Kliniğimizde izlenen neonatal kolestazlı olguların retrospektif değerlendirmesi Orijinal Araştırma

Abstract

We evaluated retrospectively 53 patients with neonatal cholestasis followed by the gastroenterology department between 1996 2000 Thirty three patients 62 3 were males 20 37 7 were females and the mean age of the whole group was 7 9 months Intrahepatic cholestasis was found in 47 patients 88 7 while 6 patients 11 3 had extrahepatic cholestasis The etiology of 53 patients was as follows: twenty three 43 4 had idiopathic neonatal hepatitis 10 18 9 had cytomegalovirus hepatitis 4 7 5 had Byler disease 2 3 8 had galactosemia 1 1 9 had tyrosinemia 1 1 9 had rubella hepatitis 1 1 9 had Alagille Syndrome 1 1 9 had nonsyndromic bile ducts paucity syndrome 1 1 9 had alpha 1 antitrypsin deficiency 1 1 9 had Niemann Pick disease 6 11 3 had extrahepatic biliary atresia Fourteen of 53 patients 26 4 died because of hepatic failure Sixteen patients 30 2 recovered completely Twenty three patients 43 4 are still being followed by the gastroenterology department Four of six patients with extrahepatic biliary atresia underwent Kasai operation; the survival rate was 33 3 Neonatal cholestasis should be considered in neonates with persisting hyperbilirubinemia longer than 14 days and the existence of acholic stools should be questioned carefully Early diagnosis and therapy is essential especially for the survival of patients with biliary atresia Key words: jaundice cholestasis biliary atresia