Table 1.
Underlying cause of cholestatic hepatobiliary disease
| Diagnosis | No of infants |
|---|---|
| Extrahepatic biliary atresia | 61 |
| Idiopathic neonatal hepatitis | 52 |
| Alagille syndrome | 17 |
| α1 Antitrypsin deficiency | 15 |
| Inherited metabolic disorders*: | |
| Galactosaemia | 5 |
| Tyrosinaemia | 1 |
| Mitochondrial cytopathy | 1 |
| Unspecified† | 3 |
| Inspissated bile syndrome | 7 |
| Idiopathic bile duct hypoplasia | 6 |
| Cytomegalovirus hepatitis | 5 |
| Fulminant hepatitis | 1 |
| Hypopituitarism | 2 |
| Sclerosing cholangitis | 1 |
| Total | 177 |
*
Excluding α1 antitrypsin deficiency.
†
Cholestatic jaundice from 1st week of life and hepatic steatosis on liver biopsy.