Child with cholestasis and Child with cholestasis and massive hepatomegaly massive hepatomegaly

Marija Dimitrovska-IvanovaFaculty of Medical Sciences - StipUniversity Children’s clinic-SkopjeRepublic of Macedonia

Medical historyMedical history

Patient: B. Lj. 2.5 years, femaleThird child from third normal pregnancy.Born normaly in the 41 gestational week with born

weight 3150g, born length 50 cm and APGAR SCORE 9/10.

The baby was breast feed just for a month and than was introduced formula milk until one year. Other non milk food was administrated in 4 months of age. Poor weight gain during infancy.

Because of frequent respiratory infections child was treated by a family doctor with wide spectrum antibiotics per os.

In the last 5 months with frequent stools, decreased appetite and poor weight gain.

Clinical status at admissionClinical status at admissionChild was taken to the family pediatrician who noticed

jaundice, large abdomen and massive hepatomegaly.She was first hospitalized in General hospital in Ohrid

and after excluding viral hepatitis was transferred to the University Children’s Clinic – Skopje

At admission girl was in malnutrition and in bad general condition, she looked seriously ill, prostrated, febrile, tachicardic, icteric . Her weight was under 3% il (BW = 9 kg ) and her height on the 3 % il for age.

Auscultation of lungs - vesicular breathing with crackles.

Abdomen - Above the chest, distended and hard on palpation. Liver was palpable 9 cm under right cost arch and fulfilled the left hypochondrium

Laboratory evaluationLaboratory evaluation

Hgb = 97…88…72…149…128 g/lEr = 3,04…2,38…2,48…5,27…4,41 x 10^12/lLe = 13,7…12,3…13,1…9,3…9,1 x 10^9/lSed rate = 67…44 CRP= 38,8…42,2 mg/lFe serum = 6,6 µmol/l. K= 2,9…3,6…4,3ABS - Ph = 7,49 HCO3 = 33,1 mmol/l, pO2 = 7,77

kPa (hypoxemia), Pco2 = 5,82 kPa Total proteins = 46...54...65…54g/l Аlbumen=23…

30…22…26 IEPh, alfa – fetoprotein, alfa – 1- antitripsin,

ceruloplazmine and antibodies for autoimmune hepatitis type 1 and type 2 were normal

Serum creatinin, urea and amino acid screening in plasma and urine were normal

Laboratory evaluationLaboratory evaluation

Liver tests – Tot. bil. = 40...52...94…38, Conj. bil.= 26...38....19...18

AST= 93…68…317…139 U/LALT= 41…43…60…52 U/L, GGT= 123…200 U/L, ALKP= 109 U/LThe protrombin time was 51s (normal <13 s) and

PTT was 71 s (normal <33 s). TTG test – negativeSweat test = 110 mmol/lGenotype – F 508 del/ F 508 delLiver biopsy – steatosis of liver with poor chronic

inflamatory infiltrate

Ultrasound of abdomen-hepatomegaly, Ultrasound of abdomen-hepatomegaly, the liver is extremely hiperechogenuousthe liver is extremely hiperechogenuous

CT of abdomen - Liver with homogeneous CT of abdomen - Liver with homogeneous structure with hypodensity on parenchyma and structure with hypodensity on parenchyma and

present hepatomegaly that fulfills the left present hepatomegaly that fulfills the left hemiabdomenhemiabdomen

On tracheal aspirate was

isolated Staphylococcus

aureus and Pseudomonas

aeruginosa

Chest x ray Consolidation in projection of the

lingula of the left lung lobe

TreatmentTreatment Parenteral antibiotic therapy according to antibiogram

(S. aureus and P. aeruginosa) Amp. Meronem i.v and per os trimetoprim sulfotetoxasole.

I.V Albumin was administrated in 4 times, Er transfusion, Vit. K, ursodeoxycolic acid and hepatoprotectiv therapy.

Pancreatic enzymes, oral nutrition energy supplementation, supplementation of liposoluble vitamins A, D, E, K and beta – caroten.

After one month child was discharged in general stable condition with better appetite, normalization of stools, progressive weight gain, without respiratory symptoms.

Follow up treatment - eradication therapy for P. aeruginosa (Colomycin for 3 months), Pancretic enzymes, Vit. ADEK, hyper caloric meals, oral nutrition energy supplementation and physical therapy.

Current nutritional status Current nutritional status

Child is follow CF outpatient clinic. She was no hospitalized during 3 years.The child is making a good weight gain BW= 20,3

kg (75 %il) Body height= 105 cm(10 %il) and BMI= 18,4.

On the last control with gradual reduction of hepatomegaly, but with present nodular cirrhosis.

Without exacerbation on lung desease.

ConclusionConclusion

Delayed diagnosis of Cystic fibrosisEarly begining of liver disease with signs of liver

insufiency.Steatosis and nodular cirrhosis of liver.