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CHAPTER 1
INTRODUCTION
1.1. Background
Malnutrition can be defined as a state of nutrition in which deficiency or excess of energy,
protein, and other nutrients causes measurable adverse effects on tissue and body form and
function, and clinical outcome. Malnutrition can be of the acute, chronic or mixed type.
Acute malnutrition is the type that usually occurs in illness, but children with underlying
chronic diseases whoare admitted to the hospital because of an acute illness can also presentwith chronic malnutrition. Anthropometric variables are used to define nutritional status
worldwide but various classification systems and cutoff points are used to define
malnutrition. One such classification method includes kwashiorkor and marasmus. These
terms were originally established to describe syndromes of protein-energy malnutrition in
children in developing countries. The most used classification system was that described by
Waterlow, in which acute and chronic malnutrition were divided into four stages, on the basis
of the actual weight to the 50th percentile of Weight For Height for acute malnutrition and
the actual height to the 50th percentile for height for chronic malnutrition.
Childhood malnutrition is a disease of relevance and importance to public health. These
children exhibit elevated morbidity and increased prevalence of hospital admissions. When
admitted to the hospital they are not generally subjected to anthropometric assessment and do
not, therefore, receive nutritional support. Hospital acquired malnutrition is caused by a
reduction in the hospitalized children nutritional intake and increase in their calorie
requirements as a result of morbidity. There is an identified number of contributing factors tothe widespread existence of under nutrition among hospitalized child and sometimes leads to
exacerbation of his or her nutritional status.
Examples are interference with meal times by ward rounds, investigations and procedures.
Nil-by-mouth orders may be used inappropriately or prolonged unnecessarily. A patient
may be kept nil-by mouth all morning only to find that their treatment has been cancelled or
delayed. In addition, many drugs cause anorexia, taste changes, nausea, vomiting or
constipation, thereby reducing food intake. The psychological outcome of hospitalized
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children remains little studied and poorly understood. Children have been observed to be
anxious, withdrawn, fearful, restless, and angry or demonstrate hostile behaviors and
therefore, contributing to malnutrition.
Cholestasis is defined as reduced bile flow and abnormal accumulation of conjugated
bilirubin, indicating impaired hepatobiliary function. Conjugated bilirubin is considered
abnormal if it is 1 mg/dl or above when the total bilirubin is less than 5 mg/dl or more than 20
percent of the total bilirubin when the total is above 5 mg/dl.
Cholestasis occurs in approximately 1 in 2,500 births. Biliary atresia and neonatal hepatitis
account for most cases. The other etiologies of cholestasis are numerous and include
anatomic obstruction (such as bile sludging and choledochal cyst), infection (such as urinary
tract infection and CMV), metabolic disorders (such as galactosemia and tyrosinemia),
genetic disorders (such as alpha-1 antitrypsin deficiency, cystic fibrosis and Alagille
syndrome), endocrine dysfunction (such as hypothyroidism and panhypopituitarism) and
toxins (such as TPN).
The primary care physician is critically important in the evaluation of the jaundiced infant. It
is recommended that all infants with persistent jaundice beyond 2 weeks old be assessed with
a fractionated bilirubin. In healthy breast-fed infants with no signs of cholestasis, thisinvestigation can be postponed until 3 weeks old. If conjugated hyperbilirubinemia is present,
prompt referral to a pediatric gastroenterologist for further evaluation is imperative.
Developmental Disability/Delay (DD) is present when functional aspects of a childs
development in one or more domains are significantly delayed compared to the expected
level for age. These functional aspects are gross/fine motor, speech/language, cognition,
social/personal, and activities of daily living. Global Developmental Delay (GDD) is a subset
of Developmental Disability/Delay, defined as significant delay in two or more
developmental domains. However, this is reserved for children less than 5 years old. An
estimated 12-16% of children have a developmental and/or behavior disorder.
