Tazelaar H D, Payne J A, Patel N S
Gastroenterology. 1984 Apr;86(4):757-60.
Congenital hepatic fibrosis has been associated with a variety of renal malformations, but rarely adult-type polycystic kidneys. The case of a 19-yr-old woman with congenital hepatic fibrosis associated with asymptomatic familial adult-type (autosomal dominant) polycystic kidney disease is described. A literature review revealed seven other reports of this association. Our patient differs because the association between congenital hepatic fibrosis and adult-type polycystic kidney disease is clear, and because her asymptomatic kidney disease accords with the later onset of symptomatic polycystic kidney disease in her family.
先天性肝纤维化与多种肾脏畸形有关，但很少与成人型多囊肾相关。本文描述了一名19岁女性，患有先天性肝纤维化并伴有无症状的家族性成人型（常染色体显性）多囊肾病。文献回顾显示还有其他7篇关于这种关联的报道。我们的患者有所不同，因为先天性肝纤维化与成人型多囊肾病之间的关联明确，且她的无症状肾病与家族中症状性多囊肾病的较晚发病情况相符。
