[Neonatal cholestatic icterus simulating atresia of the bile ducts in a patient with alpha-1-antitrypsin deficiency].

: A 40 days old infant with cholestasis is described. The liver was enlarged at 3 cm below the costal margin. No bile ducts were seen at the liver scan (IDA Tc 99) neither bile was collected after cholecystokinine IV administration. Fibrosi, bile ducts proliferation, and cholestasis without intracellular PAS positive material were seen at liver biopsy. Serum alpha-1-antitrypsin level was 42 mg/100 ml. Follow-up was satisfactory after phenobarbital and cholestiramine treatment. Cholestasis decreased and two weeks later bile excretion was obtained after cholecystokinine administration. This stress the importance of alpha-1-antitrypsin determination in cholestasis in infancy.