Ramírez Mayans J A, Mata Rivera N, Mora Tiscareño M A, Cervantes Bustamante R, Vargas Gómez M A, Aguinaga V, Rocío G
Servicio de Gastroenterología, Instituto Nacional de Pediatría, Distrito Federal, México.
Acta Gastroenterol Latinoam. 1994;25(5):297-303.
We studied 26 children with congenital hepatic fibrosis during the period 1971-1993. About half of the children were about the 6 years old. Only two had brothers with same disease. The chief clinical manifestation was hematemesis associated or not with liver enlargement, predominantly of left lobe. Only one case showed fever and cholangitis. Liver function tests were usually normal. Twenty-two children had portal hypertension. Liver biopsy was of definitive for diagnosis. Seven children died.
我们在1971年至1993年期间研究了26例先天性肝纤维化患儿。约一半患儿年龄在6岁左右。仅有两例患儿的兄弟患有相同疾病。主要临床表现为呕血，可伴有或不伴有肝脏肿大，以左叶为主。仅1例出现发热和胆管炎。肝功能检查通常正常。22例患儿有门静脉高压。肝活检对诊断具有决定性意义。7例患儿死亡。
