McGonigle R J, Mowat A P, Bewick M, Howard E R, Snowden S A, Parsons V
Q J Med. 1981 Summer;50(199):269-78.
Three patients with congenital hepatic fibrosis and childhood-type autosomal recessive polycystic kidney disease are reported. Portal hypertension in two of the children was decompressed surgically by lieno-renal shunting, and the renal failure in two children has been successfully treated with renal transplantation. Prophylactic porta-caval shunting followed by renal transplantation is ideally suited to the sequence of events occurring clinically in the intermediate form of this condition, preventing complications of bleeding from oesophageal varices and hyperplenism. The relationship of congenital hepatic fibrosis with the various forms of polycystic kidney disease is discussed and classified.
报告了3例先天性肝纤维化合并儿童型常染色体隐性遗传性多囊肾病患者。其中2例儿童的门静脉高压通过脾肾分流术进行了手术减压，2例儿童的肾衰竭已通过肾移植成功治疗。预防性门腔分流术随后进行肾移植非常适合这种疾病中间形式临床发生的事件顺序，可预防食管静脉曲张出血和脾功能亢进的并发症。讨论并分类了先天性肝纤维化与各种形式多囊肾病的关系。
