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Korean J Pediatr Gastroenterol Nutr. 1999 Mar;2(1):46-58. Korean.
Published online Mar 31, 1999.  https://doi.org/10.5223/kjpgn.1999.2.1.46
Copyright © 1999 The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition
Evaluation of the Underlying Etiology and Long-Term Prognostic Factors in Neonatal Cholestasis
Kyung Mo Kim and Jeong Kee Seo
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.
Abstract

PURPOSE

The aim of the present study was to evaluate the long-term clinical profile including the underlying etioligy and the prognostic factors of the neonatal cholestasis.

METHODS

We studied the 190 infants presented with neonatal cholestasis for the last 12 years (from 1981 to 1992). The underlying causes, clinical findings and long-term outcomes were evaluated. And the prognostic factors were also analyzed.

RESULTS

Underlying disease were neonatal hepatitis in 101 (idiopathic in 77 and infectious in 24), intrahepatic bile duct paucity in 5, biliary atresia in 79, choledochal cyst in 5. Metabolic disease was not observed in this study. The important clinical problems during follow-up were persistent high fever, gastrointestinal bleeding, hepatic encephalopathy and ascites. The main causes of the death were hepatic encephalopathy and gastrointestinal bleeding. While three fourth of infants with idiopathic and infectious neonatal hepatitis recovered usually within a year, five-year survival rate for biliary atresia was just 40%, the mortality observed usually within the first year after Kasai operation and prognostic factor was the time of operation. Underlying disease was the most important prognostic factor of neonatal cholestasis.

CONCLUSION

This study showed that most common causes of neonatal cholestasis were biliary atresia and idiopathic neonatal hepatitis, infectious neonatal hepatitis, choledochal cyst and Alagille syndrome, but few neonatal cholestasis of genetic or metabolic liver disease was observed. The most important long-term prognostic factor of neonatal cholestasis was the underlying disease.

Keywords: Neonatal cholestasis; Neonatal hepatitis; Biliary atresia; Prognosis; Prognostic factor