Neonatal Cholestasis

Question 1

Definition

Answer 1

If TSB

<5mg/dl increase in conjugated bilirubin to >/=1 mg/dl

> 5mg/dl increase in conjugated bilirubin to>/=20%

Suspected when jaundice persist>14 days

Question 2

Cause

Answer 2

Extrahepatic
1. Extrahepatic biliary atresia (EHBA)/ Non Cystic Obliterative Cholangiopathy (MC overall)
2. Choledochal cyst

Interahepatic
1.Idiopathic neonatal hepatitis (mc)
2. Infection - TORCH , neonatal sepsis, UTI
3. Metabolic- galactosemia, tyrosinemia, alpha 1 trypsin deficiency, cystic fibrosis, PFIC
4. Genetic condition - down syndrome, Edward syndrome, alagille syndrome

Question 3

Classification of EHBA

Answer 3

Perinatal type - 70%

BASM syndrome - 15%
Biliary atresia
Splenic malformation
Situs inversus
Cardiac defect
Malrotation of GIT

Other 15% a/c renal anomalies & choledochal cyst

Question 4

Investigation

Answer 4

1 LFT
2. Markers of cholestasis
3. High frequency USG (HUS)- triangular cord sign
4. HIDA scan
5. Liver biopsy best

Question 5

Markers of cholestasis

Answer 5

1. 5’ nucleotidase
2. Alkaline phosphatase
3. GGT ( gamma glutamyl transpeptidase)
   4 times increase - interahepatic
   10 times increase - extrahepatic
   Low level - PFIC

Question 6

Mc indication of liver transplant in children

Answer 6

EHBA

Question 7

Definitive mgt of EHBA

Answer 7

Liver transplant

Question 8

Medical mgt

Answer 8

Medium chain Tg supplement
Vit ADEK
Ca phosphate & Zn
UDCA & cholestyramine