作者: Jan M. Cobben , Martijn H. Breuning , Coen Schoots , Leo P. ten Kate , Klaus Zerres
DOI: 10.1038/KI.1990.286
关键词: Pathology 、 Chromosome 16 、 Congenital hepatic fibrosis 、 Autosomal dominant polycystic kidney disease 、 Polycystic kidney disease 、 Liver disease 、 Fibrosis 、 Disease 、 Medicine 、 Biopsy 、 Nephrology
摘要: Congenital hepatic fibrosis in autosomal-dominant polycystic kidney disease. was found four families with is commonly thought to be characteristic for autosomal-recessive disease, but the reported show that it can also complicate In three close linkage between mutation causing disease and DNA markers on chromosome 16 demonstrated. The clinical course of congenital differed considerably; one family children died soon after birth, other an approximately 20 years follow-up showed no detectable progression liver