македонија Вовед: Примарен медијатор во патогенезата на инфекциите, повредите и воспалението,

како и во одбрамбените механизми на организмот и во ткивната хомеостаза е полипептидниот

хормон кахектин или познат како тумор некрозен фактор алфа (TNF-α).

Цел: Да се процени значењето на биомаркерите на инфламација меѓу кои и TNF-α и

неговата улога при астматската болест.Тој е есенцијален показател при дијагноза и за

терапевтскиот протокол на водење кај истата, а истовремено може да биде од помош при

детерминирањето на еволутивниот тек на болеста и намалување на ризикот од неа.

Методи и материјали: Во овој труд беа вклучени 66 пациенти, поделени во три

групи, при што беа обработени вкупно 92 серумски примероци. Пациентите со оваа дијагноза

беа на возраст од 1 до 11 години, а беа хоспитализирани поради дијагностички и терапевтски

третман. Ниту еден од нив не примаше кортикостероидна терапија пред третманот. Крв за

анализа беше земена непосредно пред започнувањето со терапијата и по 1 месец после

терапијата.Беше одредувана концентрацијата на Тумор некрозниот фактор алфа(TNF-α) во

крвниот серум кој е во тесна релација со еволуцијата на болеста.

Резултати: Дистрибуцијата според половата застапеност покажа доминација на

машкиот пол - 18 (69,2%) во однос на женскиот пол - 8 (30,8%) кај пациентите со бронхијална

астма.

Од статистичката обработка на податоците, евидентен беше трендот на пораст на

просечните вредности на TNF-α во серумот, како што прогресира болеста. Просечната

вредност на TNFα во серумот пред и после терапијата изнесуваше x ± sd = 23,37 ± 2,05 v.s.

9,91 ± 0,45 pg/ml,( Р<0.05).

Дискусија и заклучок: Овој труд е значаен за навремено дијагностицирање и следење

на болеста.И од клинички и од научен интерес подразбира рано дијагностицирање и

мониторирање, како и доследно спроведување на антиинфламаторната терапија.

Клучни зборови: Биомаркери, TNF-α,бронхијална астма.


Overuse of antibiotics – cause for secondary immunodeficiency because od neutrophil

dysfunction in childhood

Prim. M-r sci.D-r Kristina Mironska

University Clinic for Children Diseases, Skopje

Abstract

During the last passed years, raised is the number of the children who suffered from

frequent and recurrent infections cured with antibiotic therapy and who,instead of

expected well-being,become prone to the new infections that require antibiotic therapy

again.

With the aim to define the influence of the antibiotic therapy upon the neutrophil

function, the clinical study was performed, in which 300 pediatric patients receiving

repeated and long-termed antibiotics were investigated. In 213 (71%) of the patients

neutrophil dysfunction was found. 175 (58,33%) patients had completely impaired oxidative

metabolic activity during the phagocytosis, with the main value of the spontaneous NBT of

83,58fmol/fag, which is 35,20% less than the normal activity. The additional 39 (12,67%)

patients had impaired neutrophil function manifested with inability to increase the oxidative

activity after the stimulation, with the main value of the stimulated NBT of 114,26fmol/fag,

which is 60.60% less than the normal activity. The significant negative influence of the

repeated and long-termed used antibiotics in all the patients in the study, upon their based

neutrophil activity is confirmed.

Irrational use of antibiotic therapy hides a big danger of bacterial resistance

appearance, but also hides a big danger of secondary immunodeficiency,especially because

of possibility fordeveloping of neutrophil dysfunction in childhood.


Legofer – analiza učinkovitosti kod djece u tuzlanskom kantonu

Prim. Dr. Amila Latifagić, mr.med.sci., UKC Tuzla, Mr.med.sci. Dr Admira Hadžiselimović, UKC Tuzla, Prim. Dr. Zlata Đedović – Hasukić, DZ Gradačac, Dr. Jasna Alić, DZ Tuzla, Dr. Senada Kovač, DZ Srebrenik, Dr. Suad Zelenturović, DZ Gračanica, Prim.Dr. Vahida Hodžić, DZ Banovići, Dr. Mirela Mujkić, DZ Tuzla, Prim. Dr. Selma Memić, DZ Tuzla, Prim. Dr. Ševala Paloš, DZ Tuzla, Prim. Dr.sci. Mirjana Remetić, DZ Tuzla, Prim. Dr. Tonka Mrkajić, DZ Tuzla, Prim. Dr. Sadedin Al Sadi, DZ Kalesija, Dr. Vesna Jahić, DZ Tuzla, Dr. Enver Avdić, DZ Kalesija, Dr. Sadeta Terzić, DZ Gradačac, Dr . Mirela Tobudić, DZ Tuzla, Dr. Šemsudin Okanović, DZ Gračanica,Dr. Mirsad Špirtović, ALKALOID,

Anemija je smanjenje koncentracije hemoglobina ispod dvije standardne devijacije od srednje vrijednosti za uzrast zdrave djece. U funkcionalnom smislu anemija je smanjenje sposobnosti krvi da prenosi kiseonik do organa i perifernih tkiva, a nastaje zbog smanjenja količine eritrocita, hemoglobina ili i jednog i drugog ispod vrijednosti koje su normalne za dob (Nathan i Osky, 2000). Vrijednosti hemoglobina, kao i drugih hematoloških parametara veoma su varijabilne u djetinjstvu. Za brzu orijentaciju primjenjuju se kriterijumi Svjetske zdravstvene organizacije navedeni u Tabeli 1. Tabela 1 - Kriterijumi SZO za anemiju Uzrast (godine) Hb (g/L) 0,5 – 5 110 5 – 12 115 12 – 15 120 Iako postoje mnogobrojni uzroci anemije, najvažniji je manjak željeza u ishrani. Pojavi anemije doprinose i drugi deficiti u ishrani kao što su nedovoljan unos folne kiseline i vitamina B12. Takođe, hronične bolesti, upalne bolesti crijeva, hronična krvarenja i infektivne bolesti mogu dovesti do sideropenijske anemije. Brzo povećanje tjelesne težine u prvoj godini, posebno u prvih 6 mjeseci života je dodatni faktor koji doprinosi nastanku anemije. Anemije nastaju zbog: • Nedovoljnog stvaranja eritrocita ili hemoglobina, • Pojačane razgradnje eritrocita (hemolitičke anemije), • Gubitka krvi (akutni i hronični), (Roberts, 2008). Za postavljanje dijagnoze neophodno je uzeti iscrpnu anamnezu, obaviti fizikalni pregled, te uraditi laboratorijske pretrage Za dijagnozu anemije izazvane nedostatkom željeza raspolaže se velikim brojem laboratorijskih parametara, ali svaki od njih ima svoja ograničenja. Važni dijagnostički parametri su: vrijednost hemoglobina (Hb), broj eritrocita (RBC), srednja zapremina eritrocita (MCV), raspodjela veličine eritrocita (RDW) i hematokrit (Htc). (Sherriff A, Emond A, Bell JC, 2001). Cilj rada je pokazati učinkovitost Legofera u liječenju anemija kod djece. Materijal i metode: U ovoj analizi prikazat ćemo rezultate 121 djeteta kod kojih imamo vrijednosti hemoglobina: prije uključivanja Legofera, te nakon mjesec dana i poslije 3 mjeseca. Od toga: 70 dječaka (57,9%) i 51 djevojčica (42,1%). Prosječna starost: dječaka 20 mjeseci, a djevojčica 19 mjeseci. U ovom radu prikazali smo samo verijednosti hemoglobina kao najznačajniji parametar u dijagnostici anemije. Rezultati: Prosječna vrijednost hemoglobina prije uključivanja Legofera, kod dječaka, bila je 99,84 g/L, a kod djevojčica 98,59 g/L. Dok je nakon mjesec dana zabilježen porast prosječne vrijednosti hemoglobina kod dječaka na 107,59 g/L, kod djevojčica 110 g/l. Nakon tromjesečne terapije sa Legoferom vrijednost hemoglobina kod dječaka bila je 115,64 g/L, a kod djevojčica 117,84 g/L. Kada vrijednost posmatramo kod sve djece koja su zbog anemije primala Legofer, onda dobijemo


vrijednosti prije liuječenja 99,31 g/L, nakon mjesec dana108,6 g/L, a nakon 3 mjeseca 116,57 g/L. Značajno je da nijedno dijete nije isključeno iz ovog posmatranja radi neželjenih reakcija na lijek ili ne podnošenja lijeka. Rezultati analize djece kojima smo anemiju liječili Legoferom pokazali su da već nakon mjesec dana imaju značajan porast vrijednosti hemoglobina, a da nakon tri mjeseca Legofer postiže normalizaciju vrijednosti hemoglobina. Zaključak: Legofer se pokazao vrlo učinkovitim u liječenju anemija u male djece. Ovdje nije zabilježen ni jedan slučaj isključivanja djece iz analize zbog ne podnošenja lijeka ili neke neželjene reakcije na lijek.


How to improve metered dose inhaler technique outcomes for pediatric patients

Miskova S, Kirovski I, Micevska V, Seckova L, Sazdovski A.

University Children’s Hospital, Skopje, Macedonia

Asthma as one of the most common chronic illness in children is a condition often managed through the use of metered dose inhalers as an oral inhalation delivery device. Inhaled medications are vital in the treatment of childhood asthma, and effective only if they are used properly. Using an inhaler correctly delivers the medication to the lungs and leads to a better response. If the medication is used incorrectly, little or none of it reaches the lungs. Metered Dose Inhalers (MDIs) are a commonly prescribed delivery method for prescription medications in pediatric population. Asthma control rate in children depends on the regularity of conducting therapy, usage of proper device and correct performing of the treatment technique. Because of the potential difficulties in using the MDI properly, the usage of a spacer device is highly recommended. In order to manage good asthma control rate, it is necessary to provide MDI and spacer device technique education. In our clinical practice the most common reason of low asthma control rate is the use of incorrect technique for taking the inhaling medications (topic corticosteroids). To improve the MDI technique outcomes, we have to provide an education that is comprehensive, consistent, and includes written and oral instruction and demonstration by the provider with return demonstration by the patient and repeated check controls in addition.


Кардиохируршки третман на пациенти со вродени срцеви маани

Митрев Ж., Анѓушева Т, Милев И, Христов Н, Стоицоски Е, Алексовски Е Специјална Болница по хируршки болести Филип Втори, Скопје, Македонија Цел: Вродените срцеви мани штом пројават хемодинамски ефекти, можат да бидат третирани хируршки или пак со интервентни процедури. Типот на хируршката процедура и исходот на лекувањето зависи од специфичноста на вродениот дефект. Материјал: Во периодот од 01.03.2000 до 30.09.2015 година 1028 пациенти со вродени срцеви мани ( ВСМ). се лекувани во нашата установа. Од нив 522 (50,8%) се третирани со интервентни процедури и кај 498 (49,2%) се корегирани псо хируршки пристап. 760 (74%) беа со атријален септален дефект (ASD), 41 пациенти (4%) со тип венозус и парцијално белодробно венско вливање, 72 (7%) пациенти со парцијален АВ канал, 41 (4%) пациенти со пулмонална стеноза, 31 (3%) пациенти со Мb Ebstein, 31 (3%) со вентрикуларен септален дефект (ВСД),20 (2%) со перзистентен артериски дуктус, 20 (2%) со коарктација на аортата, 10 (1%) со Т.Fallot, 10 (1%) со хипертрофична опструктивна кардиомиопатија. И 1 пациент со тумор во десна комора. Во услови на хипотермија и вонтелесна циркулација беа оперирани 498, на пулсирачко срце 25, vo uslovi na atrijalna fibrilacija so poddr{ka od vontelesna cirkulacija bez kardioplagija 36 pacienti. Радикално беа кардиохируршки третирани 1017 (99%) од пациентите, а 9 (1%) палијативно. Pациентите со инфундибуларна пулмонална стеноза и ВСД беа рекоструirani со пач пластика. Сите пациенти беа проследени со пред/интра оперативна трансезофагеална ехокардиографија (ТЕЕ); кај 257 (25%) беше направена предоперативна селективна коронарографија и деснострана срцева катетеризација Резултати: Од 1028пациенти 431(42%) беа машки, а 597 (58%) од женски пол со X 39.5 ± 11 ; 308 (30%) пациенти биле на возраст помала од 18 години. 668 (65%) беа во NYHА III/IV. 328 (32%) беа со предоперативен мидикаментозен третман; транзиторни или перманентни ритам пореметувања имаа 287 (28%) пациенти. Со цијаноза беа 20 (2%);со сигнификантна пулмонална артериска хипертензија беа 339 (33%). Реоперации се направија кај 10 (1%) пациенти. Кај пациентите со ASD II кај 250 (56%) беше поставена директна сутура на дефектот, а кај останатите се постави пач-пластика. Кај 129 пациенти со АSD II беше извршена комбинирана операција од типот на затворање на дефектот со трикуспидна реконструкција, десна атриопластика. 15(1,5%) пациента беа во комбинација со хируршка реваскуларизација на миокардот. Просечно време на вентилација беше 4.5 ± 2 часа, просечен престој во одделението за интензивна нега беше 16 ± 6 часа, со вкупен болнички престој од 4.5 ± 2 дена. Беа регистирани следните рани компликации: респираторна инсуфициенција-35, крварења-15, аритмии-120. Ран морталитет (0,1%) (2пацienti). Регистрирани доцни компликации: аритмии кај 35 (3%), касен постторакотомен синдром (PPSy) кај 35 (3%).Стапка на преживување 99,9%, Заклучок: Правилниот пристап на навремената дијагностика,и комбинираниот медикаметозен, интервентен или кардиохируршки третман даваат добри клинички резултати и долговременско преживување.


The percent of newborns in the intensive care unit in correlation with the need and importance of the aformentioned intensive care unit

Mitrevska .S; Djarlieva.M; Ristevska.S

Department of neonatology, Clinical Hospital-Bitola

Aim: The goal of the study is to show the need for an adequate treatment and care for the newborns that are in a risky situation in the sense of saving their life and minimizing the damage. MATERIAL AND METHODS: The study covers and evaluated 1178 newborns born in 2014 and the first two months of 2015. RESULTS: Of the evaluated 1178 newborns , 541 of the newborns stayed in the intensive care unite for more than one day or 45,9%. From the gestation period in term were 470 newborns or 39% who needed to be in the intensive care unit or needed therapy in the intensive care unite, 71 newborn or 8,6% were premature. From the way of delivery 25% were with the caesarean section (20% were transitory with a one day stay) one was with a forceps delivery or 0.0848%, 30 newborns were with a vacuum delivery or 2,5%. From the diagnostic: 73 or 13% had an asphyxia, 37 or 6,8% had an infection, 26 or 4,8% were with HIE and HIK, 9 or 1,6 had congenital anomalies. CONCLUSION: The percent of newborns (45,9 %) that had the need of a special treatment of the intensive care unite is significant and shows the reason for the same, like an important link in saving the life of the newborns and the prevention of the potential sequelae in the vitally endangered newborns.


Brain death criteria in infants and children Mojsova MM, Spirovska T, Srceva JM, Sofijanova A Abstract Introduction Determination of brain death in term newborns, infants, and children is a clinical diagnosis based on the absence of neurologic function with a known irreversible cause of coma. Because of insufficient data in the literature, recommendations for preterm infants less than 37 weeks gestational age are not included. By our law adopted in 03.12 2013 year these are the examination criteria for determination of brain death in infants and children Examination criteria Appropriate patients for testing: Age >37 weeks gestational age to 18 years of age Temperature >35oC Normotensive without volume depletion .Blood pressure measured by indwelling arterial catheter is preferable. Hypotension is defined as systolic blood pressure or mean arterial pressure. Metabolic disturbances capable of causing coma should be identified and corrected. Patient should have a known irreversible cause of coma. Drug intoxication, neurotoxins, and chemical exposures should be considered in cases where a cause of coma has not been identified. Medications can interfere with the neurologic examination; sedatives, analgesics, antiepileptics, and neuromuscular blocking agents require adequate time for drug clearance (Drug elimination). Stop all such medications and allow adequate time for drug metabolism. Organ system dysfunction and hypothermia can alter drug metabolism. Obtain blood or plasma levels to confirm drug levels are in the low to midtherapeutic range. If elevated levels are noted, an ancillary test can be performed. Initial exam should be deferred for at least 24 hours after trauma or resuscitation event.

• Two examinations are performed by two different attending physicians.( clinical tests) • Apnea test( after the first clinical test) • Observation period • Preclinical or ancillary tests

Examinations are separated by an observation period. Term newborns (>37 weeks gestational age) to 2years: 24 hours Children >2 years to 18 years: 12 hours. Spinal reflexes may remain intact and do not preclude a determination of brain death. Presence of diabetes insipidus does not preclude a determination of brain death. Death is declared after the ancillary test. Our experience is very poor. During this two years we had diagnosed 3 brain death in children in our clinic. They were between 5-7 years. For the first one we did all the exams and for ancillary test we performed EEG. At that time we had parent’s approval to perform explantation but we had no waiting lists and HLA tipisation for children with end stage renal disease. For the second child we couldn’t perform EEG as an ancillary test so we did a panangiografia of the brain but the results was not consistent with brain death so we didn’t proceed. On the third child we performed the hole procedure with EEG for paraclinical test but we didn’t get approval from the parents. Conclusion: determination of brain death in infants and children is very demanding stressful and responsible procedure and we still need a lot of experience in order to make this procedure familiar to all the clinicians . Because the implications of diagnosing brain death are of great consequence, examination should be conducted by experienced clinicians who are familiar with neonates, infants and children and have specific training in neurocritical care. Key words: determination, brain death, examination criteria, infants, children


Алергија на храна и Атопичен Дерматитис во детската возраст Муча Ф., Муча В. ЈЗУ Општа Болница :Др.Ферид Мурад: Детско оделение-Гостивар,Р.Македонија Вовед; Атопискиот дерматитис (АД) е хронично кожно заболување и е едно од најчестите хронични болести кое што се карактеризира со интензивен еритематозен осип на кожата на лицето,која често е влажна и е пропратена со чешање.Се јавува во сите возрасни групи но доминира кај децата на доеначка возраст. Врз основа на присуство на атопија АД може да биде алергиска и неалергиска.Според разни трудови од различни автори од различни земји алергискиот дерматит доминира кај 50-80% .Алергијата на храна е најчестата причина за појава на Атопичниот дерматитис.

Цел на трудот:

Е да се одреди степенот на колерација меѓу атопискиот дерматит и исхраната.

Материјал и методи:

Испитани се 60 деца кои што беа лекувани на нашиот оддел поради атопичен дерматит.Кај сите нив беа изведени нутритивни алергиски тестови (прицк тест).Исто така беше проценета и личната и фамилијарната алергија.

Резултати:

Од испитаните деца највише беа машки деца (70%),позастапени се децата од градските населби (63 %), кај 70% од нив се јавува во доеначкиот период. Позитивна фамилијарна анамнеза имаа 90% од децата,кај двата родитела имаа 67 %,кај едниот родител 22% а кај 11% од децата алергија имало во поширокото семејство. Доени беа само 13% од децата.

Заклучок:

Нашите резултати покажаа дека АД е најчеста во доеначкиот период,почеста е кај машките деца и кај децата од градските населби,пофреквентна е кај пациенти со позитивна фамилијарна анамнеза за алергија.Исто така нашиот труд докажа дека ексклузивното доење е најдобра превенција на АД во доеначкиот период.


Bubble CPAP – two years experience

R.Muratovska-Delimitova, A.Sofijanova, Lj.Kojik, S. Naunova-Timovska, H.Mandzukova, M.Kimovska-Hristov, T.Voinovska, S.Jankovic

Intensive care unit, University Pediatric Clinic, Skopje, Macedonia

Abstract

Background: Continuous positive airway pressure (CPAP) is a method of delivering positive end-expiratory pressure with a variable amount of oxygen to the airway of spontaneously breathing patient to maintain lung volume during expiration, as to reduce atelectasis, respiratory fatigue and to improve oxygenation. Bubble CPAP (B-CPAP) was introduced more than 30 years ago for infants with respiratory distress. Bubbling in this method of CPAP, imitates High frequency ventilation, without invasive method of intubation. Since that time, there had been reports of decreased incidence of mechanical ventilation and chronic lung disease among the premature, as well as less failure of extubation.

Aim: This report describes how this treatment modality was adopted in our NICU and or experience in two years period.

Material and Methods: Retrospective study on 38 preterm infants (28-35 GW) treated with bubble CPAP in neonatal intensive care unit at University Pediatric Clinic in two years period from October 2013 – august 2015.This study was design to show our experience with this modality treatment .

Results: From 38 preterm (71%) 27 were male, (29%) 11 female. 10.5% (4) < 28 GW, 34.2% (13) 29-33 GW, 55.3% (11) 33-36 GW. For this study the patients were divided in two groups. Group A -10 patients (25.4%) were treated from the beginning with B-CPAP. In this group 10% (1) <27 GW (100% survival), 20% (2) 29-33GW (50% (1) survival, 50% (1) needed intubation and had pneumothorax as complication with lethal outcome, 70% (7) 33-36 GW (100% survival). In the second group 28 (73.6%) preterm were treated with bubble cpap after extubation , 7.9% (2) <28 GW with 100% survival, 32.1% (9) 29-33 GW (20% fail and needed reintubation), 60% (17)33-36 GW (100% survival).

