CONGENITAL HEPATIC-FIBROSIS IN CHILDREN
CONGENITAL HEPATIC-FIBROSIS IN CHILDREN
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DOI：
10.1016/s0022-3476(81)80320-4
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发表时间：
1981-01-01
影响因子：
5.1
通讯作者：
ALAGILLE, D
中科院分区：
文献类型：
--
作者：
ALVAREZ, F;BERNARD, O;ALAGILLE, D
27例先天性肝纤维化[一种隐性常染色体疾病]患儿随访3个月至12年。大多数患儿肝脾肿大、肝功能检查正常、肾脏异常，提示通过简单的临床、生物学和放射学标准可以正确诊断先天性肝纤维化。21例食管内窥镜显示静脉曲张。16名儿童接受了门静脉-全身分流手术。随访检查未发现任何肝功能损害或肝性脑病的迹象。胆管炎仅在3例患儿中出现。
Twenty-seven children with congenital hepatic fibrosis [a recessive autosomal disease] were followed for 3 mo.-12 yr. Hepatosplenomegaly, normal liver function tests and kidney abnormalities were present in most patients, indicating that a correct diagnosis of congenital hepatic fibrosis could be made using simple clinical, biologic and radiologic criteria. Esophageal endoscopy showed varices in 21 patients. Sixteen children underwent portal-systemic shunt surgery. Follow-up examinations did not show any impairment of liver function or any sign of hepatic encephalopathy. Cholangitis was present in only 3 children.