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A Clinical and histopathological Studies of congenital Extrahepatic Biliary Atresia.

Journal of the Korean Pediatric Society 1988;31(4):436-445.
Published online April 30, 1988.
A Clinical and histopathological Studies of congenital Extrahepatic Biliary Atresia.
Kweon Ha Son1, Ki Sup Chung1, Euh Ho Whang2, Chan Il Park3
1Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea
2Department of Pediatric Surgery, College of Medicine, Yonsei University, Seoul, Korea
3Department of Pathology, College of Medicine, Yonsei University, Seoul, Korea
선천성 간외 담도 폐쇄증에 대한 임상적 및 조직학적 연구
손권하1, 정기섭1, 황의호2, 박찬일3
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 외과학교실
3연세대학교 의과대학 병리학교실
Received: 24 August 1987   • Accepted: 10 November 1987
Abstract
A clinical assessment of the 30 patients with extrahepatic biliary atresia who were admitted to the Department of Pediatrics and pediatric Surgery, Yonsei University College of Medicine from January, 1979 to January, 1987 was performed. In 21 patients who underwent hepatic portoenterostomy, the relationship between outcome and surgery was analyzed. Finally, collelations between lonterm prognosis and the histopathology of biopsied liver tissue, postoperative bile restoration, and complica- tions were evaluated in 12 patients who were followed from one and half to 24 months. The results were as follows: 1) Among 30 patients (14 male and 16 female) of congenital extrahepatic biliary atresia, 16 (53%) were younger than 2 months of age and 6 (20%) were older than 3 months of age on admission. 2) Jaundice and an acholic stool were present in all 30 cases. Hepatomegaly was noted in 70% and splenomegaly in 53%. Ileal atresia, umbilical hernia and bronchogenic cyst were associated in each of 3 cases, respectively. 3) Twenty five cases (83%) had a total serum bilirubin level between 6 and 18 mg/dl, while only one case had that of less than 6 mg/dl. The direct bilirubin level was higher than 4 mg/dl in 25 cass (83 %). SGPT levels were less than 200IU/L in 26 cases (87%), and none exceeded 400IU/L. The alpha-fetoprotein level was increased in all studied cases with the mean level of 3.34 ±1.07 yg/dl. 4) HBsAg was noted in 2 of 25 cases, rubella virus infections in 2 of 21 cases, cytomegalovirus infections in 3 of 21 cases, and congenital syphilis in 2 of 24 cases. 5) Postoperative bile restoration was achieved in 15 (71%) of the 21 cases which underwent a hepatic portoenterostomy. There were no specific correlations between postoperative bile restoration and the age at operation, the types of extrahepatic biliary atresia, or the histologic changes of liver. 6) The degree of hepatic fibrosis was correlated with age. Seventy five percent of the patients younger than 2 months of age showed moderate to severe degree of hepatic fibrosis. 7) The incidences of postoperative ascending cholangitis and portal hypertension were.75% and 50 %, respectively. 8) Of the 12 cases followed up, 4 cases (33%) showed clinical improvement. Their mean age at the time of operation was 62 days, and 3 cases showed a mild degree of hepatic fibrosis and one a moderate degree. Ascending cholangitis developed in 2 cases, but portal hypertension did not develope. Of the 8 patients who died during the follow up period, 5 showed a moderate degree of heaptic fibrosis and 3 a severe degree. Ascending cholangitis had developed in 7 cases, and portal hypertension in all 8 cases. These results suggest that the younger the age at operation, the less hepatic fibrosis, and the absence of postoperative complications are correlated with a good prognosis.
Key Words: Congenital extrahepatic biliary atresia


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