Discussion

The results from this study, showed that this modality is associated with better survival in preterm due to less apnea, decreased time spent on mechanical ventilation, less lung injury and ventilator associated pneumonia, as well as improving in cardio-respiratory function after extubation .

Key words Bubble CPAP, prematurity, respiratory failure


Acute neonatal kidney injury associated with perinatal asphyxia

Naunova Timovska S., Sofijanova A., Kojik Lj., Kimovska Hristova M., Muratovska R., Voinovska T., Mandzukovska H., Neskova S., Timovski V. University Children’s Hospital – Skopje, PZU ″d-r Timovski″ - Stip ABSTRACT Objective: Acute kidney injury (AKI) is a frequent and serious clinical condition in neonatal intensive care units. It is a common consequence of perinatal asphyxia witch results from an inadequate intake of oxygen by the baby during the birth process. The other predisposing factors of neonatal kidney injury are prematurety, sepsis, and congestive heart failure. The aim of this study was to determine the predisposing factors associated to the neonatal acute kidney injury. Materials and Methods: The study was conducted at the University Children's Hospital in Skopje. It was clinical, prospective study. In the period from January 2013 to December 2014 were evaluated 29 patients hospitalized in the neonatal intensive care unit with documented neonatal acute kidney injury. We used medical data records of admitted neonates with kidney injury. The material was statistically processed using methods of descriptive statistics.

Results: This study evaluated the neonatal patients suffering kidney injury who at the period of 2 years were treated at the University Children's Hospital in Skopje. Out of 450 patients, total, 29 patients (6.4%) were diagnosed with acute kidney injury. According to the distribution by gender, dominated the group of male infants. The male to female ratio was 2.6:1. According to the distribution of gestational age, the majority of patients were infants born at term (66%). Perinatal asphyxia was the most common predisposing factor associated to kidney injury and was observed in 56% of the patients, with a predominance of males and term infants born with low Apgar score (in the first and fifth minutes of life). The remaining patients in our study presented with the following contributing conditions: sepsis(44%), prematurity(34%) and congenital malformations (8%). %). All of the patients included in this study had more than one contributing condition.

Conclusion: Acute kidney injury is a serious condition which damages the kidney as a central mediator of the homeostasis of bodily fluids and electrolytes. Perinatal asphyxia is a dominant predisposing factor associated to neonatal kidney injury. Often, the occurrence of kidney damage in the neonatal population is multifactorial, in that there are one or more associated comorbidities.

Key words: acute kidney injury, newborns, perinatal asphyxia


Transposition of the great arteries –Dg,treatment and aftersurgery experience of the cardioreumatology department from 2013 year

Marija Neskovska Sumenkovska, Rozana Kacarska,Konstandina Kuzevska Maneva, Beti Gurkova Angelovska , Valentina Jovanovska

Introduction

Transposition of the great arteries is complex heart failor where the great arteries a.pulmonalis and aorta have abnormal position of leaving the heart.They are leaving heart in parallel position instend of ususaly position of crossing blood vessels.A.pulmonalis have abnormal position behid the aorta and she doesen`t cross the aorta. Also aorta rise from right ventrcule and a.pulmonalis from left ventricule.

There are two groups of TGA:

1.L-transposition or corrected form

2.D-transpositin –complet form of transposition

In pediatric population is more common D-transposition

Method for diagnosing this diseases is with 2D echocardiography,X-ray and clinical findings (central cyanosis ,dispnea)

Final treatment is surgical,but till surgery is very important to keep open fetal communications FOA DAP

Materials and methods

In 2013 year on the department of cardioreumatology and together with American team of doctors are diagnosed and then surgically treated 5 cases of TGA.From five cases two were diagnosed like Single ventricle and TGA, and the rest 3 newborns were diagnosed only with TGA. All newborns were preoperative investigated.

Results

All five newborn were surgically treated by the American team of doctors and after surgery they are followed on our department again.One child was operated with Senning method and the rest four were with anatomical correction (SWITCH opp),following was with echocardiography and all were stabile and without complications.

Conclusion

Department for pediatric cardioreumatology want to present this cases,because we think that early diagnosis of this complex cardiopathy is lifesaving for the newborn,and will have good quality of live.


Practical approach to cough

Prof. Dr Sci med. Nestorovic Branimir Children's University Clinic Belgrade Cough reflex is very complex. It is controlled by a center in the spinal cord. Cough involves a

synchronized activity of a large number of respiratory muscles. The receptors are located from the pharynx to the bronchioles. Any irritation is leading to

activation of the reflex. After maximum inspire, follows forced expiratory flow (muscles of the abdominal wall are pushing the abdominal contents and the pressure is transmitted to thorax). With closedlarynx, pressure in the thorax reaches up to 300 mmHg. During opening of the glottis, air flow achieves very high rate (up to 600 liters per minute). This effectively removes mucous and infectious agents or foreign bodies from the airway.

Acute cough It lasts for about 3 weeks and is almost always related to previous acute viral infection. In

suppressing of this cough is most commonly used sedative antihistamines. As almost always the cough is affiliated with mucus hypersecretion, quite often are used mucolytic. Should be noted that they are contraindicated for children below 2 years of age.

Subacute cough It lasts from 3 to 6 weeks. It is likely that patients whichcontinue coughing after viral respiratory

tract infections have certain predisposition to lasting inflammatory changes that lead to hypersensitivity of the cough receptors.

Chronic cough It lasts more than 6 weeks, and the two most common causes are postnasal secretion runoff and

asthmatic cough. A particular problem is that relatively large number of patients has multiple causes for coughing.

Literature Canning BJ.:Anatomy and neurophysiology of the cough reflex: ACCP evidence-based clinical

practice guidelines. Chest. 2006 Jan;129(1 Suppl):33S-47S. Undem BJ, Carr MJ.:Targeting primary afferent nerves for novel antitussive therapy. Chest. 2010

Jan;137(1):177-84. Bruce K, Rubin M, EngrM :Mucolytics, Expectorants, and Mucokinetic Medications [Respir Care

2007;52(7):859 – 865

http://www.ncbi.nlm.nih.gov/pubmed?term=Canning%20BJ%5BAuthor%5D&cauthor=true&cauthor_uid=16428690

http://www.ncbi.nlm.nih.gov/pubmed/16428690

http://www.ncbi.nlm.nih.gov/pubmed?term=Undem%20BJ%5BAuthor%5D&cauthor=true&cauthor_uid=20051402

http://www.ncbi.nlm.nih.gov/pubmed?term=Carr%20MJ%5BAuthor%5D&cauthor=true&cauthor_uid=20051402



Fungal rhinosinusitis

Jane Netkovski, BiljanaShirgoska, Elena TrajkovskaDokic

University clinic of otorhinolaryngology, Medical faculty - Skopje, Macedonia

Institute of microbiology and parasitology, Medical faculty – Skopje, Macedonia

Classification of fungal rhinosinusitis is founded on the immunologic relationship of the fungus to the host. There are two basic types of fungal disease: invasive and noninvasive. Within the invasive division are acute invasive and chronic invasiverhinosinusitis. Within the noninvasive division are saprophytic colonization,fungus ball (mycetoma), and allergic fungal rhinosinusitis (AFRS). AFRS is the most common fungal infestation.

Many cases of fungal rhinosinusitis are caused by species of Aspergillus, Alternaria, Cladosporium, Mucor, Rhizomucor, Rhizopus but the range of confirmed and potential fungal agents is extensive and expanding.

Patients of noninvasive division present with symptoms of chronic sinusitis, which may include facial pressure, headache, nasal stuffiness, discharge, and cough.Patients with acute invasive rhinosinusitisare usually hospitalized and are very sick with fever, cough, nasal discharge, headache, and mental status changes.Chronic invasive rhinosinusitis patientsare presented with symptoms of long-standing sinusitis.

In the diagnosis of fungal infections, the following laboratory strategies may be employed: microscopic examination of fresh clinical specimen or histopathologic preparations; culture of clinical material; serology and skin testing; radiographic techniques and polymerase chain reaction (PCR) methods to detect specific fungal DNA in clinical specimens

The treatment for invasive fungal sinusitis, whether acute or chronic, is first the reversal of the source of immunocompromised, if present, followed by systemic antifungal therapy and surgical debridement. Immunocompetent patients with fungus ball are treated best with surgical removal of the fungus ball. There is a very low rate of recurrence, and systemic antifungal therapy is not required. The only therapy for saprophytic fungal colonization is mechanical endoscopic cleaning of nasal crusts or nasal saline irrigation. The surgical management of AFRS consists of conservative surgical exenteration of all fungal containing mucin, with systemic peri and postoperative steroids to downregulate the hypersensitivity reaction. The role of fungal containing immunotherapy is promising modality in preventing recurrence of this recalcitrant disease.


Evaluation of rapid serologic test in diagnostics of celiac disease

Nikcevska N, Kostovski A, Bojadzieva S., Grujovska S., Todorovski G.

University Children’s Hospital Skopje, Department of Gastroenterohaepatology

INTRODUCTION

Coeliac disease (CD) is a systemic, imunomodulated, disease caused by gluten and related prolamines, in genetically predisposed individuals. It is characterized by a variable combination of gluten-dependent clinical manifestations, specific antibodies for CD, HLA-DQ2 or HLA-DQ8 haplotype, and usually enteropathy. Specific antibodies for CD include tissue transglutaminase (t-TG2), endomysial (a-EMA), and the antibodies against deamidated gliadin peptide (a-DGP).

AIM

To determine the effectiveness serologic tests in the diagnostic algorithm of CD, used the University Clinic for Children's Diseases in Skopje, the department Gastroenteroepatology, in the period 2008-2012, compared to the effectiveness combined with small-intestinal biopsy.

MATERIALS AND METHODS Retrospective study analysis of specificity and sensitivity for the a-tTG IgA test in the diagnosis of CD. Patients grouped according to gender, symptomatology, and risk for development of CD. Analyses of serological tests were compared with analysis of histology findings

RESULTS During five years of using a-tTG IgA antibodies, total of 417 tests were done. Of these, positive results in 36 patients. In 20 of the positives, biopsies made with histology diagnosis, 19 confirmed CD. The sensitivity of the rapid immunochromatographic test for quantitative determination of a-tTG IgA antibody is 100%, specificity 99.74%; positive predictive value of 95%.

CONCLUSION Methods for screening, diagnosis, improved diagnostic protocol for CD are in the scientific focus always. Specific serological tests represent especially indispensable base in the treatment for a patient with CD for its high specificity, sensitivity, ease of performance and cost benefit

Key words: coeleiac disease, a-tTG antibody, serology tests, duodenal biopsy


Tufting entheropathy case report Nikcevska N., Bojadzieva S., Kostovski A., Grujovska S., Todorovski G. University Children’s Clinic Skopje, Department of Gastroenterohaepatology INTRODUCTION

Tufting entheropathy (TE) is an autosomal recessive disease. Characterized with severe diarrhea, malapsorption, electrolyte disturbances, poor weight gain, liver damage, necessity of parentheral nutrition (PN). Prevalence is 1 in 50 000-100 000.

CASE REPORT

Male, born spontaneously, in term, with 3750 gr, 52 cm, with Respiratory distress, treated 3 days at ICU. Discarded stabile; weight gain of 70 gr, good breastfeeding.

Symptoms start at 3 months, with persistent watery diarrhea (>10/day), poor weight gain. Physical examination showed dehydration, weight 6200 gr. He was breastfed. Mother put on a diet without milk; but no effect seen, nor with extensive hydrolysate, or amino acid formula. Marked acidosis ( Ph 7.32 , HCO3 14.1, BE-9.7), elevated liver enzymes (AST 152, ALT 61, GGT 52 LDH 431 U/L), and low IgA < 0.25 g/l. Complications occurred, with МRSA , Streptococus Viridans sepsis. Fed with adsorbents, meat bujon, no milk. After upper gastroendoscopy, corticosteroid therapy was given. Histology with showed variable abnormalities of villi, disorganization and tufts. PN through peripheral vein given. After parents consent CVK placed. This resulted in weight gain (2 kg for 2, 5 months), overall improvement. The child now has 13 month, 8300 gr.

DISCUSSION TE is a very rare condition; the diagnosis is extremely complicated. Longterm prognosis is dubious, mortality high. This is the only pediatric case of TE, proven by electron microscopy in Macedonia. Key words Tufting entheropathy, intractable diarrhea


Rational Phytotherapy in respiratory pathology Assoc. Prof. Nikolova The Herbal medicinal product Prospan has a proven mode of action and affects the regulation processes of the β2-adrenergic receptors. Prospan increases the numbers of the β2-adrenergic receptors on the cell surface. Besides the well -known secretolytic, bronchospasmolytic and cough relieving effects, Prospan is also proven to have anti-inflammatory effect. It has been established for the mode of action that the increase of the exocytosis of surfactant by the lung epithelial cells (alveolar epithelial cells type II) determines the secretolytic effect, reduces mucous viscosity, decreases cough in frequency and intensity. The reduced intracellular levels of calcium ions leads to the relaxation of the bronchial muscles. It has been proven that Prospan indirectly increases β2- adrenergic effects, influences regulation processes of the β2- adrenergic receptors, prevents endocytosis of the β2-receptors and increases the number of active receptors on the cell surface. A number of clinical studies have been carried out on acute and chronic inflammatory lung disorders in children and adults, which shows that Prospan is exceptionally well tolerated and therefore Prospan syrup is registered in Bulgaria as suitable for all patients including newborn babies (Rx < 1 year > OTC). Prospan can also be used as adjunctive therapy in patients with bronchial asthma


Clinical examination the patient with Single ventricle,TGA,Stenosis a.pulmonalis-Case report

D.Nonkulovski,R.Kacarska,K.Kuzevska-Maneva,V.Jovanovska,B.Gjurkova,M.Neshovska-Shumenkovska,G.Paskalov,M.Xhaferi,M.Kacarska,I.Petkovska-Ivanovska,J.Gjorgievska

Department of Cardiology-University Children’s Hospital,Skopje,Macedonia

Backround and aims :A single ventricle is defined as the presence of two atrioventricular

valves with one ventricular chamber or a large dominant ventricle associated with a diminutive opposing ventricle.The most common form of single ventricle is a single left ventricle with L-transposition of the great arteries, with the aorta arising from a diminutive, leftward right ventricle and following the leftward, ascending pattern characteristic of corrected transposition

Methods: We present one year child with diagnosis:St.post opp.:single ventricle (Glenn procedure),TGA,Stenosis a.pulmonalis,DAP that ever since had been cliniclly followed up on a regular base at our Clinic.During the hospitalizacion following detailed clinical,laboratory and echocardigrahpy elavuation.Result: Central cyanosis is noted at initional day of life, echocardiographic evaluation the newborn ist additionally diagnosed with Single ventricle with TGA, DAP.Sevent month of life the Cardisurgery team from USA surgical treated the child with Glenn procedure, with good postoperative results.

Conclusion: This population of patients is complex and difficult to manage. Surgery is clearly indicated in ill newborns with pulmonary atresia or heart failure), and it is reasonable to follow the previously outlined management plan of

catheterizations and surgeries Patients whose circumstances create quite difficult clinical decisions include those who are very stable with a shunt or pulmonary stenosis (congenital or due to a pulmonary artery band), normal pulmonary artery pressure and anatomy, and a pulmonary/systemic flow ratio of 2:1 and aortic saturation about 85%. In these there is some evidence to suggest that an early bidirectional Glenn shunt/ Fontan rather than continued medical follow-up may be better.


Molecular analysis of glucose-6-phosphate dehydrogenase deficiency in families from the

Republic of Macedonia and correlation with phenotype

Anet Papazovska Cherepnalkovski1, Vjekoslav Krzelj2,Sofijanka Glamocanin1, Katica Piperkova1,

Tatijana Zemunik3, Svetlana Kocheva1, Biljana Coneska Jovanova1, Natasa Aluloska1, Nikolina

Zdraveska1. 1University Pediatric Clinic, Medical Faculty, University "St. Cyril and Methodius”, Skopje, Republic of

Macedonia 2University Hospital Split and School of Medicine, University of Split, Croatia 3Department of MedicalBiology, School of Medicine, University of Split, Croatia

G6PD deficiency is the most common enzymopathy affecting more than 400 million people

worldwide.More than 400 enzyme variants have been described based on their biochemical

properties and grouped into five classes. Molecular analysis of the G6PD gene identified 191

mutations or combination of mutations. G6PD Mediterranean is the most common variant found in

populations of the Mediterranean area. The aim of our study was to perform molecular analysis of

G6PD deficiency in families from the Republic of Macedonia and correlate the findings to clinical

presentation. Six patients and seven other family members were selected for genetic analysis, the

selection procedure involved clinical and laboratory evaluation as well as G6PD quantitative

spectrophotometric testing. All patients were first screened for the Mediterranean mutation, and

subsequently for the Seattle mutation. Mutations were detected using PCR amplification and

appropriate restriction endonuclease cleavage. Four hemizygotes and 3 heterozygous carriers for

G6PD Mediterranean were detected. None of the patients tested positive for G6PD Seattle

mutation. All G6PD deficient patients from this group showed clinical picture of hemolysis, and in

66.6% neonatal jaundice was confirmed based on history data. This study represents a step towards

a more comprehensive genetic evaluation in our population and better understanding of the health

issues involved.


Corticosteroids in the treatment of pediatric allergic rhinitis

V. Pavloski; J. Netkovski; B. Shirgoska

University ENT Clinic, Skopje

Introduction: Allergic rhinitis (AR) is a common childhood disease whose prevalence is increasing. The symptoms of AR can have profound effects on the quality of life. Furthermore, the presence of comorbid conditions such as asthma, sinusitis, and otitis media with effusion can complicate both the clinical presentation and the treatment of AR.

Purpose: The goal of treatment in pediatric allergic rhinitis is to provide effective prevention of or relief from allergic rhinitis symptoms as safely and effectively as possible.

Materials and Methods: The newer corticosteroids (CS), including mometasone furoate (MF), fluticasone propionate (FP), and budesonide (BUD) have an improved risk-benefit ratio compared with older cortico-steroids and are now considered the drug of choice for pediatric allergic rhinitis. A new intranasal corticosteroid, MF nasal spray, has been studied in children 3 to 12 years of age and has been shown to be effective.

Results:Comparing with older CS molecules, the risk of systemic effects is much lower with the newer molecules such as BDP, triamcinolone acetonide (TAA), flunisolide (FLU), BUD, fluticasone propionate (FP), and MF. These concerns are further heightened in the treatment of pediatric AR, where the benefits of appropriate therapy must be weighed against the risks of what is likely to be long-term INCS administration. These safety issues may be compounded by the presence of concomitant allergic or respiratory disorders, particularly asthma, in which corticosteroids are also the most effective therapeutic agents.

Conclusion: Topical corticosteroids are extremely effective and have an excellent safety profile. Measurement of growth in children receiving topical corticosteroids should be routine practice in every asthma and rhinitis clinic, together with peak flow. Allergen avoidance should be the initial treatment in allergic diseases, with pharmacotherapy added as necessary. Because of their good anti-inflammatory activity, poor absorption, and first-pass hepatic metabolism, second-generation topical corticosteroids are the treatment of choice for patients with allergic rhinitis.

Keywords: Allergic rhinitis, Intranasal corticosteroids.


Coverage of the nurseries in the republic of macedonia with the neonatal thyroid screening during the 2002-2014

Milica Pesevska, Violeta Anastasovska, Elizabeta Taseva and Mirjana Kocova Genetic Laboratory, Department of Endocrinology and Genetics, University Children’s Clinic, Skopje, Republic of Macedonia Background: Neonatal thyroid screening is a sensitive and reliable tool for detection of congenital hypothyroidism. It was introduced as nationwide screening program in 2007, after five years pilot study. There is one screening center, located at the University Children’s Clinic in Skopje which covers the newborns from the whole country.

Method: The detection of CH consists of fluoroimmunometric assay (DELFIA, Wallac) for neonatal thyroid-stimulating hormone (TSH) from blood spots on filter paper (Whatman 903), obtained from newborns of all 31 nurseries, 48 hours after birth. Cut of value was 10 mU/L. Coverage of the screening is calculated comparing the number of screened newborns with the number of liveborn children during a year.

Results: Out of 236,830 newborns 227,661 have been screened, during the period 2002-2014. Of them, more than 70% were born in the bigger nurseries: 24.88% University Clinic for Gynaecology and Obstetrics - Skopje, 17.27% Special Hospital for Gynaecology and Obstetrics ,,Mother Teresa’’- Skopje, 8.05% nursery in General Hospital Tetovo, 6.72% Bitola, 5.43% Kumanovo, 4.39% Strumica and 4.05% Acibadem Sistina Hospital - Skopje. The coverage was 96.13% average, ranging from 86.25% in the birth center - Radoviš to 99.49% in the General Hospital Remedika.

In 96.77% nurseries the coverage was above 92%, in 32.26% nurseries above 97%, in 9.68% above 98% and in 6.45% above 99%.

Conclusion: The national neonatal thyroid screening program has been successful. The coverage is satisfactory except for several smaller nurseries. Additional education of the personnel in these nurseries is warranted.

Key words: Neonatal TSH screening, congenital hypothyroidism


Adiponectin level and insulin resistance in obese children

Milica Pesevska, Violeta Anastasovska and Mirjana Kocova Genetic Laboratory, Department of Endocrinology and Genetics, University Clinic for Childrens’ Diseases, Skopje, Republic of Macedonia

Background: There is global increase in the prevalence of obesity accompanying with diabetes mellitus type 2, insulin resistance and cardiovascular diseases. Adiponectin is a hormone of adipose tissue that has anti-inflamatory and insulin-sensitizing (antidiabetic) effect.

