NEONATAL HYPERBILIRUBINAEMIA-lmmu1 (1).pdf

Pediatrics notes on neonatal jaundice

1. NEONATAL
HYPERBILIRUBINAEMIA

2. Objectives
1.Define neonatal jaundice
2.Differentiate pathological jaundice from physiological jaundice
3.Describe various causes of neonatal jaundice
4.Review evidence-based management options of neonatal jaundice

3. Definition
Neonatal jaundice or neonatal hyperbilirubinaemiaresults from elevated total
serum bilirubin and clinically manifests as yellowish discoloration of skin , sclera
and mucous membranes

4. Etiology
1.Unconjugated Hyperbilirubinaemia( more common type)
Physiological(75%): physiological alteration in neonatal bilirubin metabolism
TSB levels in neonates are higher than in adults (< 1mg/dl) due to :
-increased mass and a decreased RBC lifespan.
-compromised clearance of bilirubin due to impaired activity of UGT(1% activity of adult level)
-increased enterohepatic circulation
-It appears after 24 hours of age, peaks at around 48-96 hours and resolves by 2-3 weeks in full-
term neonates
Pathological:
-Presents on the 1
st
day of life
-TSB > 95
th
centile for age based on age-specific bilirubin normograms
-Bilirubin levels rise by more than 5 mg/dl/day (0.2 mg/dl/hr)
-Jaundice beyond 2-3 weeks in full-term neonates

5. Immune-mediated haemolysis:
ABO incompatibility
Rhesus incompatibility
Non-immune mediated haemolysis:
RBC membrane defects:
Hereditary spherocytosis
Elliptocytosis
G-6-PD deficiency
Pyruvate kinase deficiency
Sequestration:
Cephalhaematoma
Intracranial haemorrhage
Polycythaemia
Sepsis

6. Increased Bilirubin Production
Immune-mediated haemolysis:
ABO incompatibility
Rhesus incompatibility
Non-immune mediated haemolysis:
RBC membrane defects:
Hereditary spherocytosis
Elliptocytosis
G-6-PD deficiency
Pyruvate kinase deficiency
Sequestration:
Cephalhaematoma
Intracranial haemorrhage
Polycythaemia
Sepsis: causing oxidative damage to RBCs
Decreased Bilirubin Clearance
Due to quantitative or qualitative defects in the uridine diphosphate
glucuronosyltransferase (UGT) enzyme.
•Crigler-Najjar type I: AR ( complete absence of UGT activity): Bilirubin encephalopathy
•Crigler-Najjar type II: retain some of the activity of UGT enzymes ( No bilirubin
encephalopathy)
•Gilbert syndrome: decreased UGT production( mild jaundice )
Miscellaneous Causes
•Infant of Diabetic Mother: Polycythaemia
•Congenital hypothyroidism: decreased hepatic uptake of bilirubin, impaired
UGT activity, and sluggish gut motility.
•Drugs : displacement of bilirubin from albumin, affecting albumin binding (sulfa
drugs, ceftriaxone, and penicillins)
•Intestinal obstruction: increased bilirubin recycling by augmenting the
enterohepatic circulation.
•Pyloric stenosis:
•Breast milk jaundice
•Breastfeeding jaundice.

7. Breastfeeding jaundice
•Also known as breastfeeding failure jaundice
•Occurs in the first week of life
•Is due to inadequate intake of breast milk leading to dehydration and sometimes
hypernatremia.Breastfeeding failure
•Leads to decreased intestinal motility and decreases the elimination of bilirubin in the
stool or meconium.
Breast milk jaundice
•Occurs late in the first week, peaks in the second, and usually resolves by two weeks of
age.
•It is thought to be mainly due to inhibition of the UGT enzyme by pregnanedioland
deconjugationofconjugated bilirubin in the intestines by beta-glucuronidasepresent in
breast milk.

9. 2. Conjugated Hyperbilirubinemia(CHB) or Direct Hyperbilirubinemia
•Also referred to as neonatal cholestasis
•Is characterized by elevation of serum conjugated/direct) bilirubin (> 1.0 mg/dL)
•Is due to impaired hepatobiliary function.
•Is almost always pathologic and warrantsprompt evaluation and treatment.
•Thecauses of neonatal cholestasis/CHB are extensive and can be classified into
the following categories:
a. Obstruction of biliary flow:Biliary atresia, choledochalcysts, neonatal
sclerosingcholangitis, neonatal cholelithiasis

10. b.Infections:TORCHES,UTI
c.Geneticcauses: Alagillesyndrome, alpha-1 anti-trypsin deficiency,
galactosemia, fructosemia, Tyrosinemiatype 1, cystic fibrosis
d.Miscellaneous:Idiopathic neonatal hepatitis, parenteral nutrition induced
cholestasis

11. Pathophysiology
Neonatal physiologic jaundice results from simultaneous occurrence of the
following 2phenomena:
•Bilirubin production is elevated because of increased breakdown of fetal
erythrocytes. This is the result of the shortened lifespan of fetal erythrocytes and
the higher erythrocyte mass in neonates.
•Hepatic excretory capacity is low both because of low concentrations of the
binding protein ligandin in the hepatocytes and because of low activity of
glucuronyltransferase, the enzyme responsible for binding bilirubin to glucuronic
acid, thus making bilirubin water soluble (conjugation).

12. •Bilirubin is produced in the reticuloendothelial system as the end product of
hemecatabolism and is formed through oxidation-reduction reactions.
•Approximately 75% of bilirubin is derived from hemoglobin, but degradation of
myoglobin, cytochromes, and catalase also contributes. In the first oxidation step,
biliverdinis formed from hemethrough the action of hemeoxygenase, the rate-
limiting step in the process, releasing iron and carbon monoxide.
•The iron is conserved for reuse, whereas carbon monoxide is excreted through
the lungs and can be measured in the patient's breath to quantify bilirubin
production.

13. History & Physical Examination
The evaluation of the neonate with jaundice
starts with :
•A detailed history, including maternal, birth
and family history
•The onset /duration/ progression of jaundice
•Maternal serologies.
•Color of stool and urine presence of pruritis
Clinical presentation:

14. Unconjugated Hyperbilirubinaemia
Management

15. Aim
The aim of management of neonatal jaundice is to prevent and avoid kernicterus in case of
unconjugated bilirubinaemia.
Modalities:
Phototherapy
Exchange Transfusion
Pharmacotherapy
Investigations

18. 1. Phototherapy

19. 2. Exchange Blood Transfusion

20. 3. Pharmacotherapy

21. KERNICTERUS
1. Definition

22. KERNICTERUS
Clinical features

23. KERNICTERUS
Management

24. Conjugated Hyperbilirubinaemia
Management

26. Extra Didactic Slides

38. References