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CHAPTER 2
LITERATURE REVIEW
2.1. HOSPITAL ACQUIRED MALNUTRITION
2.1.1. DEFINITION
Malnutrition can be defined as a state of nutrition in which deficiency or excess of
energy, protein, and other nutrients causes measurable adverse effects on tissue andbody
form and function, and clinical outcome.The World Health Organization (WHO) defines
malnutrition as the cellular imbalance between the supply of nutrients and energy and the
bodys demand for them to ensure growth, maintenance, and specific functions. This
dynamic imbalance of nutrients affects children differently than adults and can have profound
implications for the developing child.
The most used classification system was that described by Waterlow,1 in which acute
and chronic malnutrition were divided into four stages, on the basis of the actual weight
to the 50th percentile of WFH for acute malnutrition and the actual height to the 50th
percentile for height for chronic malnutrition.
In 1992, the international statistical classification of disease and related health
problems used weight, expressed as SD scores, to define the probability of malnutrition.
For example, an SD score between 1 and 2 (representing 13.5% of the reference
population) indicates a probability of mild malnutrition and an SD score of less than
2 indicates a probability of severe malnutrition (2.3%). This statistical approach does
not use weight-for-height index and does not define the reference population. In 1999, theWorld Health Organization
2 recommended an additional classification for malnutrition in
children, which became widely used. The likelihood of malnutrition is defined using a
cutoff point of 2 SD. A child with a SD score between 1 and 2 is no longer defined
as malnourished. According to these WHO criteria, a SD score for WFH between 3 and
2 can be considered as moderate malnutrition and a SD score below 3 as severe
malnutrition.
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Acute malnutrition (severe and moderate) is defined as one of the following:
WFH SD score less than 2,
WFH less than 80% of the median,
% ideal body WFH less than 80,
WFH less than 5th percentile,
BMI SD score less than 2.
Chronic malnutrition is defined as:
Height for age (HFA) SD score less than 2,
HFA less than 90% of the median,
HFA less than 5th percentile.
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Hospital acquired malnutrition refers to nutrient imbalance acquired during hospitalization
and may occur with or without pre-existing malnutrition, or malnutrition that was present
prior to hospital admission. The mechanisms of nutrient imbalance in illness-related
malnutrition include decreased nutrient intake, altered utilization, increased nutrient losses, or
increased nutrient requirements (hyper metabolism) not matched by intake.
2.2 CAUSES
Hospital acquired malnutrition occurs largely among children admitted to hospital with an
underlying disease.
Cardiac disease
Cardiac cachexia refers to a syndrome of protein-energy malnutrition seen in patients
with chronic cardiac disease. A high prevalence percentage of low WFH is reported
most commonly in patients with chronic congestive failure, chronic shunt hypoxemia and
nosocomial postoperative acute and chronic states. Various studies3-5among children
with various cardiac diseases (e.g. congenital heart disease, idiopathic dilated
cardiomyopathy) showed prevalence rates between 18 and 64% on admission. The
highest rates were found in cardiac surgical patients and in children with congenital
heart diseases and left-to-right shunt.
Cystic fibrosis
Malnutrition is an extremely substantial complicating factor in patients with cystic
fibrosis. A poor nutritional status is a negative prognostic factor and malnutrition and
deterioration of lung function are interrelated and inter- dependent. Substantial
improvements in medical management including nutritional therapy have been made.
In the USA, in 1999, it was reported that 24% of the cystic fibrosis patients had a weight
less than the 5th percentile. In a very large study in the USA6, children below 1
year and above 10 years appeared tobe at more risk of acute malnutrition than children
aged 110 years.