Aim: to examine the association of adiponectin and insulin in obese children.

Methods: Adiponectin levels were examined in 94 obese children with Body mass index (BMI) 30.74±5.18 kg/m2 and control group of 56 healthy children with BMI 17.51±3.60 kg/m2. The adiponectin concentrations were measured with Enzyme-Linked Immunosorbent Assay (ELISA) method while the level of insulin was measured with the immunoturbidimetry method.

Results: The adiponectin levels in obese children were 9.62±4.60 ng/ml and were significantly lower compared to the healthy children 13.6±6.43 ng/ml, (p<0.05). Hyperinsulinemia is noticeable in obese children 94.64±80.97 IU/ml vs. 17.5±3.5 IU/ml in controof adipose tissue. There was significant negative correlation between adiponectin levels and insulinemia (r= -0.234; p=0.03) but there was no statistically significant correlation between adiponectin on one side and BMI and age on the other (p>0.05).

Conclusions: The pathophysiology behind the obesity involves the increase of adipose tissue, being inversely related to adiponectin, but directly related to insulin resistance and metabolic syndrome. Reduced serum adiponectin level is a risk factor for developing cardiovascular complications, and evaluation of its level may influence the risk evaluation in obese children.

Keywords: obese children; adiponectin; insulin resistance


Early-onset neonatal sepsis: new insights of an old puzzle Elizabeta PETKOVSKA Department of Neonatal Intensive Care and Therapy, University Clinic of Gynecology and Obstetrics, Skopje, Republic of Macedonia Summary Sepsis is common and devastating problem in the NICU. Although advances in neonatal care have improved survival and reduced complications in preterm infants, sepsis still contributes significantly to mortality and morbidity among VLBW infants in NICUs. Based on the timing of the infection early-onset sepsis (EOS) is defined as onset of sepsis in the first 3 neonatal days and is mostly the result of vertical transmission of bacteria from mothers to infants during the intrauterine and intrapartum period. The most common pathway of intrauterine infection is the ascending route. Intrauterine infection develops through four stages. Rupture of the membranes is not obligatory for occurrence of chorioamnionitis because microorganisms can cross intact membranes. The most threatening is stage IV which may be resulted with fetal bacteremia and sepsis. GBS and E. coli together account for about 70% of cases of EOS in the neonatal period. Chorioamnionitis is a major risk factor for neonatal sepsis and its incidence varies inversely with gestational age. The signs and symptoms of neonatal sepsis are nonspecific. Since sepsis is a systemic inflammatory response to infection, isolation of bacteria from blood is considered the gold standard for the diagnosis of sepsis. A lumbar puncture should be performed in infants with a positive blood culture, infants with a high probability of sepsis on the basis of clinical signs or abnormal laboratory data, or infants who do not clinically improve when treated with appropriate antimicrobial therapy. Available diagnostic testing is not helpful in deciding which neonate requires empirical antimicrobial therapy but can assist with the decision to discontinue treatment. The challenges for clinicians are threefold: identifying neonates with a high likelihood of sepsis promptly and initiating antimicrobial therapy; distinguishing “high-risk” healthy-appearing infants or infants with clinical signs who do not require treatment; and discontinuing antimicrobial therapy once sepsis is deemed unlikely. The IAIP has potential role as a theranostic biomarker. Key Words: Early-onset sepsis (EOS), intrauterine infection, chorioamnionitis, prematurity, very low birth weight (VLBW) infants, NICU, biomarkers Introduction Systemic bacterial infection in the newborn creates a significant burden due to its impact on neonatal mortality and long-term morbidity. In spite of ongoing efforts in early diagnosis, treatment, and prevention, neonatal sepsis still remains an enigmatic area for neonatologists due to changes in epidemiology and the lack of ideal diagnostic markers (1). Despite multiple failed attempts to reduce the burden of infection neither the treatment of neonatal sepsis, nor the neurodevelopmental outcomes in surviving infants has changed significantly over the last thirty years (2, 3). These disappointments have occurred in the context of tremendous advances in other areas of newborn care including nutrition, management of respiratory distress and pulmonary hypertension, and therapeutic cooling following hypoxic-ischemic encephalopathy. Incidence Neonatal sepsis remains one of the leading causes of morbidity and mortality both among term and preterm infants (4). Although advances in neonatal care have improved survival and reduced complications in preterm infants, sepsis still contributes significantly to mortality and morbidity among very-low-birth-weight (VLBW, <1500 g) infants in Neonatal Intensive Care Units (NICUs) (5, 6). In developing countries the reported incidence of neonatal sepsis varies from 7.1 (7) to 38 (8) per 1000 live births in Asia, from 6.5 (9) to 2315 per 1000 live births in Africa, and from 3.5 (10) to 8.9 (10) per 1000 live births in South America and the Caribbean. By comparison, rates reported in the United States and Australasia range from 1.5 to 3.5 per 1000 for EOS sepsis and up to 6 per 1000 live births for LOS sepsis, a total of 6–9 per 1000 for neonatal sepsis (12-15).


Even with timely antimicrobial treatment almost 40% of those affected faced death or major disability (16). The incidence of severe sepsis in newborns doubled (from 4.5 to 9.7 cases per 1,000 births) between 1995 and 2005 (17). The frequency of sepsis during the birth hospitalization varies inversely with gestational age at birth and may reach 60% in the most immature infants (18). The problem with definition Based on the timing of the infection neonatal sepsis has been classified into early-onset sepsis (EOS) and late-onset sepsis (LOS) (19). This classification helps to guide antibiotic therapy as it implies differences in the presumed mode of transmission and predominant organisms. EOS is defined as onset of sepsis in the first 3 days and is mostly the result of vertical transmission of bacteria from mothers to infants during the intrauterine and intrapartum period. To date, the accuracy of the pediatric consensus definitions for sepsis established in 2005 has not been assessed in preterm infants, nor have consensus definitions been developed or tested in this unique developmentally immature population (20).Thus, consensus definitions for sepsis in term and preterm infants are needed so that future epidemiologic studies and trials of diagnostic and therapeutic interventions can be interpreted and implemented. Definitions are also needed to help clinicians in determining true sepsis and providing appropriate clinical care (21). Pathogenesis and epidemiology Pathways of developing intrauterine infection Microorganisms may gain access to the amniotic cavity and fetus through the following pathways: ascending from the vagina and cervix; hematogenous dissemination through the placenta (transplacental infection); retrograde seeding from the peritoneal cavity through the fallopian tubes; and accidental introduction at the time of invasive procedures, such as amniocentesis, percutaneous fetal blood sampling, chorionic villous sampling or shunting (22–25). The most common pathway of intrauterine infection is the ascending route (26). Stages of developing intrauterine infection Intrauterine infection develops through four stages (26). The first stage includes a change of the vaginal and cervical microbial flora or the presence of pathological organisms in the cervix. Some forms of bacterial vaginosis may be an early manifestation of stage I. Once microorganisms gain access to the intrauterine cavity, they colonized decidua (stage II) and caused inflammatory reaction which leads to deciduitis. Microorganisms may then settle in the chorion caused choriovasculitis and shift through the amnion caused amnionitis into the amniotic cavity, leading to microbial invasion of the amniotic cavity or an intraamniotic infection (stage III). Rupture of the membranes is not obligatory for occurrence of chorioamnionitis because microorganisms can cross intact membranes. Once in the amniotic cavity, the bacteria may gain access to the fetus by different ports of entry (stage IV). Congenital pneumonia may occur by fetal aspiration of the infected fluid and conjunctivitis, omphalitis and otitis by direct dissemination of microorganisms from infected amniotic fluid. Crossing from any of these sites to the fetal circulation may be result with fetal bacteremia and sepsis. Microbiology of intraamniotic infection A gram-positive encapsulated bacterium Group B streptococcus (GBS) is the leading cause of neonatal sepsis and meningitis in the United States. Stoll et al. has recently described Escherichia coli (E. coli) to have emerged as the major pathogen of neonatal sepsis in preterm infants and the second most common cause in term infants (27). E. coli is frequently associated with severe infections and meningitis and is the leading cause of sepsis related mortality among VLBW infants (24.5%) (28, 29). GBS and E. coli together account for about 70% of cases of EOS in the neonatal period (19, 30). Chorioamnionitis is a major risk factor for neonatal sepsis. Sepsis can begin in utero when the fetus inhales or swallows infected amniotic fluid. The neonate can also develop sepsis in the hours or days after birth when colonized skin or mucosal surfaces are compromised. The essential criterion for the clinical diagnosis of chorioamnionitis is maternal fever. Other criteria are relatively insensitive.


When defining intra-amniotic infection (chorioamnionitis) for clinical research studies, the diagnosis is typically based on the presence of maternal fever of greater than 38°C (100.4°F) and at least two of the following criteria: maternal leukocytosis (greater than 15 000 cells/mm3), maternal tachycardia (greater than 100 beats/minute), fetal tachycardia (greater than 160 beats/minute), uterine tenderness, and/or foul odor of the amniotic fluid. These thresholds are associated with higher rates of neonatal and maternal morbidity (31).

The incidence of clinical chorioamnionitis varies inversely with gestational age. In the National Institute of Child Health and Human Development Neonatal Research Network, 14% to 28% of women delivering preterm infants at 22 through 28 weeks’ gestation exhibited signs compatible with chorioamnionitis (32). The major risk factors for chorioamnionitis include low parity, spontaneous labor, longer length of labor and membrane rupture, multiple digital vaginal examinations (especially with ruptured membranes), meconium-stained amniotic fluid, internal fetal or uterine monitoring, and presence of genital tract microorganisms (eg, Mycoplasma hominis) (33). The most common microbial isolates from the amniotic cavity from women with preterm labor and intact membranes are genital tract microorganisms Ureaplasma urealyticum, Fusobacterium spp. and Mycoplasma hominis (26). There are emerging evidences that Mycoplasma and Ureaplasma have ability not only to invade and infect placental and fetal tissue, inducing inflammatory cytokines and triggering pathways leading to PROM, chorioamnionitis and preterm labor, but also associate with late abortion, early onset sepsis, intraventricular hemorrhage, and bronchopulmonary dysplasia (34-37). In the meta-analysis of Silva MJ et al concluded that Chlamydia infection during pregnancy increased risk of preterm labor (relative risk (RR) = 1.35 [1.11, 1.63]), low birth weight (RR = 1.52 [1.24, 1.87]) and perinatal mortality (RR = 1.84 [1.15, 2.94]) (38). The major risk factors for early-onset neonatal sepsis are preterm birth, maternal colonization with GBS, rupture of membranes >18 hours, and maternal signs or symptoms of intra-amniotic infection. (39-41) Other variables include ethnicity, low socioeconomic status, male sex, and low Apgar scores. Preterm birth/low birth weight is the risk factor most closely associated with early-onset sepsis. (42) Infant birth weight is inversely related to risk of early-onset sepsis. The increased risk of early-onset sepsis in preterm infants is also related to complications of labor and delivery and immaturity of innate and adaptive immunity (43). Clinical sings The signs and symptoms of neonatal sepsis are nonspecific (44). These include fever or hypothermia, respiratory distress including cyanosis and apnea, feeding difficulties, lethargy or irritability, hypotonia, seizures, bulging fontanel, poor perfusion, bleeding problems, abdominal distention, hepatomegaly, gauiac-positive stools, unexplained jaundice, or more importantly, “just not looking right” (45, 46). Infants with hypoxia–acidosis may gasp in utero and lead to pneumonia and meconium aspiration (47). Diagnostic testing Biomarkers of Neonatal Sepsis Since sepsis is a systemic inflammatory response to infection, isolation of bacteria from blood is considered the gold standard for the diagnosis of sepsis (48). Sepsis cannot always be excluded even when blood cultures are found to be negative. Conversely, isolation of bacteria in a blood culture may reflect asymptomatic bacteremia or contamination. One milliliter of blood drawn before initiating antimicrobial therapy is needed to adequately detect bacteremia if a pediatric blood culture bottle is used. Cultures of superficial body sites, gastric aspirates, and urine are of no value in the diagnosis of early-onset sepsis (40). A lumbar puncture should be performed in infants with a positive blood culture, infants with a high probability of sepsis on the basis of clinical signs or abnormal laboratory data, or infants who do not clinically improve when treated with appropriate antimicrobial therapy (50).

To date, none of the biomarkers including hematologic indices, acute phase reactants (C reactive protein (CRP) and procalcitonin (PCT), cytokines (interleukin-1β (IL-1β), interleukin-6 (IL-6),


interleukin-8 (IL-8), interleukin-2 soluble receptor (SIL2R), and tumor necrosis factor α (TNF-α), and cell surface markers (CD11β and CD64) have shown sensitivity, specificity, positive and negative predictive value that are sufficiently powerful to guide the clinical management of EOS (44, 51). Non-culture dependent methods based on proteomics, in situ hybridization, gene arrays, mass spectroscopy, and polymerase chain reaction (PCR) methods to screen blood for bacteria, and other supplemental diagnostic tests based on evaluation of the immune system are being evaluated to help resolve ambiguities in these situations (52). The limitations of these studies includes failure to provide information about antibiotic resistance, inability to differentiate the false-positive results because of potential contamination during blood sampling or processing from true positive cases and high cost. Prospective evaluation is needed to determine accuracy and safety of these exciting new approaches.

Inter α inhibitor proteins (IAIP) The IAIP are serine protease inhibitors which provide protection from the increased protease activity associated with systemic immune system activation that accompanies sepsis and inflammation. They are involved in extracellular matrix stabilization, inflammation, wound healing, and play an important anti-inflammatory and regulatory role in infection (53). IAIP is one of the important serine protease inhibitors secreted by the liver, which concentration is independent of gestational age, postnatal age, and is similar to adult levels (54). Compared with non-septic age matched controls, IAIP levels are significantly lower in septic neonates In a pilot study of 573 neonates, receiver operating curve analysis has shown IAIP measurement to have a sensitivity of 89.5%, a specificity of 99%, a positive predictive value of 95% and a negative predictive value of 98% (55), Because the levels of IAIP decrease with severe sepsis, measurement may also help to guide the prognosis as lower levels are associated with adverse outcome (56). In response to antibiotic treatment the levels of IAIP rise (54). Singh et al. showed an immune-modulatory and protective role of administration of IAIP in a septic newborn mice (57). This potential role of IAIP as a theranostic marker in infants with sepsis can be use as powerful new tool of personalized medicine.

Prophylaxis and treatment The only intervention proven to decrease the incidence of early-onset neonatal GBS sepsis is maternal treatment with intrapartum intravenous antibiotics. Adequate prophylaxis is exposure to penicillin (preferred agent), ampicillin, or cefazolin given for >4 h before delivery (58, 59). Erythromycin is no longer recommended for prophylaxis because of high resistance rates. In those women with a non-serious penicillin allergy, cefazolin is the drug of choice. For a mother with a history of life-threatening penicillin allergy (anaphylaxis, rash, angioedema, respiratory symptoms), clindamycin is the substitute for penicillin, but should only be used if the rectovaginal GBS isolate is tested and found to be susceptible.A potential increase in false-negative neonatal blood cultures as well as sepsis caused by pathogens other than GBS is a potential concern with the extensive use of maternal intrapartum antibiotics. Such a change would be important as several studies have demonstrated increased severity of disease and risk of death in the neonates with gram-negative infections (60). In a study conducted by the NICHD between 1998 and 2000 on 5447 VLBW infants there was a significant reduction in the incidence of EOS caused by GBS. However, there was also a significant increase in the proportion of E. coli infections among VLBW infants (61). Though there has been a dramatic decline in the incidence of EOS due to GBS, the increasing incidence of ampicillin-resistant neonatal sepsis among VLBW infants is concerning (62). Nonetheless, the benefits from the use of antepartum antibiotic chemoprophylaxis still offset the risks of resistant bacterial infections (63).

Antibiotics for preterm labor with premature rupture of membranes (PROM) Premature rupture of membranes is strongly associated with infection of the amniotic membranes, and this infection is independently related to preterm birth, (65) cerebral palsy and chronic lung disease (64). A Cochrane review showed antibiotic treatment for PROM led to reductions in the proportion of babies born within 48 hours (RR 0.71; 95% CI 0.58-0.87), and reduced neonatal infections (RR 0.68; 95% CI 0.53-0.87), surfactant use (RR 0.83; 95% CI 0.72-0.96), oxygen therapy (RR 0.88; 95% CI 0.81-


0.96), and abnormal cerebral ultrasound scans prior to hospital discharge (RR 0.82; 95% CI 0.68-0.98) [170]. No differences in long-term follow-up were observed in the babies of intervention and control groups (67). The quality of evidence on morbidity and mortality is high and the intervention is strongly recommended to be scaled up to improve preterm survival (68). Gomez R, et al. concluded that antibiotic administration (ceftriaxone, clindamycin, and erythromycin) rarely eradicates intra-amniotic infection in patients with preterm PROM; intra-amniotic inflammation developed in one-third of patients who did not have inflammation at admission, despite antibiotic administration; a sub-group of patients with documented inflammation of the amniotic cavity demonstrated a decrease in the intensity of the inflammatory process after antibiotic administration (69).The optimal treatment of infants with suspected early-onset sepsis is broad-spectrum antimicrobial agents (ampicillin and an aminoglycoside). Once the pathogen is identified, antimicrobial therapy should be narrowed (unless synergism is needed) (49). Antibiotics may be discontinued in well-appearing term newborn infants born to women with chorioamnionitis by 48 hours of life; treatment of 72 hours might be considered for infants with greater degrees of prematurity or abnormal screening studies (50).

Clinical challengeThe clinical diagnosis of sepsis in the neonate is difficult, because many of the signs of sepsis are nonspecific and are observed with other noninfectious conditions Although a normal physical examination is evidence that sepsis is not present. (70, 71) bacteremia can occur in the absence of clinical signs (72). Available diagnostic testing is not helpful in deciding which neonate requires empirical antimicrobial therapy but can assist with the decision to discontinue treatment

(73). Most infants with suspected sepsis recover with supportive care (with or without initiation of antimicrobial therapy). Sepsis screening tests might be of value in deciding which “high-risk” healthy-appearing neonates do not need antimicrobial agents or whether therapy can be safely discontinued. The diagnosis and management of neonates with suspected early-onset sepsis are based on scientific principles modified by the “art and experience” of the practitioner. The challenges for clinicians are threefold: identifying neonates with a high likelihood of sepsis promptly and initiating antimicrobial therapy; distinguishing “high-risk” healthy-appearing infants or infants with clinical signs who do not require treatment; and discontinuing antimicrobial therapy once sepsis is deemed unlikely (40).

Conclusion In an ideal world, the practice of medicine would be based on scientific studies guiding the decisions involved in the care of an individual patient. Because high-quality randomized clinical trials are not available, clinicians in NICUs frequently depend on observational studies and the experiences of other practitioner. To date, studies cannot accurately tell us whether an individual woman has chorioamnionitis nor whether an individual infant is infected or is instead showing clinical signs corresponding with the normal transition to postnatal life or a noninfectious condition. If the treatment of sepsis were completely benign, it would make no difference if every infant with the slightest chance of infection was treated. However, treating an uninfected infant for 5 to 7 days means disrupting maternal bonding for an extended period of time, exposing the infant to drugs with potential toxicities, fostering the development of antibiotic-resistant flora, and increasing the probability that the infant will experience a more serious morbidity later in the course of hospitalization (49, 50). The need for a biomarker with high diagnostic accuracy and reliability is of prime importance as a guiding tool for physicians to assess the risk of infection, to detect infection at an early stage and need of antibiotic therapy. Inter α inhibitor proteins are with significant promise (1). But above all, it should be adopted consensus definitions which could be assessed, developed or tested in this unique developmentally immature population (21).


Complete Atrioventricular Septal Defect (CAVSD)-A Case Report

Petkovska Ivanovska I1 , Kacarska R 1, Kuzevska-Maneva K1, Gjurkova-Angelovska B1, Jovanovska V1, Neshkovska-Shumenkovska M1, Nonkulovski D1, Kacarska M1, Gjorgievska J1, Gjaferi M, 1) Cadikovski V.2) University Children’s Hospital, Skopje, Macedonia 1) University Clinic of Pediatric Surgery, Macedonia 2)

Background and aims: An estimated 2–3.5% of children born each year with congenitalheart defects (CHD) have a complete atrio-ventricular septal defect (CAVSD), a third of whom have associated Down's syndrome.1 CAVSD constitutes an anomaly of endocardial cushion development that results in the absence of atrial and ventricular septa with a common annulus for the atrioventricular (AV) valves. Surgical correction of CAVSD is the gold standard. With improved surgical and cardiopulmonary bypass technique, and advanced peri-operative care in the current era, infancy is recommended as the optimum time for the repair.

Material and methods: : We present a seven months old child diagnosed with Complete Atrioventicular Canal Defect, Patent Ductus arteriosus, Down Syndrome and Hypothyroidism during infancy that ever since had been clinically followed at out clinic.

