소아의 간경변증에 대한 임상적 연구

Abstract

Purpose : Liver cirrhosis is relatively uncommon disease in childhood, but the overall prevalence rate and etiology of the liver cirrhosis in children were uncertain in Korea. This clinical study was undertaken to help pediatricians manage the patients properly by investigating the etiology, clinical findings and the outcome on 40 pediatric patients with liver cirrhosis. Methods : This study was conducted on 40 patients with liver cirrhosis who were admitted to Department of Pediatrics, Yonsei University College Medicine from April, 1985 to June, 1993 using various diagnostic methods including liver function tests, various hematologic or serologic tests, hepatitis viral markers, esophagogram, ultraso¡ⓒ nogram, DISIDA SCAN, endoscopy, percutaneous liver biopsy with pathologic examinations. Results : 1) The age of 40 patients ranged from 2 month to 15 years and male to female ratio was 1.1:1. 2) The most common underlying cause was extrahepatic biliary atresia with 14 cases(35%), and others included neonatal hepatitis in 13(33%), Wilson disease in 7(18%), chronic active hepatitis B in 2(5%), and autoimmune chronic active hepatitis, inspissated bile syndrome, congenital hepatic fibrosis, choledochal cyst in 1 case(3%) respectively. 3) Neonatal cholestasis was the main cause up to 5 years of age. But Wilson disease, chronic active hepatitis B, autoimmune chronic active hepatitis were main causes over 5 years of age. 4) The most common clinical finding was jaundice with 33 cases(83%), and others included splenomegaly in 24, hepatomegaly in 19, ascites in 18, acholic stool in 11, and melena, fatigue, vomiting and diarrhea in 4 cases respectively. 5) Laboratry findings included anemia in 13, leukopenia in 3, thrombocytopenia in 11, hyperbilirubinemia with total bilirubin above 1O.0mg/dl in 18, hypoalbuminemia in 11, prolonged prothrombin time in 21, elevated AL T and AST in 35 cases. 6) The most common complication was portal hypertension with 24 cases(60%), and others included ascites in 18(45%), recurrent infection in 17(42.5%), hypersplenism in 16(40%), hepatic encephalopathy in 11(28%), esophageal varices in 10(25%), hepatorenal syndrome in 1 case(2.5%). 7) The patients in this study were clssified as class A in 5, B in 13, C in 22 by modified Child-Turcotte-Pugh classificaton. Twenty six of 40 casess were able to follow up and the mortality rate were 0% in class A, 23% in B, 27% in C. The overall mortality was 22.5%. Conclusion : These results suggest that the main underlying cause of liver cirr¡ⓒ hosis in infants and preschool age children was neonatal cholestasis, but main causes inschool age children were Wilson disease, chronic active hepatitis B and autoimmune chronic active hepatitis. It is considered that continuing¡¤ research works should be needed to find the epidemiologic and clinical features of liver cirrhosis in children in Korea.