Arch Dis Child 1981 May;56(5):373-6

Long-term prognosis for infants with intrahepatic cholestasis and paten
extrahepatic biliary tract

Odievre M, Hadchouel M, Landrieu P, Alagille D, Eliot N

Abstract
One hundred and three infants with prolonged cholestasis beginning before 3
months were classified as having alpha-1-antitrypsin deficiency (17 patients),
scanty interlobular bile ducts (16 patients), or "neonatal hepatitis" (70
patients). Twenty-two gradually developed chronic liver disease and the
remaining 81 recovered within a few months. Prognosis was found to be poor for
infants with alpha-1-antitrypsin deficiency, scanty interlobular bile ducts, and
familial "idiopathic" hepatitis. Patients who developed cirrhosis often
presented with severe and persistent neonatal cholestasis, mimicking
extrahepatic biliary atresia and leading to laparotomy. Thus, a high-risk group
of infants-defined by aetiology, family history, and degree of cholestasis-can
be recognised in the first months of life