Malignancy
Malnutrition in childhood cancer is a common and serious problem. Besides the
previously mentioned con- sequences of malnutrition, it is associated with adecreased tolerance to chemotherapy. Although malnutrition is not uniformly found in
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all pediatric malignancies, certain types of malignancies are at high nutritional risk (solid
tumors, medulloblastoma, acute nonlymphocytic leukemia, and multiple relapse
leukemia). Furthermore, marked differences in the prevalence of malnutrition will be
found between children on therapy and those off therapy. The use of body weight to
assess nutritional status in pediatric cancer patients has been misleading because of the
confounding effects of tumor mass. Up to 50% of pediatric patients with malignancies
were reported to be undernourished. Recently, a study6reported a prevalence rate of
9.1% acute malnutrition (
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Neurological disorders
In general, poor nutritional status and growth is often seen in children with
neurological disorders. It is advocated to use specific weight charts for specific diseases
such as Duchennes muscular dystrophy. The preciseprevalence varies depending on
the criterion by which malnutrition is defined, the degree of mental retardation, the
presence of associated problems, treatment administered, and socioeconomic and family
environment. In a study, eight different criteria were used to define the nutritional
status of mentally retarded children on the basis of anthropometric variables. The
prevalence of malnutrition increased with age, increasing intelligence quotient deficit and
cerebralpalsy.
In children with Duchenne muscular dystrophy, malnutrition occurs after the age of 14
years, involving 54% of boys at about 18 years of age. Evaluating developmentally
disabled children is often difficult because they do not fit into normal standards for
assessment. In children with cerebral palsy, malnutrition is associated with the degree
of feeding dysfunction. A s t u d y found in a mixed population7, using the criterion
less than 80% WFH, malnutrition in 24% of the children with mental retardation.
In conclusion, a summary was given of the prevalence of malnutrition in a selection ofchildren with an underlying disease. The main finding is that malnutrition is still
highly prevalent in children with an underlying disease. In children with chronic
inflammatory diseases such as chronic kidney disease, both acute and chronic mal-
nutrition remain highly prevalent, probably due to the ongoing inflammatory state. For
diseases such as inflammatory bowel disease and AIDS, the nutritional status is also
dependent on the degree of inflammation
2.3 PATHOGENESIS
Inflammatory conditions may increase requirements for nutrients while promoting a nutrient-
wasting catabolic state. Illness-related malnutrition is associated with an inflammatory
component. Inflammation promotes skeletal muscle breakdown, mediated by a cytokine-
driven pathway. Critical illness or injury promotes an acute inflammatory response that has a
rapid catabolic effect on lean body mass. The acute phase inflammatory response isassociated with elevated resting energy expenditure and nitrogen excretion and thereby
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energy and protein requirements, respectively. Nutrition supplementation alone only partly
reverses or prevents muscle protein loss in active inflammatory states. The anorexia that
accompanies inflammation will promote further loss of lean tissue if nutrition intake is
inadequate. Over the past decade, it has become increasingly evident that the
pathophysiology of disease or injury-associated malnutrition invariably includes acute or
chronic inflammation that affects body composition and biological function.
The inflammatory condition may be short-lived or chronic in nature with the severity being
influenced by the progression and extent of underlying illness/disease condition. Loss of
muscle mass and function may occur insidiously in the chronic disease state over months to
years. It is important to recognize the presence or absence of a systemic inflammatory
response in the malnourished state, as it affects the response to intervention. In the absence of
inflammation, as seen in malnutrition due to starvation, appropriate nutrient interventions
may be successful in treating malnutrition. On the other hand, the presence of inflammation
may limit the effectiveness of nutrition interventions, and the associated malnutrition may
compromise the clinical response to medical therapy. If inflammation is present, then it is
useful to clarify whether it is mild, moderate, or severe and transient or sustained. The
recently proposed adult malnutrition definition has suggested that acute disease- related
malnutrition is probably associated with a severe degree of inflammation and chronic disease-related malnutrition with a mild to moderate degree of inflammation.
However, the role of inflammation and currently available inflammatory markers, such as C-
reactive protein (CRP) or erythrocyte sedimentation rate, in classifying pediatric malnutrition
severity has not been adequately described.
