Retrospective analysis of children with neonatal cholestasis Original Article

Öz

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 cytomegalovırus 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

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Kliniğimizde izlenen neonatal kolestazlı olguların retrospektif değerlendirmesi Orijinal Araştırma

Öz

Gastroenteroloji polikliniğimizden 1996 2000 yılları arasında izlediğimiz 53 neonatal kolestaz olgusunu retrospektif olarak değerlendirdik Elli üç olgunun 33’ü erkek 62 3 20’si kız 37 7 olup yaş ortalaması 7 9 aydı Kırk yedi olguda 88 7 intrahepatik 6 olguda 11 3 ekstrahepatik kolestaz tespit edilmiştir Hastaların 23’ünde 43 4 idyopatik neonatal hepatit 10’unda 18 9 sitomegalovirüs hepatiti 4’ünde 7 5 Byler hastalığı 2’sinde 3 8 hipotiroidi 2’sinde 3 8 galaktozemi 1’inde 1 9 tirozinemi 1’inde 1 9 rubella hepatiti 1’inde 1 9 Alagille Sendromu 1’inde 1 9 nonsendromik safra yolları azlığı sendromu 1’inde 1 9 alfa 1 antitripsin eksikliği 1’inde 1 9 Niemann Pick hastalığı 6’sında 11 3 safra yolları atrezisi saptanmıştır Elli üç olgunun 14’ü 26 4 karaciğer yetmezliği ile eksitus olmuş on altı olguda 30 2 tam iyileşme gözlenmiştir Yirmi üç olgu 43 4 halen poliklinik takibindedir Safra yolları atrezisi olan 6 olgudan 4’üne Kasai operasyonu yapılmış ve sağ kalım oranı 33 3 olarak tespit edilmiştir Sarılığı 14 günden uzun süren yenidoğanlarda neonatal kolestazdan şüphe edilmeli akolik dışkı varlığı sorgulanmalıdır Erken tanı ve tedavi özellikle safra yolları atrezisi olan olgular için hayati önem taşır Anahtar Kelimeler: sarılık kolestaz safra yolları atrezisi

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