| Portal Hypertension in Children |
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Young Sook Kim, Ki Sup Chung |
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Department of Pediatrics, Yonsei University, College of Medicine, Seoul, Korea |
| 소아의 문맥압 항진증에 대한 임상적 연구 |
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김영숙, 정기섭 |
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연세대학교 의과대학 소아과학교실 |
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| Abstract |
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Portal hypertension assumes special interest in children due to the large variety of causes, the apparent difficulty of performing surgical portal systemic shunts because of the small size of vessels, the doubt still cast on the long term tolerance of portal systemic shunts and the theoretical possibility of spontaneous regression of varices with growth. Thirty cases of portal hypertension under 15 years of age who had been admitted to the Departmem of pediatrics, Yonsei University College of Medicine for 10 years between January, 1981 to June, 1991, were clinically studied and analyzed.
The results were as follows:
1) Sixteen among 30 children with portal hypertension were'female, with the sex ratio being 1.1:1. And twelve (40%) among 30 children occurred under 1 year of age.
2) Of 30 cases who were analyzed pathologically, 6 cases (20%) were prehpatic, 23 cases (77%) were intrahepatic, and 1 case (3%) was posthepatic being Budd-Chiari syndrome. Of 6 cases with prehepatic causes, 5 cases were portal vein obstruction and 1 case was splenic vein obstruction. Twenty three cases with intrahepatic pathology were found to be liver cirrhosis due to biliary atresia, and idiopathic neonatal hepatitis.
3) Of 12 cases under 1 year of age, 6 cases were liver cirrhosis due to biliary atresia, 3 cases liver cirrhosis due to neonatal hepatitis, and 2 cases congenital hepatic fibrosis. Of 3 cases between 2 and 5 year of age, 2 cases were portal vein obstruction. Of 7 cases between 6 and 10 year of age, 2 cases were idiopathic portal hypertension. Of 8 cases above 11 year of age, 3 cases were portal vein obstruction, and 2 cases Wilson뭩 disease.
4) Clinical manifestations on admission were splenomegaly (85%), hepatomegaly (50%), melena (47%), hematemesis (40%), ascites (30%), and jaundice (27%).
5) Of 14 cases with gastrointestinal bleeding, prehepatic causes were 3 (50%) among 6 cases, and intrahepatic causes were 11 (50%) among 23 cases.
6) Laboratory findings on admission were anemia (47%), neutropenia (80%), thrombopenia (50%), hyperbilirubinemia (30%), hypoalbuminemia (43%), prothrombin time prolongation (30%), and elevated SGPT (63%).
7) Diagnostic methods were esophagogram (73%), abdominal ultrasonogram (63%), endoscopy (40%), liver biopsy (30%), and angiogram (23%).
8) Of 30 cases, 10 cases (33%) atively including 1 case treated with endoscopic sclerotherapy.
9) Of 23 cases that were followed, 11 cases (48%) expired. Of 15 cases who were managed conservatively, 9 cases (39%) expired. And of 8 cases who had surgery, 2 cases (9%) expired. Mortality according to disease, liver cirrhosis due to biliary atresia is 83%, congenital hepatic fibrosis 50%, liver cirrhosis due to idiopathic neonatal hepatitis 33%, and portal vein obstruction 20%. |
| Key Words:
Portal Hypertension, Children |
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