Inflammatory cytokines can impair growth via multiple pathways. Anorexia, skeletal muscle
catabolism, and cachexia affect the growth plate via insulin-like growth factor 1 (IGF-1)
independent or IGF-1dependent pathways.The inhibitory effects of tumor necrosis factor
(TNF-)and interleukin (IL)1 on the growth plate are reversed by antiIL-1 and anti
TNF-. The effect of TNF- on IL-6 transcription and circulating leptin level may be
reversed by infliximab. In pediatric Crohns disease, growth retardation may result from a
complex interaction between nutrition status, inflammation, disease severity, and genotype,
which causes resistance to the effects of growth hormone. Elevated serum concentration of
CRP is one of the most common nontraditional markers used to stratify cardiovascular risk,
and it has been used to identify patients with chronic inflammation as it reflects a pro-
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inflammatory state. IL-6 concentrations may be an important marker of early inflammatory
response with serial levels correlating with nutrition status in critically ill children.Although
there is no doubt about the association between inflammatory state and nutrition recovery, the
precise nature of this relationship remains elusive.
2.4 PREVENTION
To prevent malnutrition and especially hospital-acquired malnutrition, the risk of
nutritional depletion needs tobe identified at the time of admission so that appropriate
nutritional intervention can be initiated at an early stage. Routine nutritional screening is
rarely carried out inpediatric patients due to the lack of a simple and valid nutritional
screening tool. Only two screening tools have been published since 2000 to identify
children at risk of malnutrition. Sermet-Gaudelus et al.8described a simple pediatric
nutritional risk score, which is suitable for routine use to identify patients at risk of
malnutrition during hospitalization. Nutritional risk was assessed prospectively in 296
children by evaluating various factors within 48 h of admission. Multivariate analysis
indicated that food intake less than 50%, pain, and grades 2 and 3 pathologic
conditions were associated with weight loss of more than 2%. These significant risk
factors were scored (one point for food intake
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divided children into the three groups with significantly different mean values for
various anthropometric measures. SGNA was considered a valid tool for assessing
nutritional status in children and identifying those at higher risk of nutrition-associated
complications and prolonged hospitalizations. Both methods described by Sermet-
Gaudelus et al. and Secker and Jeejeebhoy link nutritional status to outcome. Both
methods have their limitations in use. The tool of Sermet-Gaudelus et al. needs a period
of 48 h after admission to complete and the study results of Secker and Jeejeebhoywere
not based on a single assessor but were a composite of the data of five asessors. For both
methods, skilled staff are necessary and theprocedures seem to be time-consuming.
3.1. CHOLESTASIS
3.1.1. DEFINITION
Neonatal cholestasisis defined as impaired canalicular biliary flow resulting in accumulation
of biliary substances (bilirubin, bile acids and cholesterol) in blood and extrahepatic tissues.
Jaundice is a common clinical finding in the first 1 to 2 weeks after birth and usually
resolves spontaneously. Any infant who is jaundiced beyond 2 to 3 weeks after birth needs
further evaluation to rule out neonatal cholestasis.10
3.1.2. PATHOPHYSIOLOGY
The normal process of bile production involves two mainprocesses: uptake ofbile acids by
hepatocytes from the blood and excretion of bile acids into the biliary canaliculus. Uptake of
bile acids from sinusoidal blood is an active process at the sinusoidal membrane of the
hepatocytes. Na taurocholate cotransporting polypeptide (NTCP) and organic anion trans-
porting proteins (OATP) are the two main receptors involved in the uptake of conjugated bile
acids by the liver cells. These receptors are also responsible for the transport of other an- ions
like drugs and toxins through the hepatocellular membrane. At the biliary canaliculus, bile
salt export pump (BSEP) and the multidrug resistant proteins MRP2 and MDR3 are
involved in the secretion of bile acids into bile. These pumps are present in the canalicular
membrane.
