Cholestatic syndromes of infancy

This study aimed to provide the analysis of clinical presentation, results of laboratory and imaging investigations as well as clinical outcome of children with cholestasis. Infants with neonatal cholestasis referred to Children's Hospital from 2002 to 2007 were participated in the study in a cross-sectional prospective study. Appropriate diagnostic criteria and tests were employed for diagnosis the underlying etiologies of neonatal cholestasis. One year mortality rate was determined. One hundred twenty one infants, 75 males and 46 females, with the mean age of 58.3 +/- 15.3 (14-120) days were enrolled in study. Jaundice (94.2%) and hepatomegaly (66.1%) were the most frequent symptom and signs on admission. Idiopathic neonatal hepatitis (36.4%), extrahepatic biliary atresia (24.8%), metabolic disease (20.7%), intrahepatic ductal paucity (10.7%), intrauterine infection (3.3%) were the most frequent causes of neonatal cholestasis. One year mortality was 5.8%. There is still not one effective and specific diagnostic method in differentiating between the causes of cholestasis in the newborns and infants. Some potentially important differences in the disease pattern, initial presentation and long-term outcome are suggested from the present study when compared to previous reports from other parts of the world.