髓质海绵肾及其并发症的认识

Understanding of Medullary Sponge Kidney and Its Complications

髓质海绵肾(MSK)是一种先天性肾脏发育异常性疾病,病理以肾锥体部的乳头管呈梭形扩张和肾髓质集合管或小囊状扩张为主要表现,又称为肾锥体囊肿病或先天性肾集合管囊性扩张。近年来,陆续出现MSK病例的相关报道,对其发病机制的研究也逐渐深入,但确切病因尚不明确,多认为属于先天性疾病,目前尚无确切的根治方法,只能预防和治疗MSK的相关并发症。通过对MSK发病机制、临床表现、诊断、治疗和预后的深入认识,以掌握该疾病的诊治思路和延缓疾病进展。

Medullary sponge kidney(MSK)is a congenital renal developmental abnormality disease,with shuttle-shaped expansion of papillary duct in pyramidal part of kidney and the renal medullary collecting duct or small cystic expansion as the main pathological manifestations,also known as renal pyramidal cyst disease or congenital renal collecting cystic dilatation.In recent years,the cases of MSK have been reported one after another,and the research on its pathogenesis has also gradually deepened,but the exact cause is not clear with the main opinion of congenital disease,and now there is no radical method,but only preventive and therapeutic measures for the related complications of MSK.Here is to make a review of the in-depth understanding of the pathogenesis,clinical manifestations,diagnosis,treatment and prognosis of MSK,to master the progress of the diagnosis and treatment to delay the disease progression.