In newborn infants, the biliary system is both structurally and functionally immature making
them more susceptible to cholestasis. In hepatitis and sepsis, there is down regulation of the
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NTCP and OATP receptors resulting in decreased bile production and cholestasis. Various
genetic defects in the transporter proteins have been recognized in familial cholestasis
syndromes, eg, mutation of BSEP gene in progressive familial intrahepatic cholestasis type 2
(PFIC), defect in the MDR3 in PFIC type 3.
3.1.3 CLASSIFICATION
The differential diagnosis of neonatal cholestasis is extensive and can be classified based on
the anatomic location of the pathology into extrahepatic and intrahepatic causes. Biliary
atresia and choledochal cyst are examples of extrahepatic
causes while common intrahepatic causes include idiopathic neonatal hepatitis, infections,
1-antitrypsin deficiency and other metabolic disorders.The different causes of cholestasis can
also divided into broad etiological categories like infectious, metabolic, toxic, chromosomal,
vascular disorders and bile duct anomalies.
The causes of cholestasis are as below:
1) Idiopathic neonatal hepatitis
2) Infections
Viral
Cytomegalovirus
Rubella
Reovirus3
Adenovirus Coxsackie
Virus Human herpes
Virus 6
Varicella zoster
Herpes simplex
Parvovirus
Hepatitis B and C
Human immuno-deficiency virus
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Bacterial
Sepsis
Urinary tract infection
Syphilis Listeriosis
Tuberculosis
Parasitic
Toxoplasmosis
Malaria
Bile duct anomalies
Biliary atresia
Choledochal cyst
Alagille syndrome
Non syndromicbile duct paucity
Inspissatedbile syndrome
Caroli syndrome
Choledocholithiasis
Neonatal sclerosing cholangitis
Spontaneous bile duct perforation
Metabolic disorders
1-antitrypsin deficiency
Galactosemia
Glycogen storage disorder type IV
Cystic fibrosis
Hemochromatosis
Tyrosinemia Arginase deficiency
Zellwegers syndrome
Dubin-Johnson syndrome
Rotor syndrome
Niemann Pick disease, type C
Gauchers disease
Bile acid synthetic disorders
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Progressive familial intrahepatic cholestasis
North American Indian familial cholestasis
Aagenaes syndrome
X-linked adreno-leukodystrophy Endocrinopathies
Hypothyroidism
Hypopituitarism (Septo-optic dysplasia)
Chromosomal disorders
Turners syndrome
Trisomy 18
Trisomy 21
Trisomy 13
Cat-eye syndrome
Donahues syndrome (Leprechauns)
Toxic
Parenteral nutrition
Fetal alcohol syndrome
Drugs
Vascular
Budd-Chiari syndrome
Neonatal asphyxia
Congestive heart failure
Neoplastic
Neonatal leukemia
Histiocytosis X
Neuroblastoma
Hepatoblastoma
Erythrophagocytic lymphohistiocytosis
Miscellaneous
Neonatal lupus erythematosus
Le foie vide(infantile hepatic non regenerativedisorder)
Indianchildhoodcirrhosis
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3.1.4 CLINICAL PRESENTATIONS
An infant with cholestasis usually presents with prolonged jaundice, pale stools and dark
urine. Acholic stools are a cardinal feature of cholestasis and should be promptly evaluated.
Some infants may present with signs of coagulopathy due to deficiency of clotting factors or
vitamin K deficiency. Neurological abnormalities like irritability, lethargy, seizures and poor
feeding may indicate either sepsis or metabolic disorders.
Physical examination is remarkable for jaundice. Hepatomegaly is common. Splenomegaly
may be seen in infants with advanced liver disease. Other physical findings may include
growth retardation seen in congenital infections and syndromic facial dysmorphisms.
