儿童髓质海绵肾的合并症分析及与预后关系探讨  

Complications and prognosis of medullary sponge kidney in children

作　　者：鲍春[1,2] 周建华[1] 王嘉[3] 

机构地区：[1]华中科技大学同济医学院附属同济医院儿科,武汉430030 [2]襄阳市中医医院儿科,襄阳441000 [3]武汉市儿童医院肾内科,武汉430015

出　　处：《临床肾脏病杂志》2013年第11期492-495,共4页Journal Of Clinical Nephrology

基　　金：国家自然科学基金资助（NO.81070557）

摘　　要：目的 髓质海绵肾（Medullary sponge kidney,MSK）多在成年发病,儿童期发病者罕见且病情多较严重,导致肾功能不全者较多,这可能与同时存在影响肾脏的一些合并症有关.本文分析儿童期发病的MSK的合并症发生情况并探讨合并症与预后的关系.方法 收集20例MSK患儿的临床资料,分析其合并症发生率并探讨合并症与预后的关系.结果 20例儿童MSK患儿,其中男13例,女7例,年龄10个月～13岁,平均年龄（7.95±11.49）岁.所有病例均有特征性多发性双肾结石表现,15例（占75%） 例并发尿路感染.此外,15例合并症中有14例为远端肾小管酸中毒（dRTA）,4例为胱氨酸尿症,1例为Carolis病,1例为肝豆状核变性.慢性肾功能不全的发生与结石关系不大,而与合并症有无以及泌尿道感染发生有关,差异有统计学意义（P〈0.05）.结论 儿童期发病的MSK合并症发生率高,对于儿童MSK,应积极治疗合并症,及时控制尿路感染,以减少慢性肾衰的发生.Objective Medullary sponge kidney（MSK） occur most often in adult, rate in children. Childhood-onset MSK usually present with more severe manifestation and often lead to renal insufficiency, which may be attributed to the high prevalence of superimposed metabolic disorders. The present paper is aimed to analyze the prevalence of complications in children with medullay sponge kidney and their impact on prognosis. Methods The clinical information was summarized and the prevalence of complications and their impact on prognosis were analyzed in 20 children with medullary sponge kidney. Results All cases showed multiple fine stones in both kidneys,fifteen cases out of 20 had urinary tract infection. In addition, 75% patients had complicationsincluding distal renal tubular acidosis （dRTA）, in 14 cases, cystinuria in 4 cases,Caroli disease in one case,and hepatolenticular degeneration in one case. Chronic renal insufficiency was significantly associated with complications and urinary tract infection but not with kidney stone formation. Conclusions High prevalence of complications and urinary tract infection was found in childhood-onset medullary sponge kidney, which should be actively treated in order to reduce the incidence of chronic renal failure caused by medullary sponge kidney.

关 键 词：儿童 髓质海绵肾 合并症 预后 

分 类 号：R692.12[医药卫生—泌尿科学]