Tamura H, Kato H, Hirose S, Itoyama S, Matsumura O, Nagasawa R, Mitarai T, Isoda K
Fourth Department of Internal Medicine, Saitama Medical Center, Saitama Medical School, Japan.
Nihon Jinzo Gakkai Shi. 1994 Aug;36(8):962-7.
Congenital hepatic fibrosis is often associated with infantile, but not with adult polycystic kidney disease. We report the unusual case of an adult patient with polycystic kidney disease complicated by congenital hepatic fibrosis. A 27-year-old women was admitted to our hospital because of gross hematuria due to hemorrhage from renal cysts. She presented hematemesis from ruptured esophageal varices at the age of 14 years. She was diagnosed as having end-stage renal disease due to polycystic kidney disease at the age of 23 years, and maintenance hemodialysis was initiated the following year. Gross hematuria was managed with supportive therapy. However, the patient developed cholangitis and died of sepsis. Postmortem examinations as well as the patient's clinical course suggested that she had an autosomal dominant type of polycystic kidney disease. Histological findings of the liver were compatible with congenital hepatic fibrosis.
先天性肝纤维化常与婴儿型多囊肾病相关，但与成人型多囊肾病无关。我们报告了一例成年多囊肾病患者合并先天性肝纤维化的罕见病例。一名27岁女性因肾囊肿出血导致肉眼血尿入院。她14岁时因食管静脉曲张破裂出现呕血。23岁时被诊断为多囊肾病导致的终末期肾病，次年开始维持性血液透析。肉眼血尿采用支持治疗。然而，患者发生胆管炎并死于败血症。尸检及患者临床病程提示她患的是常染色体显性型多囊肾病。肝脏的组织学检查结果与先天性肝纤维化相符。