Choledochal cyst can present as a mass in the right upper quadrant
3.1.5 DIAGNOSIS
Any infant presenting with jaundice beyond 2 weeks after birth should be immediately
evaluated for cholestasis. A detailed history (including family history, pregnancy and
delivery history and postnatal course) and physical examination could provide clues to a
specific diagnosis. Breast- fed infants who can be reliably monitored and have an
otherwise normal history and physical examination should be reevaluated at 3
weeks of age and if still jaundiced, have fractionated serum bilirubin levels checked at that
time.Once cholestasis is established, further investigations should be done in a stepwise
manner to establish the specific cause of cholestasis. The investigations should first rule out
conditions requiring immediate intervention like sepsis, metabolic disorders like
galactosemia, glycogen storage disorders and other endocrinopathies. Once they have
been excluded, the next step is to look for biliary atresia. It is important to establish or
rule out biliary atresia early because of better prognosis if the patient undergoes surgical
intervention before 60 days of life. If biliary atresia has been excluded, further
investigations should be done to establish the cause of intrahepatic cholestasis. The
potentially extensive evaluation of an infant with cholestasis should be individualized to
efficiently and promptly establish a diagnosis.
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3.1.6 Management
Medical management of cholestasis is mostly supportive and does not alter the natural course
of the disease. It is aimed mostly at treating the complications of chronic cholestasis like
pruritus, malabsorption and nutritional deficiencies andportal hypertension.10
4.1 GLOBAL DEVELOPMENTAL DELAY
4.1.1 DEFINITION
Developmental Disability/Delay (DD) is present when functional aspects of a childs
development in one or more domains are significantly delayed compared to the expected
level for age. These functional aspects are gross/fine motor, speech/language, cognition,
social/personal, and activities of daily living. Global Developmental Delay (GDD) is a subset
of Developmental Disability/Delay, defined as significant delay in two or more
developmental domains.
Children with developmental delays often are identified early in life, because they fall
significantly behind their age-mates in meeting developmental milestones. For example, a
young child may be slow to roll over, to understand his or her name, or to exhibit fine motor
skills. Parents of infants often worry when their second child takes longer than the first to
display a specific ability. In fact, the range of ages within which an infant should be able to
perform any given skill is broad. Differences in personality can also result in variations in
developmental progress. Nevertheless, special educators and medical doctors find that the
behaviors and abilities of children who have developmental delays are well outside the age
ranges for almost every developmental benchmark.
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Identifying a child with a developmental delay involves going through a set of evaluative
processes, including intelligence tests, developmental scales, adaptive behavior evaluations,
and tests of general knowledge. Evaluation tools such as intelligence tests and behavioral
scales are normed on a large sample of the population over a long period of time, and the
scores from these sample assessments are distributed along a curve, offering a picture of how
the measured attributes occur in the general population.
Figure 4.1 illustrates a bell curve (or normal curve), the graphic shape that depicts scores on
any standardized measure. On such a curve, the mean (average) score falls in the middle, and
a statistical measure called a standard deviation is used to indicate the distance of a given
score from the mean. When educational evaluators describe children with developmental
delays, they are talking about children whose assessment scores fall at least two standard
deviations below the mean. As you can see from the figure, this means that the childrens
scores are lower than those of 95 percent of the population used to establish the norms for the
test.
4.1.2.CAUSES
The primary cause for developmental delays in school-aged children is genetic abnormalities.
For example, phenylketonuria (PKU) is a single-gene disorder also referred to as an inborn
error of metabolism. PKU leads to mental retardation and other developmental delays if
untreated in infancy because the body is unable to produce proteins or enzymes needed to
convert certain toxic chemicals into nontoxic products or to transport substances from one
place to another (Glanze, 1996). Infants with untreated PKU appear to develop typically for
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the first few months of life, but by twelve months of age most of them will have a significant
developmental delay and will be diagnosed with mental retardation before they start school.
Down syndrome is an example of a chromosomal disorder. Chromosomal disorders happen
sporadically and are caused by too many or too few chromosomes or by a change in structure
of a chromosome. In the case of Down syndrome, the children have recognizable physical
characteristics and limited intellectual endowment because of the presence of an extra
chromosome 21.