The diagnosis was performed by use of : clinical signs and symptoms( Failure to thrive, repeated respiratory infections in early infancy) , Chest X-ray( showed cardiomegaly ) , 12 channel ECG and 2 Dimensional Color –Dopler echocardiography a main method. Clinical findings showed a grade of 3/6 holosystolic murmur. The systolic murmur of mitral regurgitation audible at the apex. Echocardiography confirmed presence of complete atrioventricular septal defect, large primum ASD, large inlet VSD, Common left sided AV valve (mitral valve) regurgitation, RV-RA shunt, Patent Ductus Arteriosus. The Complete AV Canal in this patient was resolved by a surgical repair , Complete AV canal repair with 2 patch technique, patches close ASD, VSD and separate common AV valve into two valves. and PDA ligation. Early and late postoperative clinical and echocardiography evaluations reveled no complications and no effusion. Following short post-operative hospitalization the child was discharged from the hospital clinically stable with no signs of infection.

Conclusions: Most of the infants have Complete AV Canal that is unresponsive to medical therapy, and some have elevated pulmonary vascular resistance. Early surgical repair of the defect is especially important for infants with Down syndrome because of their known tendency to develop early pulmonary vascular obstructive disease.

Keywords: Complete Atrioventicular Canal Defect (Complete atrio-ventricular septal defect), Patent Ductus arteriosus, Down syndrome.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4306093/#bib0005


Tracheomalacia in children with reccurent low respiratory diseases

Petrushevska-Kolekjevska Lidija,Lazarevska Iskra,Buzarov Jane,Galbova Sofija,Boshkovska Katerina, JZU “ Institute for respiratory diseases in children-Kozle”,Skopje,Republic of Macedonia Congenital airway malacia is one of the causes of irreversibile airways obstruction in children.The incidence in general population is unknown as if it is ofthen diagnosed

Later in children and it is mixed with asthma.

Aim:To performe flexible bronchoscopy as a diagnostic procedure in repeated and prolonged wheezing in children.

Methods:We analysed all flexible bronchoscopies performed at our department from 2007 to 2013.We summarized clinical features(wheezing,cought,reccurent pneumonia) of children with primary airmalacia estimated the predictive value of a clinical diagnosis by pediatritions.

Results:In a total of 109 performed bronchoscopies,airway malacia was diagnosed in 25 children(22,94%).The mean age was 4,2 years(range 0-17).We find tracheomalacia in 17 children,bronchomalacia of the right main bronchi in 12 children and 6 children have bronchomalacia of the left bronchi.In 53% of the airway malacia diagnosis the diagnose was not suspected prior to bronchoscopy.Presenting clinical signs of children with airway malacia wre variable and atipical and similar with features of asthma.

Conclusion: Primary airway malacia is not rare condition.It is difficult to recognize the malacia based on clinical features but we have to think of performing bronchoscopy in children with reccurent lower respiratory infections and therapy resistant asthma.


Дали Микробиолошкиот Наод Неминовно Условува Антибактериски Третман Г Попова, Т Илиевска, К Бошковска, И А Доневска, В Стевиќ Институт по белодробни болести кај децата –Козле, Скопје, Р.М.

Вовед Streptococcus pneumoniae и Haemophilus influenzae се најчести причинители на долно-респираторните инфекции. Стандардната колекција на спутум и трахеален аспират, не ја избегнува можноста за контаминација на примерокот со микробиолошка флора која ги колонизира горните дишни патишта.

Материјал и методи Анализирани се примероци од долните дишни патишта на пациенти хоспитализирани на Институтот по белодробни болести кај децата- Козле, во период од 01.09.2014 до 01.03.2015год. Стандардни микробиолошки процедури беа применети за изолација и детекција на бактериите. Таму каде што имаше изолација на S.pneumoniae и/или H.influenzae, направена е компарација со биохемиските параметри (SE, CRP, Le) и клиничката презентација.

Резултати Од вкупно анализираните примероци, 31,7% (84/265) биле со изолација на S.pneumoniae и/или H.influenzae. Од позитивните изолати кај 63% (53/84) е докажан S.pneumoniae, кај 34,5% (29/84) - H.influenzae и кај 2,4% (2/84) се изолирани двете бактерии. При направената компарација со биохемискте параметри, кај 41,7% (35/84) беа евидентирани биохемиски параметри кои укажуваат на бактериска инфекција - CRP во границите од 25 до 118 mg/l, SE од 25/52 до 83/110 и Le од 10,6 до 31,2 х 109/l.

Заклучок Според анализата на податоците од оваа студија, може да заклучиме дека бактериската изолација сама за себе не е показтел за неопходноста на антибактериската терапија.

Клучни зборови : S.pneumoniae, H.influenzae, Ц-реактивен протеин, седиментација, леукоцити


Здравствен сиситем заснован на вредности

Мерење и известување (запишување) за исходот од лекување и трошоците кои се прават за лекување на еден пациент

Анита Поповска1,

1Институт за белодробни заболувања и туберкулоза

Без исклучок за која индустрија се работи подобрување на перформансите значи и подобрување на вредноста. Концептот на вредност е поврзан со исходот на лекување кој се постигнува во однос на трошоците кои се прават за да се постигне посакуваниот исход од лекување. Дефинирањето и мерењето на вредноста се основни предуслови за да се разберат основните карактеристики на една организација, но и истата да се води напред. т.е. да се подобруваат и унапредуваат перформансите. Оваа значајна алатка му е одземена на здравствениот сектор и поради тоа неможе да се унапредува.

Во здравството вредноста се дефинира како исходот од лекувањето што го добиваат пациентите во однос на потрошените пари. И покрај важната улога која ја има вредноста за здравствениот систем се уште не го има најдено правото месо и нејзиното значење се уште е многу малку познато за творците на здравствените политики.

Креаторите на здравствена политика имаат голем број на цели кои треба да ги реализираат како што се пристап до здравствени услуги, профитабилност, подобар квалитет , одржување на трошоци задоволни пациенти, но никаде не се спомнува вредноста како дел од унапредувањето и подобрувањето на здравствените услуги. Постигнувањето на квалитет како круцијален концепт за унапредување на здравствениот систем се користи на различни начини кои доведуваат да се изгуби значењето на квалитет и да биде комплетно неупотребливо. И покрај многубројните напори да се унапреди и подобри давањето на здравствена грижа сите обиди паѓаат во вода поради неуспехот да се одредат параметри кои ќе можат да ја мерат вредноста Ова е и единствената причина која ги прави реформите во здравствениот сектор неодржливи и не применливи во однос на другите индустрии.

Вредноста не е апстрактен поим, ниту пак поим за намалување на трошоците, таа претставува рамка за подобрување на перформансите во здравствениот сектор и основна алатка за да го води системот во прогрес. Но, до ден денес примена на вредноста во здравствениот систем не постои.

Земено пошироко, без оглед за која дејност се работи вредноста без исклучок се дефинира за потрошувачот а не за производителот или снабдувачот, целосно, за оној за кого е наменет крајниот производ за тој се дефинира вредноста. Бидејќи во здравството вредноста се дефинира како исход од лекувањето кое го добиваат пациентите во однос на потрошените пари, логично е централното место да го имаат пациентите а не другите учесници во системот. Вредноста се мери преку исходот од лекување на излезните елементи (output), а не преку услугите кои се дадени за да се постигне исходот од влезни елементи (input).


Така што, вредноста зависи од исходот на лекување на пациентите, а не од услугите кои пациентот ги добил за време на своето лекување. Давањето на повеќе здравствени услуги како и медикаментозна терапија не значи подобро здравје за пациентот. Овие констатации и искуства ни укажуваат на фактот дека фокусот на вниманието при креирањето на здравствената политика треба да се насочи, од давање на услуги во голема количина во давање на услуги со зголемена вредност. Мерењето на вредноста во здравството не треба да се заснова на процесите кои се прават за да се обезбеди здравствена грижа иако се тие добра тактика за да се обезбеди подобрување на процесот, но не и, замена за мерење на исходот од лекување и трошоците. Точно е дека преоценката на трошоците е основата за да се достигне вредноста во здравствените системи, но трошоците сами по себе не значат вредност. Многу е зачестена практиката да се намалуваат услугите кои треба да се дадат на пациентите за да се намали цената на услугата која се дава на пациентот. Но, ваквата постапка значи дека трошокот во одреден период ќе се зголеми и со тоа не е постигната целта, а тоа е да се излекува пациентот. Намалувањето на трошоците без притоа да се постигне добар исход од лекувањето е многу опасен потфат и претставува самоуништувачки чин за секоја здравствена установа која има стратегија на овој начин да ги намалува трошоците на установата. Ваквиот начин на штедење ја лимитира здравствената ефикасност на здравствената установа.

Фокусирањето на вредност, а не на трошоци значи елиминирање на здравствени услуги кои се скапи, но во исто време неефикасни

Целта на овој труд е да се анализираат поимите вредност и квалитет на здравствениот систем како и начините како таа вредност да се измери. Од тие причини во овој труд ќе се дефинира вредноста при давањето на здравствени услуги, компонентите на вредноста и како таа треба да се мери. Вредноста мора да биде главна цел во секој здравствен систем, мерењето на вредноста мора да постане водечка сила за секој учесник во здравствениот систем.

Како што ќе се виде од излагањето на овој труд недостатокот на сеопфатен и точен исход и мерење на трошоците е една од најголемите слабости за подобрување на здравствениот систем. Фактот дека вредноста не се мери значи дека не се поседува најмоќната алатка за подобрување на вредноста за пациентите. Примената на концептот на вредност ќе значи заголемена вредност за пациентите, подобар исход од лекувањето на пациентите со помали трошоци, што со сигурност ќе води кон подобрување на здравствениот систем. Вредноста треба да биде главна цел на секој давател на здравствени услуги, примената на вредноста од страна на давателите на услуги директно би се одразило врз подобрувањето на целиот здравствен систем во земјата.

Клучни зборови: Вредност, мерење на вредност, здравствени системи, исход од лекување


Vitamin D in the light of current knowledge

Nedeljko Radlović

School of Medicine University of Belgrade; University Children’s Hospital, Belgrade; Academy of Medical Sciences of Serbian Medical Association, Serbia

Abstract

Vitamin D, i.e., 1.25(OH)2D, is an essential factor, not only of homeostasis of calcium and phosphorus, but also of cell proliferation, differentiation and apoptosis, immune and hormonal regulation, as well as other body processes.Thus, its optimal presence in the body is of exceptional significance for health, both of children, as well as adults and elderly persons. Today, it is known that the lack of vitamin D, besides having negative effects on the skeleton and teeth, also contributes to the development of various malignancies, primarily of the large bowel, prostate and breasts, as well as of autoimmune and allergic diseases, diabetes mellitus type II, arterial hypertension and others. Considered from the biological aspect, physiological requirements in vitamin D are achieved by cutaneous synthesis from 7-dehydrocholesterol during sun exposure, while, except rarely, it is very scarce in food. Having in mind extensive evidence that sun exposure presents a high risk for the development of skin malignancies, primarily melanoma, it is clear that humans are deprived of the natural and basic source of vitamin D. In accordance, as well as based on numerous epidemiological studies showing the increase of diseases, in the basis of which vitamin D deficiency plays the important role, next led to the recommended dietary allowance of vitamin D, regardless of age. According to current attitudes, it is recommended that the daily dietary allowances of vitamin D, i.e., the quantity of oral intake that would safely cover the optimal body requirements should be 400 IU for ages 0-18 years, 600 IU for ages 19-70 years and 800 IU for persons aged over 70 years.


The best practices and risks assessment strategy for unlicensed and “off -label” use of medicines in pediatric population

Vasilka Nica1, Elizabeta Zisovska2, Maja Glavash Dodov3, Maja Simonovska Crcarevska3, Jasmina Tonich Ribarska3 and Renata Slaveska Raichki3

1Public Institution in the Health Sector, for the needs of PHI University Clinics, Institution and Urgent Centre, St. Mother Theresa 47, Skopje, [email protected] 2University Clinic for Gynecology and Obstetrics, Faculty of Medical Sciences, University Goce Delcev, Shtip, St. Krste Misirkov, bb, Stip 3Faculty of Pharmacy, University Ss Cyril and Methodius, St. Mother Theresa 47, Skopje, [email protected]

Despite many global initiatives and efforts to improve the availability of marketing

authorized dosage forms appropriate for pediatric population, there is still a supportive evidence and widespread need for the implementation of the concept of unlicensed (UL) and “off-label” (OL) medicines use as a common therapeutic strategy or as the only treatment option and standard of health care for this vulnerable population. As acknowledge, prescription, compounding, dispensing and administration of UL and OL use of medicines should be regulated within the national profile of health care policy. Bearing in mind that in our country there is no formal mechanism for management of OL drug prescribing and use that could lead to their quality use, this concept continues to be an important public health issue. For this underlining reason the purpose of our survey is to present the best practices and risk assessment strategy for UL and OU of medicines in pediatric population. First of all it is of paramount importance to establish national policies governing UL and OL prescribing and use along with ethical standard since prescribing by clinicians is an area of practice that is not regulated by drug regulatory authorities. Strategies for collaboration and the shared responsibilities among prescribers, clinicians, pharmacists and regulators with regard to the OL and UL medicines use should be developed and adopted on every level of pediatric health care. The process of determining the need for UL and OL medicines for pediatric population will serve for regulation of certain uses. Responsible OL and UL prescribing also require development of explicit guidance for pediatric clinicians to assess appropriateness, to evaluate safety and efficacy of OL and UL prescribing justified by high-quality evidence as well as in the cases where adequate evidence is lacking. Moreover, monitoring system for OL and UL medicines use by indication then, active collection of safety data and systematically monitoring of pediatric patient responses to OL use will decrease and prevent risky and ineffective OL prescribing. There is a need of policy reforms to promote care giver and public interest in evidence-based OL prescribing. Another issue that has to be regulated is the potential cost associated with this concept of use of medicines in paediatric drug therapy.


Icterus neonatal in bitola in the period from 2009 to 2013 year Domnika Rajchanovska,1I.Filov,1T. Jovanovska1, S.V.Prodanovska1, G. R. Dimitrovska1, I.Timovski.2

1University "St.KlimentOhridski",Higher Medical School, Bitola,R Macedonia 2PHO"Dr. Angelovska-Dr. Timovski"Skopje, R. Macedonia Introduction: Icterus neonatal is a physiological condition that occurs more often in premature infants. The result is the immaturity of the liver enzyme system and change the type of eritrocitarniot hemoglobin. Purpose: To show the prevalence of neonatal jaundice in newborns in the period from 2009 to 2013 in Bitola. Materials and methods: The research was carried out on neonatoloshkoto department at the Clinical Hospital in Bitola. In a retrospective epidemiological study analyzed all babies born in the Clinical Hospital in Bitola in the period from 2009 to 2013. Data from the medical records of newborns. Diagnosis of icterus neonatalwas set based on clinical status and laboratory analyzes. Pathological icterus is excluded according to the criteria of the existence of the same. Results: In the analyzed time period of five years, were born a total of 6546 infants, of which 91.86% were full-term and preterm 8.14%. Icterus neonatalwas manifested in 1226 newborns (18.73%), 51.46% male, 48.54% female. In 98.37% of cases jaundice was physiological. From infants to full-term physiological icterus were 83.12% and 16.88% preterm. Of the total number of newborns with icterus neonatal 52.94% were treated with phototherapy, and other conservative. Conclusion: The prevention of icterus neonatal, significant good control during pregnancy, vocational guidance and atraumatic end in childbirth, prevention of prematurity and early onset of breast feeding the infant. Keywords: icterus neonatal, full-term, phototherapy prevention.


Obstructive bronchitis in childhood in Bitola

Domnika Rajchanovska1,T. Jovanovska1, S.V.Prodanovska1, I.Filov1, G. R. Dimitrovska1, I.Timovski.2

1University "St.KlimentOhridski", Higher Medical School, Bitola,R Macedonia 2PHO"Dr. Angelovska-Dr. Timovski"Skopje, R Macedonia Introduction: Pulmonary diseases are frequent during childhood. The impact of these diseases in relation to total morbidity in children is high and during the infant period and small child comes to 70%. Number of these diseases according to annual reviews from children ambulances, in the last two decades is growing. Aims: To determine the spreading of obstructive bronchitis in childhood in relation to age, gender, place of living and season of the most frequent occurrence in the territory of Bitola's community in 2014. Materials and methods: The study was conducted in the department of children diseases in Clinical hospital in Bitola. Retrospectively were analyzed children treated because of lower respiratory disease in the age of 3 months to 14 years in 2014. Results: During the entire 2014, in Clinical hospital in Bitola were examined 8002 patients from whom 350 (4.4%) had diagnosis of obstructive bronchitis. Out of them 64.62% were male, 76.35% lived in town, 48% of children were 3 years, and mainly the etiology was infective, after viral infections in spring and autumn. Conclusion: Obstructive bronchitis occurs in small children in accordance to age, gender, place of living and season. These children should be followed especially if repeated obstructions and if positive family anamnesis exists. Key words: obstruction, bronchitis, childhood, prevention


Vein of Galen malformation- case report

V. Sabolic, F. Duma, N. Angelkova, G. Damjanovski, P. Lasjaunias, A. Sopfijanova, Lj. Kojic, Z. Kirova, V. Mancev, Lj. Muaremoska, F.Bajrami, Sh.Useini

University Children s hospital Skopje. Macedonia

Institute of Radiodiology Skopje. Macedonia

Centre Hospitalier Universiaire de Bicetre.Paris. France

Introduction: The Vein of Galen malformations (VGM) are rare congenital malformation in childhood usually presenting as vein Galen aneurisms or vein Galen arterio venous malformation( VGAM ) Depending of age three clinical manifestations can be present. Diagnosis of malformation is with ultrasonography of CNS, MRI and MRI angiography. Treatment of malformation is endoscopic embolisation. We present two and half year’s old girl who was admitted in hospital because of a alteration of conscious and seizures. On physical examination megalencephalea with dilated veins over the scalp was found with normal neurological status. Laboratory analyses and cardiological examination were normal. MRI was done hydrocephalus aneurisms and arteriovenous malformation of vein of Galena was found. MRI angiogram asses the diagnosis of high flow fistula from the right plexus chorioideus draining into dilated ecstatic vein of Galen with retrograde reflux in internal cerebral veins and vermin superior vein. Endovascular embolization was done. Neurological examination after 3 and 6 months was normal. Malformation of vein Galen can be treated in specializated centre. The endovascular embolisation is described as the treatment of choice. The goal of treatment is normal neurological and neurocognitive development of children.


How parents’ attitude can affect children’s rehydratation plan

Aurela Saliaj MD,PhD, Majlinda Zahaj MD,PhD, Alketa Hoxha MD,PhD, Sonila Nikaj RN,MSc, Prena Halilaj RN,MSc

Faculty of Public Health, University ‘Ismail Qemali’ Vlorë, Albania

Introduction: Oral rehydration (ORT) is the treatment of choice in the mild and moderated dehydration. A program launched by WHO, promoting the use of oral rehydration solutions to outpatient, reduced mortality rates from diarrhea by 74% from 1980 to 2008, worldwide. Yet despite recommendations, oral rehydration is not widely used in the clinic. 75% of health clinics all over the world have intravenously rehydration first line treatment of gastroenteritis. One of the factors thought to affect this practice is the attitude of the parents and the pressure exerted by them on the physicians.

Objective: This study aims to assess how the attitude and knowledge of parents affect their children’s rehydration plan.

Material and Methods: The study was conducted at the Regional Hospital of Vlora, in the beginning of 2015. The sample included 97 parents who had children admitted to the pediatric ward with clinical diagnosis: acute gastroenteritis. Parents were questioned on their information and experience about rehydration of children.

Results: During the study period in the pediatric ward, 63% of children, hospitalized with gastroenteritis, were subjected to parenteral rehydration, while 26% to oral rehydration. 47% of parents stated that they expected their child to get IV fluids, while 42% expected oral rehydration. Regarding the parents’ attitude toward the doctors, 34% of parents said they have put pressure on the doctor to decide on giving IV fluids to their child. Parents, who have given ORT or tea to their children prior to the hospitalization, mostly live in urban areas than in the rural ones (7 times more).

Questioned to whom they wanted to speak about gaining information on dehydration, most parents responded that they relayed more on health professionals. 73% of them would like to speak with a physician, 22% with a nurse and 3% with their mothers, as more reliable.

Conclusions: There is a statistically significant relationship between the child’s current rehydration plan and parents’ expectations for oral/IV dehydration’s treatment (Pearson correlation index = 0.3808). We can also say that the expectation of parents is connected with the pressure they put on physicians (Pearson correlation index = 0.4336). While the pressure of parents do not seem to have any strong connection with the physician’s decision about rehydration plan (Pearson correlation index = 0.2300). The study showed that there is a very strong relationship (Pearson correlation index = 0.5191) between the parent’s information on oral rehydration and the management of children’s dehydration at home, before sending them to the hospital.

Key words: dehydration, oral and intravenous rehydration, parental attitude.


