Table 2 Differential diagnosis of neonatal cholestasis

Viral: Cytomegalovirus

α1-antitrypsin deficiency

 Rubella

Galactosemia

 Reovirus3

Glycogen storage disorder type IV

 Adenovirus

Fructosemia

 Echovirus

Cystic fibrosis

 Coxsackie virus

Hemochromatosis

 Human herpes virus 6

Tyrosinemia

 Varicella zoster

Arginase deficiency

 Herpes simplex

Zellweger’s syndrome

 Parvovirus

Dubin-Johnson syndrome

 Hepatitis B and C

Rotor syndrome

 Human immuno-deficiency virus

Hereditary fructosemia

Bacterial: sepsis

Niemann Pick disease, type C

 Urinary tract infection

Gaucher’s disease

 Syphilis

Wolman’s disease

 Listeriosis

Bile acid synthetic disorders

 Tuberculosis

Progressive familial intrahepatic cholestasis

Parasitic: Toxoplasmosis

North American Indian familial cholestasis

 Malaria

Aagenaes syndrome

X-linked adreno-leukodystrophy

Chromosomal disorders

Vascular disorders

Turner’s syndrome

Budd-Chiari syndrome

Trisomy 18

Neonatal asphyxia

Trisomy 21

Multiple haemangiomata

Trisomy 13

Congestive heart failure

Cat-eye syndrome

Donahue’s syndrome (Leprechauns)

Bile duct anomalies

Neoplastic disorders

Biliary atresia

Neonatal leukemia

Choledochal cyst

Histiocytosis X

Alagille syndrome

Neuroblastoma

Non-syndromic bile duct paucity

Hepatoblastoma

Inspissated bile syndrome

Erythrophagocytic lymphohistiocytosis

Caroli syndrome

Choledocholithiasis

Gall-stones

Neonatal sclerosing cholangitis

Spontaneous common bile duct perforation

Toxicity

Miscellaneous

Parenteral nutrition

Neonatal lupus erythematosus

Fetal alcohol syndrome

’Le foie vide’ (infantile hepatic non-regenerative disorder)

Drugs

Indian childhood cirrosi

ARC syndrome (Arthrogryposis, renal tubular dysfunction and cholestasis)