Similarly, fragile X syndrome arises from a single gene located on the X (female)
chromosome. It is the leading inherited cause of mental retardation.
Other causes of developmental delays include these:
Problems during pregnancy. Use of alcohol or drugs by a pregnant mother can cause
mental retardation and developmental delays in the child. Research suggests that
smoking also increases the risk of developmental delays. Other risks include
malnutrition, certain environmental contaminants, and illnesses of the mother during
pregnancy, such as toxoplasmosis, cytomegalovirus, rubella, and syphilis. Pregnantwomen who are infected with HIV may pass the virus to their child, leading to future
neurological damage.
Problems at birth. Although any birth condition of unusual stress may injure the
infants brain, prematurity and low birth weight predict serious problems more often
than any other conditions.
Problems after birth. Childhood diseases such as whooping cough, chicken pox,
measles, and HIB disease (which may lead to meningitis and encephalitis) can
damage the brain, as can accidents such as a blow to the head or near drowning. Lead,
mercury, and other environmental toxins can cause irreparable damage to the brain
and nervous system. It is important to note that some children with developmental
delays have problems caused by abuse or neglect. Although accidents and injuries can
result in brain damage, it is often difficult to determine whether the childs problems
existed prior to the accident.
Measured by both intelligence and adaptive behavior measures, approximately 1 percent of
the general population has developmental delays. According to states data reported to the
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4.1.4 MANAGEMENT
Management of children with developmental delays are usually in the form of therapy. The
therapies are
Occupational and physical therapy. Therapy can help children with motor skills (such
as increasing range of motion and fine motor skills); perceptual skills (for instance,
helping a child track an object in two- or three-dimensional space); and social-
emotional skills (working in groups and taking turns). Occupational therapy also
focuses on the use of adaptive and assistive technologies.
Speech/language therapy.This type of therapy can help children with articulation and
expressive disorders; it also boosts receptive language skills.
Psychotherapy and psychiatric therapy. Broadly speaking, psychological therapy
helps children with the process of recognizing, defining, and overcoming
psychological and interpersonal difficulties. School psychologists are also responsible
for administering many of the assessment inventories mentioned earlier. Psychiatrists
have medical credentials and are responsible for managing any medication therapy the
child may receive for psychological issues, such as anxiety, depression, and sleep
disorders.
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CHAPTER 3
CASE REPORT
Name : AD
Age : 1 years 11 month
Sex : Male
Date of Admission : Augusts, 16th 2013
Main complain: Yellowish pigmentation throughout the body. Patient has been experiencing
this approximately 1 week ago before visiting the hospital. Initially, it started on both eyes,
spreading on face and throughout the body. Fever was found about 1 week ago, not too high
and subsided with medication. Convulsion or seizure was not found. Patient was found
shivering during fever.
Patient was also suffering from diarrhea since 3 days ago, with a frequency of less than 3
times a day. The consistency of the feces was watery with residues. It was slimy indicating
the presence of mucus. However, blood was not found.
Patient's urine was in a colour similar with dark tea (dark and brownish) since 1 week ago.
At the time of the physical examination, patient is unable to face down and lift his head. He is
only able to tilt his head to the left and right.
Patient was given food and drinks through NGT because he is not able to eat and drink
properly with his mouth.
History of previous illness: Patient was treated in a hospital before at the age of 3 months
old with the diagnose of coagulated blood due to trauma and had an head operation.
Physical Examination
Body weight : 5.5kg
Height : 53.4 cm
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Presence status
Sens. Compos Mentis, Body temperature: 36.5 oC, Pulse: 152 bpm, Respiratory Rate: 40bpm.
Locali zed status
1.
Head : Microcephalic Eye : Light reflexes(+/+), isochoric pupil, conjunctiva
palpebra inferior ane (+/+), icteric (+/+) , Ear : Normal appereance ,
Mouth : Sianosis (-), Nose: Normal appereance.