Long-term follow-up of 17 pediatric cases with high-grade non-metastatic osteosarcoma of extremities

Samardziski M, Popovska A, Georgieva D, Dzoleva-Tolevska R, Atanasov N, Dalipi R. Abstract Background. Currently, 80-85% of the patients with osteosarcoma on the extremities can be safely treated with wide resection and limb preservation. Neo-adjuvant chemotherapy and a number of options for reconstruction after osteosarcoma resection (especially in chemotherapy-sensitive tumors) have increased long-term survival rates. The main goal of this study was to analyze long-term follow-up treatment results of pediatric and adolescent osteosarcoma patients in Macedonia. Methods. From the group of 22 patients with high-grade osteosarcoma, 4/22 patients were excluded, owing to lung metastases at first presentation or pelvic localization. Another 1/18 patient was excluded from the study due to indication for ablative surgery. Seventeen patients were planned for limb-sparing surgery. The rest 17 patients received neo-adjuvant chemotherapy protocol according to the Scandinavian Sarcoma Group XIV. After neo-adjuvant chemotherapy a clinical and radiological response of the tumor has been observed. Results. Response to neo-adjuvant chemotherapy was good in 9/17 patients (52.4%). Early local recurrence appeared in 7/17 patients (41,2%). In 2 (2/7) of these patients recurrence was diagnosed in the second month after limb-salvage surgery. From seventeen patients with limb-sparing surgery, additional 4/17 ended with amputation due to local recurrence (23,5%). Lung metastases appeared in 11/17 patients or 64.7%. Mean survival time of the already deceased patients was 30,6 ± 18,4 months. Four (4/17) of the examinees (23,5%) survived longer than 5 years. Up to date 35.3% of the patients (6/17) are disease or event free with mean survival time of 55 months (range 27-108). Conclusion. There was significant different overall survival time, in our study, between the groups of patients with good response to neo-adjuvant chemotherapy compared to the group of patients with bad response (p=0.0047). Furthermore, overall survival time in our group of patients was shorter than the time reported in the literature. We assume that the ”fund of lost time” prior the diagnosis was the main reason for that. Key words: Neo-adjuvant chemotherapy, high-grade osteosarcoma, Macedonia.


HEREDITARY ANGIOEDEMA IN PEDIATRIC PATIENTS

BiljanaShirgoska, Jane Netkovski Hereditary angioedema (HAE) is an inherited disorder characterized by recurrent, circumscribed, subepithelial swelling of sudden onset, which fades during the course of 48-72 hours, but can persist for up to 1 week. Lesions involve the extremities, larynx, face, esophagus, and bowel wall. There are three forms of HAE. Type I and type II HAE are rare autosomal dominant diseases due to mutations in the C1-inhibitor gene (SERPING1). C1-inhibitor mutations that cause type I HAE occur throughout the gene and result in truncated or misfiled proteins with a deficiency in the levels of antigenic and functional C1-inhibitor. Mutations that cause type II HAE generally involve exon 8 at or adjacent to the active site, resulting in an antigenically intact but dysfunctional mutant protein. Type III HAE (also called estrogen-dependent HAE) is characterized by normal C1-inhibitor activity. Estrogens may exacerbate attacks, and in some patients attacks are precipitated by trauma, inflammation, or psychological stress The diagnosis of HAE is suggested by a positive family history, the absence of accompanying pruritus or urticarial, the presence of recurrent gastrointestinal attacks of colic, and episodes of laryngeal edema. For type I and type II HAE, diminished C4 concentrations are highly suggestive for the diagnosis. Further laboratory diagnosis depends on demonstrating a deficiency of C1-inhibitor antigen (type I) in most kindred’s, but some kindred’s have an antigenically intact but dysfunctional protein (type II) and require a functional assay to establish the diagnosis. There are no particular laboratory findings in type III HAE. Patients with HAE experience angioedema because of a defective control of the plasma kinin-forming cascade that is activated through contact with negatively charged endothelial macromolecules leading to binding and auto-activation of coagulation factor XII, activation of prekallikrein to kallikrein by factor XIIa, and cleavage of high-molecular-weight kininogen by kallikrein to release the highly potent vasodilator bradykininWe had a patient 5 years old admitted at our hospital with laryngotracheal edema, inspiratory stridor and insufficient breathing. The airway was established by urgent tracheostomy. The patient continuous to breathe spontaneously. Anti-edematous therapy, with short and long time duration in high doses were given to reduce the edema. The mandatory monitoring included SpO2, number of respirations, heart rate, EKG and blood pressure monitoring.

The edema persisted up to 48 ours. Laboratory test showed a deficiency of functional C1 esterase inhibitor.Literature suggest prophylactic administration of either 17alpha-alkylated androgens or synthetic antifibrinolytic agents has proven useful in reducing the frequency or severity of attacks. Plasma-derived C1-inhibitor concentrate, recombinant C1-inhibitor, ecallantide (DX88; a plasma kallikrein inhibitor) and icatibant (a bradykinin B (2) receptor antagonist) have demonstrated significant efficacy in the treatment of acute attacks, whereas the C1-inhibitor concentrate has also provided a significant benefit as long-term prophylaxis. However, these drugs are not licensed in all countries and are not always readily available.

1. Ebo DGet all. Hereditary angioedema in childhood: an approach to management. Paediatr Drugs. 2010 Aug 1;12(4):257-68. doi: 10.2165/11532590-000000000-00000.

2. Canonica GW, Rossi O. Diagnosis and treatment of hereditary angioedema.Panminerva Med. 2012 Sep;54(3):241-53.

3. Georgy MS, Pongracic JAAllergy Asthma Proc.Chapter 22: Hereditary and acquired angioedema 2012 May-Jun;33Suppl 1:S73-6. doi: 10.2500/aap.2012.33.3555.

http://www.ncbi.nlm.nih.gov/pubmed/?term=Ebo%20DG%5BAuthor%5D&cauthor=true&cauthor_uid=20593909



http://www.ncbi.nlm.nih.gov/pubmed/?term=Canonica%20GW%5BAuthor%5D&cauthor=true&cauthor_uid=22801442

http://www.ncbi.nlm.nih.gov/pubmed/?term=Rossi%20O%5BAuthor%5D&cauthor=true&cauthor_uid=22801442


http://www.ncbi.nlm.nih.gov/pubmed/?term=Georgy%20MS%5BAuthor%5D&cauthor=true&cauthor_uid=22794695

http://www.ncbi.nlm.nih.gov/pubmed/?term=Pongracic%20JA%5BAuthor%5D&cauthor=true&cauthor_uid=22794695

http://www.ncbi.nlm.nih.gov/pubmed/?term=Pongracic%20JA%5BAuthor%5D&cauthor=true&cauthor_uid=22794695


4. Suzuki D, Suzuki T, Matsui T, Matsuzuka T, Horiuchi T, Omori K. A case of hereditary angioedema defined by gene analysis.Nihon Jibiinkoka Gakkai Kaiho. 2014 Oct;117(10):1270-6.

5. Richard G Goweret all. Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies. World Allergy Organ J. 2011 Feb; 4(Suppl 2): S9–S21.

http://www.ncbi.nlm.nih.gov/pubmed/?term=Suzuki%20D%5BAuthor%5D&cauthor=true&cauthor_uid=25735130

http://www.ncbi.nlm.nih.gov/pubmed/?term=Suzuki%20T%5BAuthor%5D&cauthor=true&cauthor_uid=25735130

http://www.ncbi.nlm.nih.gov/pubmed/?term=Matsui%20T%5BAuthor%5D&cauthor=true&cauthor_uid=25735130

http://www.ncbi.nlm.nih.gov/pubmed/?term=Matsuzuka%20T%5BAuthor%5D&cauthor=true&cauthor_uid=25735130

http://www.ncbi.nlm.nih.gov/pubmed/?term=Horiuchi%20T%5BAuthor%5D&cauthor=true&cauthor_uid=25735130

http://www.ncbi.nlm.nih.gov/pubmed/?term=Omori%20K%5BAuthor%5D&cauthor=true&cauthor_uid=25735130


http://www.ncbi.nlm.nih.gov/pubmed/?term=Gower%20RG%5Bauth%5D


Methods for deciphering intellectual disabilities and autistic spectrum disorders

Sukarova-Angelovska E*, Kocova M*, Angelkova N*, Sukarova-Stefanovska E#

*University Children Hospital, Skopje, Macedonia, #Research centre for genetic engineering and biotechnology, MASA, Skopje, Macedonia

Intelectual disability (ID) and autistic spectrum disorders represent a complex issue for affected individuals, family, doctors, researchers and society. It is a condition characterised by significantly below average mental achievements, and inability for independent function in all areas of daily life. The causes for ID comprise any condition that impairs development of the brain before, during, or shortly after birth. Genetic causes are among the most common, since there are more than 2000 genes which are critical for normal brain development. The most common causes for ID are Down syndrome and fragile X syndrome. Up to now, more than 6000 syndromes that encompass ID have been described in the literature so far. First efforts for detecting genetic causes for ID were made 60 years ago, using conventional karyotyping, afterwards several banding techniques were utilised in order to detect minor chromosomal errors. Implementation of several molecular and cytogenetic methods such as fluorescent in situ hybridisation, MLPA, aCGH, SNP array, methylation analysis, whole genome sequencing, etc, broaden the spectrum of detected causes for ID. Clinical investigation tools such as dysmorphology databases, software systems for photographic detection of a specific facial gestalt also help deciphering causes for ID. We will give overview of many examples of detected genetic causes for ID. However, although many tools of molecular biology have been discovered, many dilemmas and a certain number of cases remain unknown.


Surgical treatment in male children at the Clinic for Pediatric Surgery-Skopje

Simeonov R., Jovanovski-Srceva M., Simeonova A

University clinic for pediatric surgery, Skopje on daily bases performs all type of operations on pediatric patients (expect cardiac –surgery) of all age and is leading pediatric surgery center in Macedonia. Literature shows data that male children are more prone to surgery due to gender predispositions in the age groups from 1-10 years. In Macedonia, there is insufficient data to show the relationship between certain surgical diagnosis and age in male population. The aim of this study was to evaluate the proportion of surgical interventions done on male children during the period from January to August 2015 at the University clinic for pediatric Surgery –Skopje.

Methods: In retrospective study we evaluate the records of all done elective surgery interventions in children. We analyzed the records for the age and gender. In further analyzes, male children, aged 1-10 years, were analyzed for the type of surgery and placed in surgical intervention respectfully.

Results: 877 records of children all ages were evaluated (57%male: 43% female). Out of all male patients, 426 (85.2%) were 1-10 years old. The most common surgical interventions in this age group were cryptorchismus (126 pts), hernia repair (127 pts), phimosis(56 pts), fractures(46pts), hypospadias (31 pts) and other (40 pts) in percentage (29.5%:29.9%:13.2%:10.7%:9.4%).Out of all interventions, male gender predisposed once were 50%.

Conclusions: At our clinic more male children has been operated for the period from January to August 2015. As a conclusion and in respect, surgical interventions done in age group 1-10 years are not only in relation with male gender.

Key words: male children, age, surgery intervention


S100B Protein concetrationd in cord blood: correlations with gestational age in term and preterm infants

University Children’s Hospital Skopje Sofijanova Aspazija

Intraduction: Diagnosing perinatal insults is still primarily based on adequate documentation of general medical and obstetric data, as well as radiological and laboratory results. The measurement of brain constituents, such as S100B protein, may be an alternative and a direct indicator of cell damage in central nervous system when clinical and laboratory resultsremain unclear to the end. A new diagnostic method involving a new brain specific biological marker S100 B protein has been introduced. This will make possible the monitoring of certain events even at the earliest stage of a brain damage, such as neurodegenerative diseases, cerebral tumors, cerebral traumas and cerebral diseases. Measuring blood levels of the protein as a marker for brain trauma is the easiest and most widely used in laboratories worldwide. Research aims : The purpose of this study was to investigated the relationship between S100B concentrations within gestational age in normal preterm and term infants. Metodology: Bold sampals were taken from all 119 neonatal, on the first day of admission ( within the first 12 -24 hours from the time of birth) on Day 14 and Day 7.Hhese samples werw kept in refrigerator at a temperatyre of +2°C +4°C and then processed at the Institute of Clinical Biochemistry at the Faculty of Medicine in Skopje. The principle of determining S100B protein using the ECLIA method ( ECLIA= ElectroChemilLuminiscence ImmunoAssay By Elecsys 2010 Roche Diagnostic) Conclusion: S100B Protein in term and preterm infants is a significantly higher in the first 24h after birth. It is a good in-dicator of starting brain damage in term neonates the first 24h after birth and it is a also indicator for early in-tervention. In preterm neonates it is indicator for possible early brain damage.


S100B Protein Marker for detection of brain distress in intrauterine growth-retarded fetuses

and neonates born with neurological deficit

University Children’s Hospital Skopje Sofijanova Aspazija

Intraduction: S100B protein is detected in several biological fluids, such as cerebrospinal fluid (CSF), blood, urine and amniotic fluid, with the application of immunoassay methods. Firstly, it is detected in CSF as a marker of active brain damage. Studies of adults and children have shown that high concentrations of the protein indicate the occurrence of brain damage, such as neurodegenerative diseases, cerebral tumors, cere-bral trauma and cerebrovascular diseases. S100B protein meets the criteria for a brain damage marker in perinatal medicine for the following rea-sons: · it is simple to measure; · it is detected in a variety of biological fluids; · it could be applied in longitudinal monitoring in sus pected neonates, and thus it represents; · an early quantitative marker of brain damage; The necessity of measuring this protein in the umbil-ical cord blood in high-risk pregnancies, especially those with intrauterine growth retardation (IUGR), where increased concentration of S100B protein is an indicator of a developmental brain anomaly, indi-cating a brain injury attributable to an impaired fe-to-placental barrier, is of huge significance. S100B protein is particularly increased in IUGR fe-tuses where a redistribution of fetal-placental blood supply occurs, with the purpose of the so-called brain-sparing effect, which, in turn, is an important indicator of further brain damage in that group, compared to the IUGR group with no high levels of S100B Research aims: Monitoring the effectiveness of the therapeutic in-terventions in all high-risk and premature neonates included in the study, including IUGR cases, as well as their correlation depending on the dynamics of the movement of S100B protein. Methods: Coming up with predictive indicators of possible abnor-mal brain development with long-lasting consequences. Comparing and monitoring the dynamics of S100B protein in a separate group with developmental anoma-lies (spina bifida, etc.). The principle of determining S100B protein using the ECLIA method ( ECLIA= ElectroChemilLuminiscence ImmunoAssay By Elecsys 2010 Roche Diagnostic) Conclusion: The STUDY provides edivence that circulating S100B PROTEIN is increased in IGUR and the one with Neurological defects fetuses and correlates with cerebral hemodynamics, suggesting that it may represent anindex of cerebral cell damage in the perinatal period.


Development of epilepsy after neonatal seizures

Aneta Soltirovska Šalamon

Department of Neonatology

University Children's Hospital, University Medical Centre Ljubljana

Outcome studies confirmed that neonatal seizures are a major risk for death or subsequent

neurological disability. Despite advances in perinatal and neonatal care, development of childhood

epilepsy after neonatal seizures still occurs in a substantial proportion of children. In order to

identify the correlation between risk factors for adverse outcome many studies found that the

strongest predictors are the underlying cause and background electroencephalographic activity.

In our study on development of epilepsy after neonatal seizures in which the follow up period was

from 2 to almost 12 years, the incidence of epilepsy in our cohort was 18.2% and was the highest in

the first 12 months. In addition to the other risk factors we identified that the duration of seizures

after the first month was as an independent risk factor for developing epilepsy so our data support

the evidence that recurrent and prolonged neonatal seizures may act on epileptogenic substrate

mainly observed in synaptogenesis, causing further damage including synaptic reorganization which

is responsible for the subsequent clinical expression of epilepsy. In our study we aimed to design a

scoring system for prediction of epilepsy but contrary to the three previous studies which were

designed to predict overall adverse neurologic outcome we concluded that the construction of a

universal scoring system was not possible duo to the interaction between duration of seizures after

the first month as the best predictor of epilepsy and gestational age. The duration of seizures after

the neonatal period had a different effect on the development of epilepsy with respect to the

gestational age.


Pneumonia in children caused by mycoplasma pneumoniae

Spasovska Z.

Private health Institution “Happy child” , Skopje, R. Macedonia

INTRODUCTION: In primary care often encounter the infections on lower-respiratory organs caused by Mycoplasma pneumoniae. Usually occurs in children of school age. But in recent years, we diagnose and early childhood, that children under three years of age.

AIM: The purpose is to show that in primary care are made efforts for detailed monitoring of children with infections on lower-respiratory organs in order to detect the cause of atypical pneumonia and their proper treatment, especially in children of preschool age. METHODS: Processed data from medical records of 2100 children in the period from 2014 to 2015 year. A total of 193 chlidren is diagnosed with an infection of the lower respiratory tract. In 13 ( 6,73 %) due to prolonged disease course and persistence of cough is realized indirect immunofluorescence Pneumoslide, wherein the proven cause of atypical pneumonia, that were found IgM + antibodies to Mycoplasma pneumoiae. Despite the positive findings of Mycoplasma pneumonia in 1 child ( 7,6%) were found and IgM + antibodies to Influenzae A and in 1 chlid ( 7,6%) and IgM+antibodies to Legionela pneumophila. Realized laboratory analyzes in only 2 children ( 15%) showed positive markers of inflamation or elevated white blood cells and neutrophils in the differential blood count and elevation of C-reactive protein. Due to the severity of the clinical picture 2 chlidren ( 15 %) were referred for hospital treatment at the Clinic for Children’s Diseases , where is made X-ray of the lungs that in bouth cases in favor of bronchopneumonia.The remaining 11 children (84%) were treated ambulatory with macrolide preparation and 9 children (81 %) were placed on therapy with azithromycin, while 2 children ( 18%) were treated with clarithromycin. In all optained good clinical response with regression of pulmonary auscultatory findings. Five children ( 38 %) diagnosed with atypical pneumonia of Mycoplasma pneumonia cause is under the age of 3 years, starting from smallest of 16 months and the remaining 23, 24, 26 and 28 months of age. The rest are of school age and 3 children of 7 years ( 23%), 2 children 8 years old ( 15 %), 1 child of 9 years ( 7,69 %) and 1 child of 11 years ( 7,69%). Prevalence is higher among girls and 9 were girls ( 69 %) and 4 boys ( 31%). CONCLUSION: As evident from the data the atypical pneumonia caused by Mycoplasma pneumonia cause are more likely encounter in our practice in very early childhood before the age of 3 years. That is why it is necessary early detection and adequate treatment in order to avoid possible complications and reduce the need for hospital stays.


Споредбени вредности на apgar score кај новородени добиени соsectio caeareaводен

во општа или спинална анестезија

Автори: д-р Сашо Спасовски, д-р Дафина Караџова, д-р Киро Чурлинов, прим.д-р Елена

Славенска и прим.д-р Виолета Божиновска.

1. Вовед Во последниве години бројот на новородени добиени по пат на sectio caesarea е во

постојан пораст, па затоа е потребна подетална анализа на apgar score кај новородените на пациентките чие породување е спроведено по пат на sectio caesarea. 2. Цел

Цел на трудот е да се прикажат споредбените вредности на apgar score кај новородени добиени со општа и спинална aнестезија за sectio caesarea. 3. Материјали и методи

За истражувањето на овој труд беа опфатени 100 пациентки од кои 70 беа водени во спинална, а 30 во општа анестезија. За општа анестезија по претходна премедикација со Amp.reglan и ranital се пристапи кон вовед со propofol 1,5-2mg/kg и leptosuccin 1,5-2mg/kg телесна тежина и сооднос на гасови O2:NO2 50:50 и izofluran 0,6% до климување на папочната врвца, по што следуваше исклучување на izofluranи следеше натамошно продолжување на анестезија со опиоидfentanyl 0,2-0,25 mg и релаксант esmeron 30-40mg. Притисокот (ТА) кај пациентките за цело време се движеше од на вовед 125-145 со 75-95 mmhg, до 115-125 со 60-85mmhg интраоперативно a SAO2 (cатурација) oд 96-98% и пулс 85-90/мин. на вовед со 65-80/мин. интраоперативно,како и apgar score на новородени 7-8 кај 40% и 8-9 кај 60% од пациентките. За спиналната анестезија по претходно хидрирање на пациентките со 500мл 0,9%Naclи бришење на полето се пристапи кон апликација на спиналната анестезија на ниво L3-L4 со игла 27G и давање на 0,5%marcain 1,8-2,2ml и опиоид fentanyl 20mcgr како и подршка со О2 од 6l.За цело време ТА се движеше од на вовед 120-140 со 80-100mmhg со 90-110 со 50-70mmhg интраоперативно.SaO2 се движеше од 99-100% и пулс од 80-115/мин.Apgar score кај новородените беше со вредност од 8-9 кај20% и 9-10 кај 80% од пациентките. 4. Заклучок:

Од прикажаните резултати може слободно да се заклучи дека новородените кои биле родени од пациентки кои биле водени со спинална анестезија за sectio caesarea биле родени со подобар apgar score. Врзоснованатоасметамедекаsectio caesarea воден врз основа на спинална анестезија дава подобри вредности на apgar score кај новородените.


Methods for assessment of malnutrition in hospitalized children

Lidija Spirevska, Stojka Fustik, Tatjana Jakovska, Tatjana Zorcec

University Pediatric Clinic Skopje

Malnutrition is very common at the time of hospital admission tended to increase during the hospital stay and has negative effects on prognosis, increases the incidence of complications and the morbidity and mortality. Diagnosis of malnutrition in ill child must be based on an objective assessment of nutritional status; this includes an adequate history of recent food intake and weight loss and adequate measurements. Objective: To present methods for assessment the malnutrition in hospitalized children and pointing out their advantages, limitations and risks.

Methods: Were analyzed chapters in textbooks, articles in relevant journals and theses related to the topic.

Results: Anthropometric measurements and body composition analysis are most commonly methods due to its easy application, low cost and invasiveness. Hematological parameters are of very little value in the nutritional evaluation. Biochemical parameters are very useful. Immunity and electrolyte levels are commonly used, but these changes are not generally related to problems of nutrition.

