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Neonatal Cholestasis: an update

Gastroenterology & Hepatology: Open Access
Salahuddin Mahmud,1 Moinak Sen Sarma,2 Darma A,3 Syed Shafi Ahmed1


Any infant who is jaundiced beyond two to three weeks of life should be evaluated for neonatal cholestasis. Neonatal cholestasis is defined as accumulation of bile substances in blood due to impaired excretion. The most common causes of cholestatic jaundice in the first months of life are biliary atresia, idiopathic neonatal hepatitis, infections (Cytomegalovirus, herpes simplex toxoplasma, rubella, urinary tract infection, sepsis), endocrine (hypothyroidism), metabolic (Galactosemia, tyrosinemia, neonatal hemochromatosis), genetic (progressive familial intrahepatic cholestasis, Down syndrome, Alagille syndrome) along with many unknown or multifactorial (eg, parenteral nutrition-related) one. Conjugated hyperbilirubinemia, pale stools and dark urine are the cardinal features of neonatal cholestasis. Newborn screening for biliary atresia by using stool color cards is potentially life-saving and cost-effective. The differential diagnosis of cholestasis is extensive and a systematic approach is helpful to quickly establish the diagnosis. Early recognition, prompt evaluation, timely referrals to the pediatric gastroenterologist/hepatologist and algorithmbased management will improve outcome in neonatal cholestasis. Despite the unavailability of any specific treatments for some causes of neonatal cholestasis, the patient can benefit from nutritional management and early medical intervention.


neonatal cholestasis, preterm low birth weight, cystic fibrosis, magnetic resonance cholangiography, endoscopic retrograde cholangiography, liver transplantation