2.Neck : Lymph node enlargement (-)
3. Thorax : Icteric (+) Symmetrical fusiformis. Epigastria retraction (-).
HR:152bpm, regular, murmur (+) systolic grade III-IV HR: 40bpm,
regular. Crackles (-/-), interposed rib clearly visible
4. Abdomen : Distention (+), symmetrical, Decreased tenderness, Peristaltic
movement normal. Liver: palpable 3cm from costal margin,
concentration kenyal, flat surface, Pain(-). Spleen : Normal
5.Extremities : Pulse 152 bpm, regular, adequate pressure and volume, warm
extremities, CRT< 2.
Working Diagnosis : Cholestasis Jaundice + Global Development delay + Microchepaly
Diffential Diagnosis : - Cholestasis Jaundice + Global Development Delay + Microchepaly
- Suspected of hepatitis
- Suspected of sepsis
- Microchepaly
Medication : IVFD D 5% NaCl 0.2 25% 20gtt/I micro
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Follow Up
August, 16th 2013
S:Yellowish skin colour on the whole body (+)
O:Sens: CM, Temp: 37 oC
Head Microsefali ,Eye : Light reflexes(+/+), isochoric pupil, pale conjunctiva
palpebra inferior (-/-), icteric (+/+) , Ear : Normal appereance ,Mouth :
Sianosis (-), Nose: Normal appereance Face icteric (+)
Thorax Ikteric (+), Symmetrical fusiformis. Retracsi (-).
HR: 139 bpm, reguler, murmur (-)
RR: 28 bpm, reguler. Crackles (-/-)
Abdomen Icteric (+), symmetrical, Decreased tenderness, Peristaltic (-). Liver:
palpable 4cm from costal margin, sharp edge, flat surface, Pain(-)Spleen:normal
Extremities Pulse 139 bpm, regular, adequate pressure and volume, warm acral, CRT1 bulan : 0,2-1,0
4-6 hari : 0,1 -12,6
3 hari : 0,7 -12,7
2 hari : 1,3-11,3
< 1 hari :
Bilirubin
Direct
19,39 Mg/dl 0-0,2
HBsAg NEgatif
TV:
0.01
Negatif Indeks : =0,13
Alkaline
Phospatase
2515 U/L P: 53-128 W:42-98
SGOT 121 U/L P:
8/10/2019 Lapkas Anak (Prof Dr. Hj. Bidasari)
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Laboratory Result:August,5th 2013
Blood Gases
Ph 7.174 7.35-7.45
pCO2 mmHg 60.8 38-42pO2 mmHg 164.3 85-42
Bikarbonat (HCO3) mmol/L 21.9 22-26
Total CO2 mmol/L 23.7 19-25
Kelebihan Basa (BE) Mmol/L -6.6 (-2)-(+2)
Saturasi % 98.6 95-100
Carbohydrate
metabolism
Glukosa ad random Mg/dl 362.00
8/10/2019 Lapkas Anak (Prof Dr. Hj. Bidasari)
27/74
August, 17th 2013
S:yellowish skin colour on the whole body (+)
O:Sens: CM, Temp: 37 oC
Head Microsefali ,Eye : Light reflexes(+/+), isochoric pupil, pale conjunctiva
palpebra inferior (-/-), icteric (+/+) , Ear : Normal appereance ,Mouth :Sianosis (-), Nose: Normal appereance Face icteric (+),
Thorax Ikteric (+), Symmetrical fusiformis. Retracsi (-).
HR: 132 bpm, reguler, murmur (-)
RR: 36 bpm, reguler. Crackles (-/-),
Abdomen Icteric (+), symmetrical, Decreased tenderness, Peristaltic (-). Liver:
palpable 3cm from costal margin, sharp edge, flat surface, Pain(-)
Spleen:Normal
Extremities Pulse 132 bpm, regular, adequate pressure and volume, warm acral, CRT
Recommended