Conclusion: Adequate assessment of nutritional status is the most important of early nutritional intervention. Future goals are individualized diet according to the specific characteristics of each sick child. The diet should be continuously adjusted according to possible metabolic changes and nutritional status. Therefore, it is important to do studies that will identify the most sensitive methods to assess malnutrition, which can be applied to most of the sick children.


Трендови на раст и нутритивен статус кај предучилишни деца во РМ

Игор Спироски1,2, Мира Митреска3, Верица Добросављевиќ4, Светлана Пешевска5, Василка Келкоцева6, Снежана Петрова7, Наќе Туфекчиев8, Емилија Богоевска9, Иванка Наумческа10,

Надица Ачовска11, Јовица Станимировиќ12

1Институт за јавно здравје на РМ – Скопје 2УКИМ Медицински факултет – Скопје 3Центар за јавно здравје – Охрид 4Центар за јавно здравје – Скопје 5Центар за јавно здравје – Куманово 6Центар за јавно здравје – Штип 7Центар за јавно здравје – Велес 8Центар за јавно здравје – Струмица 9Центар за јавно здравје – Битола 10Центар за јавно здравје – Тетово 11Центар за јавно здравје – Кочани 12Центар за јавно здравје – Прилеп Вовед: Редовните мерења на висината и тежината можат да бидат индикација за тоа дали растот на децата е согласно препораките за раст на деца на определена возраст. Цел на нашето истражување беше да се прикаже трендот на раст и нутритивниот статус на децата на возраст од 4 до 5 години во РМ.

Материјал и методи: Во просек, на по 1320 деца на возраст од 4 до 5 години им е мерена висина и тежина, во периодот од 2011 до 2015 година. Со мерењата се опфатени деца од сите региони во државата. Мерењата, како и проценките на растот и нутритивниот статус се прават согласно стандардите за раст на СЗО.

Резултати: Низок е процентот да деца со акутна или хронична неисхранетост кај оваа популација (5-годишен просек: 1% хронично и 1,7% акутно неисхранети). Просечно, 30,2% од децата се со зголемена телесна тежина, а 13,1% се дебели. Поголем е процентот на машки деца, во споредба со женските, кои се со зголемена тежина или се дебели. Зголемената телесна тежина и дебелина кај оваа популација има тренд на лесно, но континуирано опаѓање. Во 2015 година 27% од децата биле со зголемена телесна тежина, а 10,4% биле дебели (31,4% и 15,1% во 2011, 29,4% и 11,6% во 2014).

Заклучок: Иако податоците покажуваат тренд на намалување на дебелина кај оваа популација, постои широк простор за понатамошни подобрувања. Спроведувањето на јавноздравствени политики и акции треба и понатаму да се евалуира преку мерење на нутритивниот статус на овие деца.


АЗОТ ОКСИДУЛ (N2O) ВО ПЕДИЈАТРИСКАТА АНЕСТЕЗИЈА: ДА СЕ НАПУШТИ ИЛИ НЕ?

Спировска Т, Гавриловска-Брзанов А, Кузмановска Б, Карталов А, Селмани Р 1Универзитетска Клиника за Трауматологија, Ортопедски болести, Анестезија, Реанимација, Интензивно лекување и Ургентен центар, Клинички центар Мајка Тереза, Скопје Абстракт: Азотниот оксидул (N2O) се користи во клиничката пракса повеќе од 150 години. Многу е честа и неговата примена во педијатриската анестезија: при вовед и водење на анестезија како и за аналго-седациски процедури. Но, употребата на N2O се повеќе се напушташе поради неговите евентуалните штетни ефекти. Европската асоцијација на анестезиологија повика експерти од областа на анестезија со цел да се одговори на следните прашања и да се донесе консензус дали е основано се пореткото користење на N2O во секојдневната пракса:

1. Кое е местото на N2O во денешниот периоперативен анестезиолошки менаџмент? 2. Кое е неговото место во аналго-седациските процедури? 3. Дали е неговата примена поврзана со ризик за пациентите и/или професионалните

работници? Кое е местото на N2O во денешниот периоперативен анестезиолошки менаџмент? При користење на N2O воведот во анестезија е побрз, поедноставен и со подобрена оксигенација. При одржување на анестезија со N2O се намалува потребата од други анестетици. N2O предизвикува постанестезиолошко гадење и повраќање кај интервенции кој траат подолго од 1 час. Овој ефект може да се контролира со антиеметичка профилакса. Кое е неговото место во аналго-седациските процедури? Освен во анестезијата се повеќе се зголемува интересот за негова употреба кај останатите здраствени специјалности како аналгетска терапија при болни интервенции во стоматологијата, гинекологијата и акушерството, педијатријата. Дали е неговата примена поврзана со ризик за пациентите и/или професионалните работници? Не постојат докази дека употребата на N2O во клиничката пракса може да го зголеми ризикот за пациентите или здраствените работници. Контраиндикации за неговата примена се: присутна затворена шуплина исполнета со гас (пнеумоторакс) или абнормалности во метаболизмот на витамин Б12. Некои експерти препорачуваат N2O да не се користи повеќе од два пати неделно. Потенцијалниот тератоген ефект на N2O забележан во неколку експериментални животински студии не може да биде екстраполиран на луѓето. Заклучок: Експертите донесоа консензус дека во моментов не постојат клинички релевантни докази за воздржување од употреба на азотниот оксидул (N2O). Тој може безбедно да се користи во педијатриската анестезија и во процедурите со минорен интензитет на болка. Не постои доказ кој покажува зголемен ризик по здраствените работници или пациентите изложени на N2O.

Клучни зборови: азотен оксидул - N2O, педијатриска анестезија.


COMPARISON OF TWO DIFFERENT CONCENTRATION OF BUPIVACAIN IN CAUDAL BLOCK IN PEDIATRIC PATIENTS

Spirovska T, Gavrilovska-Brzanov A, Kuzmanovska B, Kartalov A, Selmani R

1University Clinic for Anesthesiology, Reanimation and Intensive Care Medicine Clinical Center Mother Theresa, Skopje ABSTRACT:Background and objectives: More than 15 years caudal blockade with bupivacain 0,25% has been used in the University clinic for anesthesia reanimation and intensive care, Pediatric department- Skopje. Due to the fact that this concentration is considered safe and it has been associated with a high success rate and a low incidence of adverse events, in this prospective, comparative, interventional clinical study we wanted to a assess the analgesic efficacy and safety of lower concentration 0,125% bupivacain for pediatric caudal block. Material and methods: The analgesic efficacy and safety of a single caudal injection of a bupivacaine 0,125% and 0,25% was investigated in 60 children, aged 1-7 yr and American Society of Anesthesiologist physical status I-II undergoing lower abdominal and genito- urological surgical procedures . After induction of anesthesia and before surgery, the children were assigned to receive a caudal injection of 1.0 ml.kg bupivacaine 0.125% or either bupivacain 0,25% 1.0 ml.kg. After completion of surgery, patients were assessed in the recovery room for six hours from the time of the caudal injection and for a further 12h on the ward. The evaluation of postoperative analgesia was accomplished through the visual analog pain scale, the motor blockade assessed by modified Bromage scale and the level of sedation through the three-point scale. Other complications were also recorded. Postoperative analgesia was provided by intravenous Acetaminophen. Analgesic requirements were recorded for the 24h study period. Results: Pain and sedation scores did not differ between groups. The adequacy of caudal analgesia and supplemental analgesic requirements did not differ between the two groups at any time during the first 12h after surgery. Respiratory depression or hypoxia did not occur. The incidences of other side effects did not differ. Conclusion: We conclude that a single caudal injection of a bupivacaine 0,25% administered prior to surgery, while safe, offers no advantage over an injection of bupivacaine 0.125% for pediatric lower abdominal and genito- urological surgical procedures. . Key wards: bupivacaine; analgesia; caudal blockade.


Cardiomyopathy in children for non-cardiac surgery-Anesthesiological aspect

Jovanovski- Srceva M., Simeonov R., Donev l., Kartalov A., Mojsova M., Gavrilovska A.,Todorova Z

ABSTRACT

The World Health Organization definite "cardiomyopathy" as ‘a disease of the myocardium associated with cardiac dysfunction”(1). Although, this is a rаre disease in general pediatric population, the prognosis and outcome of this patients during surgical interventions might be detrimental and depend on many preoperative as well as intraoperativ factors.

Anesthetic implications in these children are various and are associated with a high risk of death and complications during anesthesia. This presentation aim is to review the data from the current literature and summarize the knowledge for anesthesiological approach in children with cardiomyopathy that are scheduled for non-cardiosurgical interventions . Within this presentation the etiology and classification of cardiomyopathies is elaborated. Special emphasis is given on the anatomical, morphological and pathophysiological changes in various types of cardiomyopathies and their different influence on haemodynamic stability during anesthesia.

Conclusion: Cardiomyopathy is the third cause of heart transplantation in pediatric population. Non-cardiac operations in these children carry a high risk of mortality. Knowledge of this disease and its anesthetic implications are crucial in patient outcome. Adaptation of anesthesiology procedures and techniques, as well as avoidance of myocardial depression, maintaining normovolemis, avoiding a drop in arterial blood pressure during induction (by over 10%) and avoiding ventricular strain are part of anesthetic targets.

Key words: cardiomyopathy, anesthesia, non-cardiac surgery


Does peroperative given ondasetron prevent postoperative vomiting and nausea (ponv) in male children ?

Jovanovski-Srceva M1, Simeonov R2, Gavrilovska A1, Stavridis S3,Nikolova Z4,Temelkovska M1, Donev Lj1,Todorov R5, Mojsova M1

1University Clinic for anesthesia reanimation and intensive Care, Skopje 2University Clinic for pediatric surgery, Skopje 3University Clinic for urology-Skopje 4Eye hospital -Skopje 5Clinic for general surgery, Skopje

Background and objectives: Postoperative nausea and vomiting (PONV) is common, but in children may be detrimental. Incidence of PONV in children is related to many factors (type of anesthesia, gender, type of surgery etc.). Ondasetron (setronin 3receptor antagonist) at our clinic is commonly used for treatment of PONV. The aim of this study was to evaluate whether prophylactic dose of ondasetron is effective in preventing PONV in male children.

Method: A cut off analyzes was done in August 2015 (for the year of 2015) on the 150 records of male children, ages 5-10, ASA I/II, who underwent general anesthesia for surgical treatment for cryptorchisms, hernia, phimosis and hypospadia repair. We analyzed the records for given preoperative prophylactic dose of ondasetron (0.1mg/kg) in correlation with the incidence of PONV during the first 24h. PONV was recorded with standard scale: 0(no nausea or reattachment); 1 (nausea and reattachment); 2(one vomiting and nausea); 3 (several episodes of vomiting).

Results: Distribution to the type of the surgical interventions was: cryptorchismus in 60 patients; hernia repair in 40 pts; phimosis in 28 pts and hypospadia in 11 pts (47.3%:26.7%:18.7%:7.3%). In the total number of patients prophylactic ondasetron was given in 106 patients (70.7%). Out of them some degree of PONV occurred in 66.9% (71 pts). Significantly higher percentage of children had PONV grade 1 (71.8%) compared to grade 2 or 3(18.4%: 9.8%).

Conclusion: Despite commonly used anti-emetic prophylaxis with ondasetron, male children aged 5-10 years still develop some score of post operative nausea and vomiting.

Key words: PONV, male children, ondastron, surgery


Preoperative and peroperative menagment of child with dilatative cardiomyopathy

Jovanovski-Srceva M1, Simeonov R2, Cadikovski V2,Spirovski Z5, Gavrilovska A1, Stavridis S3,Mojsova M1, Donev Lj1,Todorov R5

1University Clinic for anesthesia reanimation and intensive Care, Skopje 2University Clinic for pediatric surgery, Skopje 3University Clinic for urology-Skopje 4Clinic for general surgery, Skopje 5Clinic for thoracic surgery,Skopje

Background: Dilatatative cardiomyopathy (DCM) is a rare disease in the pediatric population and is characterized with biventricular or left ventricular dilatation and altered ventricular contractility. Anesthesiology approach to children with DCM scheduled for elective non-cardiac surgery intervention is rarely reported through the literature. Preoperative preparation, introduction, maintenance of anesthesia and their repercussions on the hemodynamic parameters and myocardial function are part of the spectrum of problems that may be encountered during the anesthesiologist intervention

Case report: We report the preoperative and intraoperativ management of four year old boy, with secondary hypo kinetic dilatative cardiomyopathy. Child was admitted to the surgery unit for surgical treatment of Vesico Urethral Reflux (VUR). Parents of the child revealed that he was hospitalized several times for some irregular heart rhythm and that he is taking Tabl.Captopril a 25mg 2x1/2. Detailed cardiologic evaluation showed DCM , ventricular ejection fraction (LVEF) of 30%, Fractional Shortening of 22%, tricuspid and mitral valve insufficiency and tachycardia 123 bpm.We sustained the surgery at that time(introduced urine catheter, antibiotics and Iv fluids)and in cooperation with pediatric cardiologist the following therapy was prescribed: Tabl.Amioderone a 100 mg 1x1/4;tabl.Lanicor 2x1/4 vp ;tabl.Bipreso a 2,5 mg 1x1/2;tabl Captopril a 25 mg 2x1/4;Tabl.Spirinolactone a 25 mg 1/4 every second day and CaCO3 3x1/2.

After four weeks the child underwent surgery. Anesthesia induction was stared after preoxigenation for 5 minutes with oxygen, low doses of fentanyl 0.25mcgr/kg, thiopental 2mg/kg and intubation with rocuronium bromide 0,6mg/kg. Anesthesia was maintained with sevoflurane 1-1.5%MAC and fentanyl. Issues that we were focused on were: normovolemia, avoiding myocardial depression and hypotension at any time, adequate analgesia and diuresis. Intervention was ended after 120 minutes and Exciso valvulae urtrae posterior and ureterocutanostomia billateralis was done. Recovery of the child was fully and no postoperative incidents were recorded.

Conclusion: Children with DCM, especially if LVEF <45% require special attention and good preoperative myocardial stabilization as well as intraoperativ balanced dose adapted anesthesia, fully monitoring of the hemodynamic and adequate vascular volume to avoid any incidents.

Key words: Dilatative cardiomyopathy, anesthesia, hemodynamic, preoperative preparation


Цистична ехинококоза во општата и детската популација (перманентен проблем низ годините)

Олгица Стефановска, Институт за белодробни заболувања кај децата Козле Цистичната ехинококоза (ЦЕ) е антропозооноза предизвикана од паразитот Echinococcus granulosus, и претставува голем медицински ветеринарен општествено економски и социјален проблем. Болеста е широко распространета насекаде низ светот,но во ендемските подрачја на кои припага и нашата земја проблемите што оваа паразитоза ги имплицира се позначајни. Према податоците на СЗО во светот од ехинококоза во било кој момент има повеќе од 1000000 заболени. Ризикот од болеста е особено голем во руралните делови на ендемските региони. Економскиот товар што ЦЕ го условува се цени на три билиони US $. Неформалната група за ехинококоза како и неколкуте подгрупи кои функционираат во склоп на СЗО спроведуваат амбициозни превентивни програми за справување со ехинококозата во повеке ендемски региони во светот. Цел на трудот- компаративна евалуација на некои епидемиолошки карактеристики на ЦЕ во нашата земја,со особен осврт на детската популација следена низ годините, како индикатор за нашата ефикасност во справувањето со болеста. Материјал и методи-анализирани се статистички податоци добиени од Републичкиот завод за здравствена заштита(РЗЗ) и Институтот за белодробни заболувања кај децата Козле, кои се однесуваат на бројот на пријавени заболени од ЦЕ во последните 15 години, возрасната возрасната структура, половата застапеност, сезонската појава и географската дистрибуција. Резултати- заболени од ЦЕ во Р.Македонија во последните 15 години: Годинa вкупно заболени: деца: 2000 23 (8м ,15ж) 5 (0м ,5ж) 2001 12 (6м ,6ж) 7 (5м ,2ж) 2002 6 (4м , 2ж) 0 2003 12 (8м ,4ж) 6 (5м ,1ж) 2004 12 (6м ,6ж) 3 (0м , 3ж) 2005 17 (7м ,10ж) 9 (4м ,5ж) 2006 19 (6м ,13ж) 7 (2м ,5ж) 2007 17 (9м ,8ж) 5 (5м ,0ж) 2008 44 (24м ,20ж) 7 (6м ,1ж) 2009 39 (18м ,21ж) 12 (8м ,4ж) 2010 38 (17м ,21ж) 12 (7м ,5ж) 2011 33 (16м ,17ж) 9 (4м ,5ж) 2012 18 (9м ,9ж) 5 (2м ,3ж) 2013 27 (14м ,13ж) 11 (7м ,4ж) 2014 21 (11м ,10ж) 3 (1м ,2ж) Болеста има рамномерно сезонско пројавување. Географската дистрибуција на болеста покажува најголема застапеност на ЦЕ во скопскиот, кумановскиот, прелепскиот и велешкиот регион. Заклучок: ЦЕ е перманентно присутна во целата наша држава со години наназад. Детската популација е особено вулнерабилна. И кај вкупната популација и кај детската популација бележиме лесна предоминантна засегнатост на машкиот пол. Географската дистрибуција покажува варијации кои се доведуваат во врска со застапеноста на сточарството во поедини региони на Р.Македонија.


Ендемската застапеност на болеста секако имплицира сериозни медицински, ветеринарни, социоекономски како и индивидуални психолошки проблеми. ЦЕ во детската популација наметнува далекусежни последици. Добро разработени превентивни програми инкорпорирани во единствена национална стратегија за справување со болеста е императив.


Cranyosinostosis: Acrocephalosyndactily (Apert syndrome) diagnosed in a newborn

Orhideja Stomnaroska Damceski, Georgi Filev, Elizabete Petkovska, Snezana Jancevska Pediatric department, University Clinic for Gynecology and Obstetritics, Medical Faculty Skopje Case report: We report a 10 days old newborn with brachycephaly, midfacial hypoplasia, syndactily and broad distal phalanx of thumb and big toe. The girl is the second child of young and healthy parents with uneventful pregnancy and delivery. At the 20th gestational weeks an enlargement of the left cerebral ventricle and malformation of the fingers of the hands and toes was noticed on a regular ultrasound examination. The pregnancy continued until vaginal delivery at 40th gestational week. At birth and at the age of 11 months the aforementioned malformations were observed. The large fontal was closed; the small one was palpable at the tip of the finger. Brachicephaly was evident with high full forehead, flat occiput, and irregular cranyosinostosis especially at the coronal suture. There was a supraorbital horizontal groove, hypertelorism with shallow orbites, down slanting palpebral fissures, small nose, and maxillary hypoplasia. Cutaneous syndactily was present at both hands (fingers II-V), with almost complete fusion of the second, third and fourth fingers. Distal phalanges of the thumbs were broad, distal hallux was broad. There was cutaneous syndactily of the feet. Mental development at the age of 11 months was normal. Height and weight were normal. No heart or kidney malformations were detected. X-rays of the skull was not done. MRI is pending, as well as the genetic analysis.

Conclusion: Most frequently Apert syndrome is a sporadic disorder. Inheritance is autosomal dominant. Appropriate management includes surgical treatment of the syndactilies, follow up of the eventual airway compromise and hearing difficulties.


Рационален третман на уринарни инфекции кај деца Велибор Тасиќ Клиника за Детски болести, Медицински факултет, Скопје Уринарни инфекции се на трето место после респираторните и гастроинтестиналните. Се смета дека кај 7% фебрилни девојчиња и 2% машки деца се работи во основа за уринарна инфекција. Третман на уринарна инфекција има за цел да се олеснат симптомите, спречи системска инфекција, како и краткорочни и долгорочни компликации. Затоа е потребно мудро да се користат антимикробните средства кај деца со уроинфекции. Потребно е да се знае локалната состојба со резистентните соеви. Прв чекор е да се препознае уринарна инфекција, што е многу тешко кај новородено, доенче и мало дете, заради неспецифичните симптоми. Кај секое дете кое има немотивирана температура, потебно е да се прегледа обична урина. Кај уринарна инфекција урината е непрозрачна со различит степен на заматеност, постоји леукоцитурија и евентулно позитивни нитрити. Кај бебиња и мали деца каде што урината се фаќа со кесичка контаминација се среќава дури во 50% на случаеви. Од тие причини доволно е да се прегледа урината на заматеност/непрозрачност. Кај бистра,прозрачна урина нема инфекција и нерационално е да се праќа за уринокултура. Иницијалниот третман на дете со уринарна инфекција зависи од општата состојба. Доколку општата состојба и покрај фебрилноста е запазена (се мисли дека нема повраќање) може да се спроведе орален третман со цефиксим (8мг/кг/ден) 10 до 14 дена. Големата студија од Питсбург покажа дека парентерален третман три дена со Цефтриаксон +11 дена цефиксим немал никаква предност во однос на орален третман со цефиксим 14 дена. Алтернативно може да се препише и амоксицилин/клавулонска киселина. Котримоксазолот е неефикасен бидејки кај нас резистенцијата се движи до 50%. Доколку детето е интоксицирано и повраќа иницијално се започнува парентерално со Цефтриаксон или Цефотаксим додека општата состојба не се подобри и започне орален третман. Може да се администрира и аминогликозид (Гентамицин или Амикацин) но не повеќе од 5 до 7 дена, при што треба да се мониторира бубрежната функција заради нефротоксичноста на овие лекови. Аминогликозидите се администрираат во една доза дневно (Гентамицин 3-5 мг/кг/ден, Амикацин 10-15 мг/кг/ден). Кинолоните не се препорачуваат во детскаат возраст заради инхибиторниот ефектот на растот на рскавицата. Може да се администрираат само во случај на комплицирани уроинфекции, или доколку не постои антибиотски избор врз сензитивноста.

