Conjugated Hyperbilirubinaemia

Question 1

What are causes of conjugated hyperbilirubinaemia in infants?

Answer 1

Extrahepatic:

• biliary atresia
• choledochal cyst
• mass (gallstone/neoplasia)
• spontaneous perforation of common bile duct
• inspissated bile syndrome
• neonatal sclerosing cholangitis

Intrahepatic

• idiopathic
• idiopathic neonatal hepatitis
• recurrent intrahepatic cholestasis
• alagille syndrome

Anatomical

• congenital hepatic fibrosis
• caroli’s disease

Storage and metabolic disorders

• cystic fibrosis
• bile acid synthesis disorder
• lipid storage disorders
• infantile copper overload
• a1 antitrypsin deficiency
• carbohydrate disorders (galactosaemia)
• amino acid disorders (tyrosinaemia)

Endocrine

• hypothyroidism
• hypopituitarism

Infection

• viral: CMV, HSV, HIV, hepatitis B, rubella
• bacterial: syphilis, sepsis, UTI (E. coli)
• protozoal: toxoplasma gondii
• other: adenovirus, Epstein-Barr virus

Toxins

• drug induced
• fetal alcohol syndrome
• parenteral nutrition-associated cholestasis

Chromosomal

• trisomy 21, 13, 18
• turner’s syndrome

Miscellaneous

• shock/hypoperfusion
• haemophagocytic lymphohistiocytosis
• veno-occlusive disease