Table 2.

Treatable causes of neonatal cholestasis

Disorder	Treatment
Infection (viral, bacterial, spirochete, parasite)	Antimicrobials
Galactosemia	Galactose-free diet
Tyrosinemia type 1	NTBC (2-(2- nitro-4-trifluoromethylbenzol)-1,3-cyclohexanedione), low tyrosine or phenylalanine diet
Hereditary fructose intolerance	Fructose or sucrose free diet
Hypothyroidism	Thyroid hormone replacement
Cystic fibrosis	Pancreatic enzymes
Hypopituitarism	Thyroid, growth hormone, cortisol replacement
Bile acid synthesis defects	Cholic acid or ursodeoxycholic acid supplementation
Biliary atresia	Hepatoportoenterostomy (Kasai procedure)
Choledochal cyst	Mucosectomy and Choledochoenterostomy
Spontaneous perforation of the common bile duct	Surgical drainage
Inspissated bile, common bile duct stone	Biliary tract irrigation
Parenteral nutrition associated cholestasis (intestinal failure associated cholestasis)	Intravenous lipid emulsion modification, advance enteral feedings

Adapted from Feldman AG, Sokol RJ, Nat Rev Gastroenterol Hepatol, 2019: Table 153