Парентерална терапија е индицирана кај бебиња помали од 3 месеци, имунокомпромитирани пациенти, како и кај оние со комплициран пијелонефритис.

Третман на дистални уринарни инефкции трае 3-5 дена. За жал кај нас заради високата резистенција кон котримоксазолот, лек од прва линија е нитрофурантоинот. Се дава 3-5 мг/кг/ден разделено во 3-4 дози. Несаканите ефекти на лекот се манифестираат во гастроинтестиналниот дискомфорт (гадење, повраќање).

Профилакса на уринарни инфекции денеска се оспорува од многу автори. Но сепак кај деца со рефлукс од висок степен (IV, V) степен потребно е да се спроведе нискодозирана профилакса со нитрофурантоин 1-2 мг во една вечерна доза. Во случај на неподносливоста алтернатива се антиобиотици од цефалоспоринска група (цефалексин, цефадроксил, цефиксим). Долготрајна профилакса е индицирана кај cystitis cystic, ентитет кој е доста непознат на педијатрите. Долготраен третман 1-2 години (нитрофурантоин, цефиксим) води до излекување.


Correlation between perinatal risk factors and abnormal brain ultrasound findings

Tavchioska G1, Kochoska B2, Hristoska L2, Koviloska R2, Hristova A3

1 General Hospital Prilep, department of pediatrics 2 General Hospital Prilep, department of neonatology 3 General Hospital Kumanovo, department of pediatrics Aims: to present the prevalence of abnormal brain ultrasound findings among term and preterm newborns and to present their correlation with perinatal risk factors according to National list of risk factors.

Material and Methods: data for the purpose of this retrospective study were collected from the medical histories of the mothers and their newborns born in the General Hospital Prilep from 1.11.2014 to 31.08.2015. Correlation analysis was used to determine the correlation between the ultrasound abnormality and the risk factor.

Results: Out of 45 newborns, 21 (46,6%) had abnormal findings on brain. 8 (38,1%) of them were preterm newborns (33-36 GW). More than one risk factor was present in seven newborns. 15 (71.4%) of the newborns with abnormal ultrasound finding were suffering perinatal asphyxia, one was with conatal infection, two with preeclampsia in the mother.

Conclusion: Ultrasound abnormalities of the brain were significantly associated (p <0,05) with gestational age and APGAR score of less than 5 in the first minute and less than 8 in the fifth minute.


Intraventricular obstructive hydrocephalus – a case report

Tavchioska G1, Duma F2, Angelkova N2, Sabolic V2, Dimovska K2, Kochoska B3, Useini T4

1 General Hospital Prilep, department of pediatrics 2 University Children’s Hospital, Skopje 3 General Hospital Prilep, department of neonatology 4 Clinical Hospital Bitola, department of pediatrics Introduction: Intraventricular obstructive hydrocephalus may occur as a result of brain developmental anomaly, due to intracranial hemorrhage or meningitis.

Case report: we present a case of seven-month old infant, born out of controlled pregnancy by spontaneous delivery, in 36 GW, with APGAR score 4/6/8. PV-IVH gr II (Papille) was found at the age of 7 days. Control brain ultrasound finding in favor of hydrocephalus. Additional neuroimaging investigations placed doubt on the existence of Dandy Walker Variant. Ventricular-peritoneal shunt was placed at the age of two months.Regular follow-up assessments of the infant showed normal neurodevelopment.

Conclusion: ultrasound allows diagnose of early brain damage before the onset of neurological symptoms which is crucial for early intervention.


Етиолошка верификација на акутни долно респираторни инфекции во детска возраст

Б. Грозданова Темелковска1, В. Темелковска2, М. Мишиќ Илиевска1, Т Лалевска3 1Клиничка болница Д-р.Трифун Пановски –Битола , детско одделение 2ПЗУ Д-р. Благица 3ЈЗУ Здравствен дом –Битола Целта на трудот ни беше преку епидемиолошка студија да направиме идентификација на причинителите на долно респираторните инфекции кај децата хоспитализирани на Детско одделение, со што со адекватен третман по антибиограм ќе постигнеме комплетна санација кај истиот без можни комликации и секвели. Иследувани се вкупни 103 деца од новороденачка возраст до 14 години хоспитализирани во првите шест месеци од годината, од кои машки беа 64, а женски 39. Пациентите ги поделивме на три возрасни групни: деца до 3 години, од 4 до 6 години и над 7 години. Како материјал за иследување користевме секрет земен наутро со трахеален аспират или утрински искашлок. Од иследуваните деца со позитивен изолат беа 47, а 56 беа негативни. Од вкупно 70 деца на возраст до три години добивме позитивни изолат кај 36, кај пациентите од 4 до 6 години од 14 имавме 8 позитивни изолати, а кај возрасната групз над 7 години која броеше 19, три деца имаа позитивен изолат. Најчесто изолирана бактерија беше streptococcus pneumoniea и тоа кај 13 деца, staphilococcus aureus беше изолирана кај 11 иследувани, moraxella catharatis ја добивме кај 10 пациенти, hamophillus infulanzea имаа 9 деца, а esherichia colli беше изолирана само кај три деца, додека пак staphilococcus koagulaza negativen беше откриен само кај еден пациент. Најчесто застапената бактериja, streptococcus pnaeumoniae ја имаа 9 деца до 3 годишна возраст, 2 деца од 4 до 6 години и 2 деца над 7 години. Втората бактерија по честота staphilococcus aureus беше изолирана кај 8 деца до 3 години , кај 2 деца од 4 до 6 години и само кај едно дете над 7 години. Moraxella catharalis ја имаа 8 деца до 3 години и 2 деца од 4 до 6 години.Haemophilus influanzea беше присутна кај 8 деца до 3 години и кај едно дете од 4 до 6 години. Escherichia coli пак беше изолирана само кај помалата возрасна група до 3 години. Staphylococus coagulaza neg. беше изолирана само кај 1 дете до 3 години. Можеме да заклучиме дека за долно респираторни инфекции почесто се хоспитализираат деца до 3 години, почесто се јавуваат кај машките пациенти, кај скоро половината од пациентите се работи за бактериска инфекција, а најчесто изолирани бактерии се: Streptococus pneumoniae, Staphylococus aureus, Moraxella catharalis и Haemophilus influenzae. Бактериите Escherichia coli и Staphylococus coagulaza neg. се изолирани кај децата од помалата возрасна група


Dijagnosticirawe na alergiski kowuktivitis vo detska vozrast

V. Temelkovska* ,B. Temelkovska Grozdanova**, T. Lalevska*** * PZU" Dr. Blagica"- Bitola ** Klini~ka bolnica " Dr. Trifun Panovski " detsko oddelenie -Bitola *** Zdrastven dom- Bitola Alergijata prestavuva edna relevantna imunolo{ka patologija i vo detskata vozrast. Ova ja nalaga potrebata za postavuvawe na rana dijagnoza od {to }e rezultira kvaliteten ìvot bez komlikacii i sekveli. Celta na trudot be{e da se dijagnosticira alergija kaj deca so recidivanten kowuktivitis preku primena na alergolo{ki kutani testovi na inhalatorni alergeni so prick metodata i odreduvawe na procentot na eozinofilite vo krvna razmaska boena po MGG.

Vo istraùvaweto se vklu~eni 23 deca na vozrast od 4 do 15 godini. Od niv ma{ki bea 16 , a ènski 7. spored vozrasta od 4 do 7 godini bea ~etiri deca, od 8 do 11 godini osum pacienti , a od 12 do 15 godini edinaeset deca. Sezonski alergi~en konjunktivitis (SAC) e dijagnosticiran kaj 13 pacienti, perenialen alergi~en konjunktivitis (PAC) e kaj 3 pacienti, vernalen keratokonjunktivitis ( VKC) vo 4 slu~ai i atopi~en keratokonjunktivitis (AKC) kaj 3 pacienti. Od vkupniot broj na ispitanici kaj 21 dete dobivme podatok za alergija vo semejstvoto. Posle napravenite kutani testovi konstatiravme, deka 12 deca imaat alergija na polen od treva, kaj 7 deca otkrivme alergija na polen od drvo, dve deca imaa pozitiven test na doma{na pra{ina, edno dete be{e pozitivno na polen od korov i edno dete ima{e alergija na ìvotinski vlakna. Kaj 12 pacienti od isleduvanite najdovme vrednosti nad 0,10% eozinofili vo krvnata razmaska, kaj 9 deca eozinofilite bea od 0,08 do 0.10%, a kaj 2 deca imavme vrednosti od 0,06 do 0,08%. Vrednosti na eozinofili vo krvta nad 0,10% najdovme kaj 3 pacienti so VKC i kaj 3 deca so AKC. Od vkupniot broj na ispitanici od anamnesti~kite podatoci doznavme za postoewe na dermatitis atopica vo doena~ka vozrast kaj osum deca( во сите 3 случаи на AKC), dvajca pacienti imale astmati~ni krizi, a dvanaset deca manifestirale simptomi ñ na rhinitis alergica (кај 11 случаи со SAC).

Moème da zaklu~ime deka alergijata e po~esta kaj deca od ma{ki pol, osobeno e manifestna kaj pacienti so pozitivna familijarna anamneza, kaj site ispitanici imavme zgolemuvawe na procentot na eozinofilite vo krvnata razmaska boena po MGG при што највисоки вредности на еозинофили во крвна размаска беа најдени кај деца со VKC и AKC. Dermatitis atopica е презентиран во сите 3 случаи кај деца со AKC, a rhinitis allergica најчесто се јавува кај пациенти со SAC. Najgolem broj od ispitanicite pokaàa pozitivni testovi na polenskite alergeni a pove}e od 90% manifestirale ñ klini~ka slika na alergija od drug organ so {to ja potvrdija i nivnata atopiska konstitucija.


Laparoscopy in children – anesthesiologic challenges

Dr. I. Temenugova, Dr. T. Spirovska, Dr. R. Selmani, PhD.,

Dr. A.Kartalov PhD., Dr. B. Kuzmanovska PhD., Dr. J. Businoska

Abstract

Minimally invasive surgery or medically known as laparoscopy is a surgical intervention that is well examined in adult population with proven benefits in comparison to the open laparotomy. With the development of technology on one hand, and the medical researches on the other, the past decade there is an increase in interest among pediatric surgeons for this kind of method. Children, infants included, with their anatomic and physiological specificities, require very thorough preoperative, perioperative and postoperative treatment.

The need for such extensive treatment comes from the effects of the iatrogenic pneumoperitoneum primarily on the cardiovascular and respiratory system, but on other organs and organic systems as well. The effects are caused by the increased intraabdominal pressure as a mechanic force, by the hypercapnia formed from CO2 apsorption as an insufflating gas, and intesification of those effects by the need of certain positions, sometimes even extreme. Certainly, as any other surgical or medical intervention, laparoscopy is followed by complications, which in skilled and experienced hands are minimal and transitory.

The motivation for writing of this review paper is to present the challenges that an anesthesiologist must face during laparoscopic surgeries in children.

Keywords: iatrogenic pneumoperitoneum, increased intraabdominal pressure, CO2

apsorption, extreme positions, benefits, complications


Pregolema upotreba na antibiotici vo primarna zdravstvena zastita

S.Todorovska1, S.Smugreska2, M.Trenceva3, K.Cekic4

1)Pzu Pantelejmon, 2)PZU Dr Smugreska, 3)PZU Dr Trenceva, 4)PZU Pinokio Golema e dilemata za antibiotic vo primarna zdravstvena zastita.Antibiotici se propisuvaat bez dokazana bakteriska infekcija.Problemite se I so pacientite ,a I so doktorite.Pacientite setaat od doctor do doctor,sakaat antibiotic.Se stvara golema rezistencija na antibioticite.Se slucuva da se otpise pacient od doktorot dokolku ne mu bide propisan antibiotic I da se zapise kaj doktorot koj mu propisal antibiotic,po negova zelba. Ne se sledat pokazatelite za dokazana bakteriska infekcija (krvna slika,urina ,CRP,brz strep test),sto moze da se primenuvaat vo primarna zdravstvena zastita.Ne se primenuvaat doktrinarni stavovi.Se davaat antibiotici preventivno,sto e pogresno.Ne sekoe crveno grlo e streptokok,brziot strep test ke ni ja rascisti dilemata ,antibiotic da ili ne.Vo najgolem procent vo primarna zdravstvena zastita se zastapeni gornorespiratornite infekcii od virusna etiologija.Kaj sekoe febrilno dete I so golo oko moze da se vidi urina dali e bistra ili matna za da ne se ispusti urinarna infekcija,a isto I so brzite test lenti.

Zaklucok:Pri sekoe davanje na antibiotic,dobro da se proceni I da se napravat potrebnite isleduvanja pred da se dade ,kako b I se izbegnalo stvaranjeto na rezistencija.I site doktori da se pridrzuvaat na doktrinarnite stavovi,odnosno medicina bazirana na dokazi.


Use of Nasal colonization with MRSA, for the surveillance of late-onset healthcare-associated infections in the Neonatal Intensive Care Unit of Tirana`s University Maternity

Hospital “K. Gliozheni” E. Tushe*, G. Rjepaj**, V. Kasneci**, B. Dardha* Neonatal Department *, Microbiologic Laboratory ** Abstract Introduction Neonatal intensive care units (NICUs) provide care for high-risk infants, including preterm and seriously ill full-term newborns.The need for prolonged stay, along with the large use of invasivelife support measures,increase the opportunity for acquisition of late-onset healthcare-associated infections (HAIs). Over 60% of NICU`s infections are caused by Gram+ S.aureus, Enterococcus spp. and coagulase – Staph.). Methicillin-resistant Staphylococcus aureus (MRSA) is the major nosocomial pathogen worldwide. NICU patients may be particularly susceptible to colonization, infection and severe complications caused by this organism. Frequency MRSA, is considered a quality indicator of performance of prevention and control measures in a specific NICU. Objective To give a picture of actual situation, concerning MRSA frequency in our NICU. To introduce the roles of “colonization pressure,” in acquisition of MRSA in neonatal intensive care units, as quality indicator in infection control measures. Methods Setting The one room, 20 bed NICU is located in a referral University Maternity Hospital with about 5000 births/year (with ≈30% outborn babies). There are 6 intensive beds, 12 sub intensive and 2 for outpatients. Patient admission is approximately 400 patients per year. There are situations of overcrowding and understaffing (4 nurses in a shift and > 20 neonates). Unit is open for mothers; Kangaroo mother care is used and the performance with hand washing is considered good. Methods It was a prospective cohort study, in university-affiliated NICU. In study were included all NICU-babies, born from 1 Jan – 31 March 2015. Every Tuesday, a nasal specimens were obtained using cotton-tip swabs. Routine clinical cultures were obtained as clinically indicated. We analyzed also antimicrobial resistance profiles. Colonization was defined as isolation of MRSA from nares, and infection according to the international protocols (locally adapted). It was calculated infection rates during period of study, colonization pressure, acquisition rates, and prevalence risk for sepsis. Results During study period were admitted in NICU 145 patients. 5 patients had a infection (1.06/1000 pts days). Colonization pressure was 11.4/1000pts days with acquisition rates 0.42, and prevalence risk for sepsis 0.036. Conclusions NICU population is particularly vulnerable to infections, especially premature ones, necessitating support with multiple devices and for prolonged time. We are conscious for the limitations of the study, but it was only one effort to demonstrate a proper strategy to measure the risk for nosocomial infections. Maybe some approaches in our unit, as KMC, minimal handling, breast milk from the beginning, , handwashing, could explain low colonization pressure. Maybe we need to extend the microbiological control, also to other microbes, strongly related with nosocomial infections.


Clinically significant HDFN- causing anti-erythrocyte antibodies during pregnancy

E. Velkova, T. Makarovska-Bojadzieva, V. Dejanova- Ilieska, I. Nikolovska, J. Angelovski, J. Samonikov-

Tosevska, M. Blagoevska

Introduction: Maternal aloimmunization to erythrocyte antigens is possible during any pregnancy. Anti- D antibodies are the most frequent cause of Hemolytic Disease of Foetus and Newborn (HDFN), but non D antibodies can cause HDFN of different degree as well. Knowing antibody specifity is essential in prognosis and management of HDFN. Purpose: The purpose of this issue is to represent results of anti- erythrocyte antibodies screening in 10-year period. Materials and methods: Screening for anti- erythrocyte antibodies was performed in14842 pregnant women and 200 newborns; elution and identification of antibodies was performed by using microgel method. Results: Of total 14842 pregnant women, antibodies were found in 204, with 213 aloantibodies causing HDFN of different degree:

Aloantibodies Senzited No therapy Photo therapy EST

Intrauntenne hydrops Hb<go g/l

D 132 64 (48%) 43 (32,5%) 25 (18,9%) C 13 9 (69%) 4 (31%) 0 c 9 5 (55%) 3 (33%) 1 (11%) E 10 9 (90%) 1 (10%) 0 K 1 0 1 (100%) 0 k 1 0 1 (100%) 0 Jkb 1 0 1 (100%) 0 I 3 3 (100%) 0 0 Lea 2 2 (100%) 0 0 Leb 14 14 (100%) 0 0 M 2 1 (50%) 1 (50%) 0 неодредена 25 25 (100%) 0 0

Вкупно 213 132 (62%) 55 (25%) 26 (11,8%)

Conclusion: In 62% of sensitized mothers, newborns didn’t present with symptoms of HDFS; in 25% newborns presented with symptoms of medium – grade HDFN and 11.8% with symptoms of high grade HDF, hydrops and death. According to results anti D and C antibodies are most significant.


Overweight and asthma – what is the true link?

Emilija Vlashki

University Children's Clinic, Skopje

An epidemic of overweight/obesity accompanied by an increase in prevalence and incidence of adult and childhood asthma in developed countries worldwide has been observed in the last few decades. A great number of cross-sectional, prospective and longitudinal studies have reported an increased risk of asthma development in overweight/obese adults and children with inconsistent results regarding atopic status, overweight/obesity onset and duration, gender and airway hyperresponsiveness. Several theories to explain the complex association between overweight/obesity and asthma include an impact of genetic/epigenetic factors, environmental factors (diet/nutrition especially prenatal and infant diet, physical activity) and mechanical factors with altered lung function and GERD. Additionally, there is a theory related to immunologic mechanisms (Th-2 dependant and Th-2 independent) which may be involved through adipokines and pro-inflammatory cytokines (adipose tissue - derived leptin, adiponectin, IL-6, TNF-?) in a development of systemic inflammation or in an airway inflammation (non-eosinophilic pattern with neutrophilia). A non-inflammatory mechanism (disruption of autonomic nervous system through leptin) with resulting bronchoconstriction has been as well postulated as an underlying mechanism. Despite the consistent evidence linking overweight/obesity to asthma in a distinct clinical asthma phenotype, characterized by a more severe disease and poor steroid responsiveness, the true link between them remains a mystery. An urgent clarification of the definitive mechanism of the relationship is an imperative for an adequate diagnosis and optimal treatment of obese children suffering from asthma.


Tetralogy of Fallot - Case report

M. Xhaferi, R. Kacarska ,K. Kuzevska - Maneva, B.Gjurkova-Angelovska, V.Jovanovska M.Neshkovska-

Shumenkovska, M.Kacarska, D.Nonkulovski, J.Gjorgievska, I.I Petkovska, Gj.PaskalovDepartment of

Cardiology - University Children’s Hospital, Skopje, Macedonia

Background and aims : Tetralogy of Fallot is a cardiac anomaly that refers to a combination of four

related heart defects that commonly occur together. The four defects are :

Ventricular septal defect (VSD)

Overriding aorta

Pulmonary stenosis

Right ventricular hypertrophy

It is the most common cyanotic heart defect.

Children with a TOF will sometimes appear blue around the lips, mouth or finger beds. This is called

cyanosis.

Methods :We present a case of a one year old male patient with a congenital heart defect, prenatal

diagnosed. The child was diagnosed with Tetralogy of Fallot. Immediately after the diagnoses was

confirmed in the department of cardiology. During echocardiographic examination there were

noticed : a large VSD ,RVOT obstruction, RVH.

Results : Based on the assigned diagnosis for the child, a cardiac intervention was performed by total

correction of TOF. It was closed a VSD and was widened a RVOT by resection and it was implanted

patch.

Conclusion : Early complete TOF repair can be accomplished with a low mortality. Longer follow-up,

with emphasis on arrythmias and right ventricular function, is required to define the long-term

benefits of early repair


Anxiety during chemotherapy in childhood cancer

And art therapy

Yllka Voka1; Gentjana Çekani2; Anila Godo3; Mirela Xhafa3; Donjeta Bali3

1Psychologist 2Social worker 3Oncohemaologist

- atService of Pediatric Onco-Hematology, - University Hospital Center “Mother Theresa”, Tirana, Albania.

