Infants with Cholestasis: Diagnosis, Management and Outcome

Objective: Infants with cholestatic jaundice were evaluated retrospectively in terms of etiologies, diagnostic methods, laboratory findings, treatment procedures and long- term prognosis.Patients and Methods: The study consisted of 70 children (52.8% male, 47.1% female) with cholestasis ranging in age from 15 days to 8 months (mean age, 60±26 days). Patients were divided into three groups according to the diagnosis: (i) patients with extrahepatic biliary atresia, (ii) patients with intrahepatic biliary hypoplasia, and (iii) patients with hepatocellular disease. Their clinical parameters were evaluated.Results: In the group with extrahepatic biliary atresia the onset of jaundice was significantly earlier and the presence of acholic stool and total bilirubin levels were remarkably higher than in the groups with intrahepatic biliary hypoplasia or hepatocellular disease. Serum gamma-glutamyl transpeptidase (GGT) and alkaline phosphotase (ALP) levels were found to be significantly higher in the groups with extrahepatic biliary atresia and intrahepatic biliary hypoplasia than the group with hepatocellular disease (p<0.001 and p<0.01, respectively). The contribution of technetium-99m (99mTc) scintigraphy to the diagnosis was significantly higher in the group with extrahepatic biliary atresia than the groups with intrahepatic biliary hypoplasia and hepatocellular disease (p<0.002).Conclusion: It was found that cholestasis, acholic stool and elevated GGT are better markers for extrahepatic biliary atresia than for intrahepatic biliary hypoplasia or hepatocellular disease in infants. The contribution of scintigraphy to the diagnosis was found to be higher in the group with extrahepatic biliary atresia than in the other groups. (Marmara Medical Journal 2012;25:83-6)