title: [Congenital hepatic fibrosis and polycystic disease of the kidneys in two siblings (author's transl)].
abstract: Report of 2 siblings, aged 12 1/2 and 9 years, with congenital hepatic fibrosis and polycystic disease of the kidneys. Hepatosplenomegaly had been noted in both children at birth. The younger child had suffered from oliguria aged 2 1/2 years. At diagnosis both children had low platelet counts, one also had leucopenia. The cystic disease of the kidneys was verified by angiography. Coeliacography and splenopartography were diagnostically irrelevant. The diagnosis only became apparent from liver biopsy which was performed during splenectomy. After splenectomy there was an increase of platelets, white blood cells and the clotting factors II, V and X. The three years follow-up showed a constancy of renal impairment and of the minor oesophageal varices observed in the one patient who did not have a spontaneous spleno-renal anastomosis. So far no bleeding has been observed. Porto caval anastomosis was omitted in both children. Pros and cons are being discussed.
mesh_terms: D002648:Child; D004932:Esophageal and Gastric Varices / Q000150:complications; D005260:Female; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D008103:Liver Cirrhosis / Q000151:congenital / Q000000981:diagnostic imaging / Q000235:genetics*; D007690:Polycystic Kidney Diseases / Q000151:congenital / Q000000981:diagnostic imaging / Q000235:genetics*; D011859:Radiography; D013156:Splenectomy; D013163:Splenomegaly / Q000209:etiology
vernacular_title: Congenitale Leberfibrose und Zystennieren bei zwei Geschwistern.