Introduction: Literature shows that art therapy affects anxiety symptoms in childhood cancer. Aim: was to evaluate the influence of art therapy at anxiety level during chemotherapy treatment in childhood cancer. Method:comparative research, for data analysis was used SPSS statistic program. Assumptions: H.-art therapy influences the anxiety level of children who are treated with chemotherapy. Participants: children, age 8 to 14 years old, who received treatment for malignant diagnosis, at the service of Onco-Hematology, UHC “Mother Theresa”-Tirana, Albania. The sample was divided in two groups: the intervention group who received art therapy and the control group who did not received art therapy. Procedure: art therapy was applied as children weretaking chemotherapy. Instruments were completed before and after taking chemotherapy. Measuring instruments: for measurement of anxiety were adapted: the Depression, Anxiety and Stress Scale (DASS-21) and The Hospital Anxiety and Depression Scale (HADS). Results: Statistical analyses consisted of descriptive statistic and statistic significance through t-test for comparing of two groups. From the comparison of the results between two groups resulted significant differences in the level of the significance p=0.05 for of variables of the study. The results of statistical analysis supported the hypothesis of the study. Conclusion: Art therapy influences the anxiety level of children who are taking chemotherapy. Findings of this study support the application of art therapy in children who are suffering from malignant disease and who are treated with chemotherapy. Key words: art therapy, anxiety, childhood cancer.


Reasons for irrational antibiotic therapy in children, in outpatient polyclinic practice in R.Macedonia

Zafirovski Lj.1 Matevska L.2 Panovski N.3 Stamenkovic H.4 Petrova G.5 Koviloska R.5 Zafirovski M.7

1Institute for Respiratory diseases in children, Skopje, R.Macedonia 2University Clinic of Anesthesiology, Reanimation and Intensive Care, Faculty of Medicine, Skopje, R.M 3University Institute of Microbiology, Faculty of Medicine, UKIM, Skopje, R.Macedonia 4University Clinic for Children's Internal Diseases, Clinical Center Nis, Republic of Serbia 5University pediatric clinic, UKC, Sofia, Bulgaria 6Children's department., PHI General Hospital, Prilep, Republic of Macedonia 7University Dental Clinical Center,-“St.Pantelejmon”, Skopje, Republic of Macedonia Introduction: Although we are frequently informed,for rapidly growing bacterial resistance,to antibiotics-(AB), and toxic,allergic,immuno-depressoring,etc.harmful,antibiotic-activity!!!,but however,-AB,are still too often prescribed,in R.M.!!!,(although children,particulary-in collectives,in=96-98%,suffer from acute respiratory-viral-infections,for which -AB,are powerless,useless,harmful,expensive)!!! Objective: We searched reasons for irrational use of antibiotics in pediatric outpatient polyclinic practice in RM Material and Methods: We interviewed doctors and parents,of=970-(small,+pre-school-children,and pupils),-patients,of the IRDCh-Kozle-(hospital),+Duty-pediatric-services,(in polyclinic”J.Sandanski”),+from General Hospitals,in various cities in-RM,+from family-doctors,etc. Results: From interviewed doctors and patients,we listed the following reasons,for often prescribing antibiotics,-(arranged according to their frequency): 1).-Uneducated doctors and difficult clinical distinguish viral from bacterial-infections; 2).-Patient’s and doctor’s?fear,that our local children,(who too often,received antibiotics),had already been destroyed theirs-immunity,and is risky to remain them without antibiotic? 3).-Ignoring epidemiological anamnesis; 4).-Unavailable rapid diagnosis-(PCR,haematological,Rtg,biochemical, microbiological,etc.). 5).-Too literally,interpretation-of:-haematological,biochemical,microbiological-"findings", etc; 6).-Insufficient˂time↓,after the"mine-term",(for explanations,to patients that there is no drug for virus-infection)!!! 7).-Pressures of ignorant patients who-themselves,require-AB,because they-"always",were given-AB,harmful accustomed to previous-doctors). 8).-Fight for:"payment by quantitative-effect",and need,to win over the more impressing patients,with multiple-medications! 9).-Fear of losing already-"conquered"-patients,if they do not give them-AB; 10).-Fear if undertake-"unsatisfied"-patients,from other-colleagues,who too easily, frequently-prescribed-AB; 11).-Bad and harmful-habits of physicians,(ambulatory,and rescue-doctors on duty), in the crowd,(without wasting time for evaluation whether children need-AB or not?),but summarily-prescribed-AB! 12).-Overproduction,and over-employment,of˃doctors↑ in RM,especially in-Skopje,plus non-collegiality, and non-solidarity,unfair-competition,without dialing,a manner-"clutch and consume"-more˃patients↑, impressing them with more,over ˃drugs↑. Conclusion: It is necessary reformation,of antimicrobial-treatment in R.Macedonia and its adaption to the EU and international-worldwide standards,as examples of developed health-care systems.


Insufficiently,is just a locally activity,only from few individuals-enthusiastic-doctors-practitioners,but also systemic,(sanction-award),etc.,and measures designed and coordinated by the Ministry of Health,etc.


Recidivate laryngitis episodes and their correlation with allergic constitution in childhood

Zafirovski O, Dacevski D, Galbova S, Buzarov J, Sarevska Z

Institute for Respiratory Diseases in Children-Kozle, Skopje, Macedonia

Aim. To determine the degree of correlation between repeated laryngitis episodes and allergic component.

Material and methods. Study period -5 years (2007-2012), included -561 children with treated laryngitis. From them, 104 (18.53%) patients with medical history for repeated laryngitis episodes and hospital treatments. All clinical investigations were performed including skin prick tests (allergy predisposition) and personal and familiar allergy evidence analyze.

Results. Skin prick tests positive results were confirmed in 53 (50.96%) patients. From them, in correlation with the basic disease, allergic constitution was determined in 39 (73.58%) patients. The most positive allergen causes: pollens -24(45.28%), Dermatophagoides ptt.-19 (35.84%). Negative results on allergic skin prick tests- 39 patients (with positive anamnesis of personal/familiar allergy-17 (43.58%), allergic dermatitis- 8, asthma- 6, recidivate broncho-obstructive episodes -5, positive asthma anamnesis – 3). Allergy was not evidenced in only 12 (11.53%) patients.

Conclusion. Repeated laryngitis episodes have high degree of significant correlation with allergic constitution in childhood. Allergy is appeared as very important factor in repeated laryngitis episodes etiology. The underlying cause of repeated laryngitis episodes necessarily have to be diagnosed and treated.

Key words: paediatric pulmology, laryngitis, allergy, children


Brain abscess – a rare complication of neonatal meningitis Nikolina Zdraveska, Anet Papazovska-Cherepnalkovski, Natasa Aluloska, Snezana Palcevska Department for Neonatology, University Children’s Hospital, Skopje, Macedonia Brain abscess is rare in the neonatal age-group. It occurs as a complication of bacterial meningitis and sepsis. Citrobacter koseri and Proteus species are the most common causative organisms of neonatal brain abscesses in the published literature. Klebsiella is a common cause of neonatal sepsis, but rarely associated with brain abscess and until now only few cases have been reported, mainly considering preterm babies. We report a term neonate without any obvious risk factors for infection, who developed large brain abscesses following Klebsiella pneumoniae meningitis. The child presented with generalized clonic seizures on 23 day postnatal age. CSF examitnation confirmed meningitis and the response to the antimicrobial treatment was poor. The diagnosis of hydrocephalus and brain abscess was made with ultrasonogram and cranial tomography. Surgical abscess aspiration, ventricular peritoneal shunt placement, as well as prolonged antibiotic course was provided. The interest of the case is in the rarity of the causative organism in term infant and the atypical features, such as not clear onset of infection and negative blood culture.

All newborns with diagnosis of bacterial meningitis should be constantly monitored for any signs of acute neurological complications that would necessitate additional evaluation. Although rare the possibility of developing brain abscess should be keep in mind in neonatal infections.


Listeria monocytogenes – well known but rare pathogen in neonates Nikolina Zdraveska, Anet Papazovska-Cherepnalkovski, Natasa Aluloska, Snezana Palcevska Department for Neonatology, University Children’s Hospital, Skopje, Macedonia Listeria monocytogenes is a very important life-threatening bacteria in certain risk groups including neonates. Although very rare in many countries, it is still recognized as a cause of severe neonatal infection. The presentation is indistinguishable from other causes of neonatal sepsis and meningitis. We report clinical and laboratory findings, treatment and outcome in 2 newborns treated with Listeria monocytogenes septicemia and meningitis in the period of Listeria outbreak in our country. Both were term neonates, admitted to our Department in the first 24-hours postnatal age. The main presenting symptoms were fever and respiratory distress. Antibiotic treatment for early neonatal sepsis was immediately started. Beside the therapy, the second neonate showed progressive worsening of general condition with high fever, erythematous rash, and bulging fontanel, thus required control lumbar puncture, which was suggestive for bacterial meningitis, as well as antimicrobial regimen change. The blood cultures identified Listeria monocytogenes in both children by automated identification and antibiogram system (VITEK 2, bioMérieux). The outcome was good in both cases. There was no epidemiological relation although the neonates came from the same maternity unit. L. monocytogenes infection in neonates is a serious concern. We should always consider Listeria as a possible aetiological agent of sepsis and meningitis in neonatal age group, considering that cephalosporins, which are the most commonly used antimicrobials for neonatal infections in our circumstances are unlikely to elicit a favourable outcome in such cases.


Children’s rights through the accreditation of the hospitals Zisovska Elizabeta, Isahi Lirim, Darkovska Tanja Agency for Quality and accreditation of health care institutions, Skopje Republic of Macedonia

The process of accreditation ensures and confirms the quality of health care for the patients.

The evaluation of the quality could be performed directly (observation, procedures’ checks, documents, proofs of implementation of pre-defined standards and criteria, quantitative and qualitative results of the hospital care), and indirectly, through patient satisfaction analysis. The aim of this paper is to discuss some social and ethical problems and obstacles in the evaluation of the QuHC in pediatric practice. The most frequent dilemma is in the field of standards concerning collaboration with the patients and their associations.

Within the set of standards Management and Stewardship, critical are standards for patient safety and reporting adverse events. In this important topic, patients’ associations play a role, but the associations are constituted of parents of children, as their guardians. There are also two more standards different of those aimed for the care of adults, as for example respecting the rights of the patients, and conducting clinical trials, which are very few in pediatric population, just because understandably strict eligible criteria. The plan for improvement of the quality of care comprises also of survey for patient satisfaction. But, are pediatric patients appropriately represented by their parents/guardians in the survey? Is there possibility for discordance between patient satisfaction (children) and their parents?

In the Chapter Risk management and patient safety, there are many dilemmas regarding participatory approach by the patient. The prevention of the intrahospital infections requires collaboration of the patient. In what extent the child as a patient can participate in this procedure? Children carry many risks regarding transmission, taking into consideration their way of living, behaving, communication, and using same toys, cups and plates. What is the role of the health care professionals in protection of the children’s rights and obligations in such cases?

In the Chapter for patient’s treatment, the treatment plan has to be reviewed together with the patient, in pediatrics, with the parents. Do always parents have enough knowledge and awareness about the treatment benefit? Is it a case that insufficient information leads to harmful decisions (negative campaigns led by the parents in terms of treatment of their children)?

The last Chapter, specific clinical services, the section about intensive care requires strict control over the risk of adverse events. The literature data report 3-4 fold increased risk in pediatric intensive care, and even higher risk in neonatal intensive care units. Does the registration and reporting is appropriate, because the children and neonates cannot react properly and on time? What is the strength of the control over the medical errors, especially for neonates, where the medications sometimes are more than ten times diluted?

Many of these issues have to be discussed within professional associations, and between medical professionals and parents. Key words: patient, accreditation, adverse event


Neonatal asphyxia in phi clinical hospital stip During time period of 5 years (2010-2014)

V.Zlatkovska, M.Gacova, L.Doneva, J.Breslieva, E.Shpritova

PHI CLINICAL HOSPITAL - STIP, REPUBLIC OF MACEDONIA

The transition ofthe newborns from intrauterine to extrauterine life usually goes without problems,

and bylittle or no assistance at all. The consequences ofimproperhelp can last during lifetime period,

and can even lead todeath. Neonatal asphyxia is a pathological condition of improper breathing,

resulting in low levels of oxygen and blood in the tissues, and increased concentration of carbon

dioxide.

OBJECTIVE AND METHODS: A retrospective analysis is made regarding cases withneonatal asphyxia

of the total number of births, during time period of five years. During data processing, the following

criteria were evidenced: gender, weight, gestational age, type of birth, transport to tertiary

institution, and lethal cases.

RESULTS: A group of 4503 newborns were evidenced during time period of five years (2010-2014), of

which 79 cases were evidenced with asphyxia (1,75%).

Cases per year: 2010-16(2,17%), 2011-16 (1,99%), 2012–15(1,57%), 2013–15(1,47%), 2014 –

17(1,71%);

Regarding gender: male 60,94%, and female 39,06%;

Regarding birth weight: 38,05% with birth weight less than or equal to 2.500 g, 58,1% with

birth weight between 2500 and 4000 g, and 3,83% with birth weight above 4000 g;

Regarding gestational age: 42,61% of the cases belong to the group ofprematurely born,

while 57,39% to the group of on-time delivered newborns. Of the total number of newborns

with asphyxia, 36 cases (45,6%) were transported in a tertiary institution. In this period,

thenewborn mortality due to asphyxia at the department of neonatology is 7,6%.

CONCLUSION: The asphyxia is a major problems in neonatal pathology. Competent and proper

reanimationalong with symptomatic therapy leads to a decrease of late sequelas at newborn.


Неонатални асфиксии во јзу клиничка болница-штип Во период од 5 години (2010-2015 )

В.ЗЛАТКОВСКА, М.ГАЦОВА, Л.ДОНЕВА, Ј.БРЕСЛИЕВА, Е.ШПРИТОВА ЈЗУ КЛИНИЧКА БОЛНИЦА ШТИП , Р.МАКЕДОНИЈА

Транзицијата на новородените од интраутерин на екстраутерин живот, најчесто минува без проблеми и со пружање малку помош или без помош. Последиците од неадекватната помош можат да траат цел живот или да доведат до смртен исход. Неонатална асфиксија е патолошка состојба која е резултат на неадекватно дишење и резултира со ниски нивои на кислород во ткивата и крвта и зголемена концентрација на јаглерод диоксид.

ЦЕЛ И МЕТОДИ: Направена е ретроспективна анализа на вкупниот број асфиксии од вкупниот број раѓања во тек на пет години. При обработката на материјалот е следен полот, телесна тежина, гестациска возраст, начин на раѓање, транспорт во терциерна установа и летални исходи.

РЕЗУЛТАТ: Евалуирана е група од 4.503 новородени во период од пет години (2010-2014) од кои со асфиксија се 79 (1,75%).

• По години се движи: 2010-16 (2,17%), 2011-16 (1,99%), 2012-15 (1,57%), 2013-15 (1,47% ), 2014 -17 (1,71%);

• Спрема полот: машки 60,94% , а женски 39,06%.• Според РТ: 38,05% со РТ помала или еднаква на 2500 g , 58,11% со РТ од

2500-4000 g и 3,83% со РТ над 4000 g.• Според гестациската старост: 42,61% се јавува кај прематурни, а 57,39% кај

доносени новородени. Од вкупниот број новородени со асфиксии, во установа одтерциерен тип се транспортирани 36 (45,6%). Во овој период смртноста од асфиксии нанеонатолошкиот оддел изнесува 7,6%.

ЗАКЛУЧОК: Асфиксијата е еден од главните проблеми во неонаталната патологија. Стручната и адекватна реанимација заедно со симптоматска терапија води до намалување на касните секвели кај новородените .


A Survey of Caregivers of Children with Autism in Republic of Macedonia Zorcec Tatjana1, Daniels Amy2, Shih Andy3, Jasmina Trosanska4

1University children’s hospital, Skopje, Macedonia 2The Simons Foundation, New York, USA

3Autism Speaks, New York, USA 4pecial school d-r Zlatan Sremec, Skopje, Macedonia

Introduction:Autism spectrum disorders (ASD) is a group of complex lifelong neurodevelopmental disorders, characterized by difficulties in social interaction, verbal/nonverbal communication and repetitive behaviors.Statistics from the United States Centers for Disease Control and Prevention, identify one in 68 children (one in 42 boys/one in 189 girls) as having autism. The rapid growth of autism prevalence over the last few decades represents a significant health, social and economic issue. Aim of the study: survey to investigate the needs of children with autism and the challenges and difficulties faced by them and their families in seeking and receiving care in the Republic of Macedonia. Method: a survey was developed by Autism Speaks and the National Coordinatorsfor autism from eight countries of the Southeast European Autism Network to address the needs and difficulties of the ASD children and their families. Subjects: From January to August 2015, 60 primary caregivers of ASD childreninRepublic of Macedonia completed the survey. Results: Parents first concerns about the children’s development occurred at 20 months (±15.23), and the average age at diagnosis was 29 months (±9.49). The most common first concerns included poor eye contact, notresponding to child’s name, living in “own world” and unusual gestures/walking/posture. 81.66% of the childrenreceived combination of sensory, speech/language treatments, and medications.Treatments were typically provided by private special educatorsand the majority (78.57%) of parents are payingfull amount for months or years. Majority of the children are attending state kindergartens (40%) and special schools (41.66%). Parents ranked obtaining ASD information mainly from the internet, professionals and other parents. Autism has caused serious financial problems for the families(in 75%) andemotional issues for the parents (in 70%). The primary challenges ranked by families include poor communicational skills (40%),difficulties in social interactions (35%), andstereotyped behaviors of the child (33.33%). Parents rangedfirstly difficulties in anda need for improved health care services(72.41%), then improved educational possibilities (65%) and enhanced social care system(51.66%). Conclusion: Autism is serious burden for the families in Republic of Macedonia. Parents and ASD children are facing financial and personal issues and would need improved services from the health, educational and social system. Key words: autism, caregiver needs survey, Republic of Macedonia


Joint Attention, Symbolic Play and Engagement Regulation (JASPER) in Children with Autistic Spectrum Disorders: Case Report

Tatjana Zorcec1, Connie Kasari2, Stephanie Yoshiko-Shire2, Caitlin McCracken2, Anastazija Spasovska1, Valentina Dukovska1, LidijaTrpkovska1

1University Children’s Hospital, Skopje, Macedonia 2University of California Los Angeles (UCLA), Los Angeles, USA

Abstract Introduction:Autistic spectrum disorders (ASD) is a serious neurodevelopmental disorder which is impairing a child's ability to communicate/interact with others and also includes restricted repetitive behaviors, interests and activities. These issues can producesignificant impairment in social, cognitive, occupational and other areas of functioning.

Aim of the study:a case report of a boy with ASD and his progress achieved during the six monthsimplementation of the early intervention treatment program.

Subjects and methods: a single ASD child, aged 20 months, diagnosed and treated for ASD at the University Children’s Hospital, Skopje, Macedonia and supervised by University of California Los Angeles, Center for autism research and treatment. For the assessment of the child’s abilities (joint attention skills, requesting skills and play skills)we have used Short Play and Communication Evaluation (SPACE) in the first session and six months later. As a treatment method we have appliedJoint Attention, Symbolic Play and Engagement Regulation (JASPER). The objective for every JASPER session was to increase joint engagement, expand play skills and promote nonverbal and verbal communication.

Results:in the first six months of the treatment,young boy has achieved significant improvement in the initiations and regulation of thebehavior, enhanced joint attention and eye contact,developed new type and improved play skills, achieved better interaction with the therapists and family members, developed better verbal and nonverbal communication. Moreover, the child has achieved generalization of thesenewly acquired skills in the home environment and kindergarten too.Conclusion:Intervention with JASPER seems to be very effective for this child with ASD. Further studies on a group of young children with ASD are needed.

Key words:autistic spectrum disorders, JASPER, case report


Quality of life in patientswith cystic fibrosis

Tatjana Zorcec, Stojka Fustik, Lidija Spirevska, Tatjana Jakovska

University Children’s Hospital, Medical Faculty, Ss. Cyril and Methodius University, Skopje, Macedonia

Abstract

Introduction: Cystic fibrosis (CF) is a genetic, life-threateningdisease that affects many body organs, but mostly the lungs and digestive system. It’s a very compoundcondition, with various forms and manifestation of the symptoms. A variety offactors like age of diagnosis, number, type and control of infections, treatment options, comorbid conditions, etc. can affect patient’s general health, progressionof the disease and his/her quality of life. In present times, majority of the CF patients will achieve adulthood, so the quality of life is crucial for the generalhealth and daily functioning.

Aim of the study: to evaluate the quality of life in patients with CF in Republic of Macedonia.

Subjects and methods: in the study we have enrolled 67 CF patients, divided in two age groups-up to 14 (mean age 9.3±3.14) and above 14 years of age (19.62±5.98). We have producedquestioners specially designed for this study, with questions addressing their daily coping with CF.

Results: generally, majority of the patients (68.42%) with CF live normal life, have common and frequent social interactions(in 74.81% of the cases), emotionally are stable (in 83.06% of the cases) but stillthe fear from the stigma in the society is present. Conclusion:regardless ofthe various issues CF patients must overwhelmedin the daily life, they still could have completeand meaningful lives and cope with the condition in a productive way.

Keywords: cystic fibrosis, chronic illness, quality of life

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КНИГА СО ТРУДОВИ - zpm.org.mk · Remedika - Skopje and Acibadem Sistina Hospital Skopje, each. Only one newborn with CH was - Only one newborn with CH was - obtained