儿童髓质海绵肾11例临床分析

目的分析儿童期发病的髓质海绵肾（MSK）的临床特点。方法回顾性分析1990年1月至2011年4月华中科技大学同济医学院附属同济医院儿科影像学诊断的MSK患儿的临床表现、实验室检查、治疗和随访等资料。结果 11例MSK患儿进入分析,男9例,女2例,年龄3个月至11岁,平均年龄4.54岁,〉3岁6例。①以泌尿系统症状（多尿、尿频、尿急、尿痛、排尿不畅、水肿）就诊5例,以泌尿系统外症状（发热、呕吐、生长发育落后、腹泻、咳嗽）就诊6例。尿蛋白阳性9例,镜下血尿8例,白细胞尿7例。合并远端肾小管酸中毒者9例,泌尿系统感染6例,双肾多发性结石7例,肾性贫血4例,双肾多发性钙化3例,Caroli＇s病1例,生长发育落后5例。②5/9例腹部X线片显示双肾髓质区可见较对称、呈扇状钙质影沿肾乳头分布,大小略显不等;10/11例腹部B超检查示双肾髓质回声增强,其中7例显示双肾内有多个强回声团,后方伴声影,肾积水2例,且均为右肾;4例肾盂静脉造影检查均显示肾锥体内集合管扩张,呈扇形、条纹状或葡萄串状改变,受损肾组织达1～3个椎体,双侧呈对称性病理改变;2例CT检查均可见双肾多发散在小结石成簇状排列。③11例入院后均予纠酸、补钾、抗感染和排石等对症治疗,出院后予长期口服枸橼酸钠钾。1例失访,10例随访（5.2±1.9）年,6例发展为肾功能不全,2例身高仍明显落后于同龄儿童。结论儿童期发病的MSK可以泌尿系统外症状起病,且可合并其他肾内肾外疾病。诊断主要依据影像学检查,积极治疗有可能延缓肾功能逐步恶化。

Clinical analysis of 11 cases with medullary sponge kidney in children

Objective To analyze the characteristics of 11 cases with medullary sponge kidney（MSK） in childhood.Methods The clinical information was summarized in 11 children with MSK who were hospitalized in Tongji Hospital from January 1990 to April 2011.Results There were 9 boys and 2 girls in 11 cases aged from 3 months to 11 years,with 5 cases younger than 3 years old and 6 cases elder than 3 years old.Five cases were hospitalized for renal symptoms like urorrhagia,frequent micturition,urgent urination and odynuria.The other 6 cases were hospitalized for extrarenal symptoms like fever,vomiting,growth retardation,diarrhea and cough.Diagnosis of MSK in these children was taken based on renal ultrasonography,intravenous pyelography or computed tomography.Most patients were accompanied by other renal diseases or extrarenal diseases,such as distal renal tubular acidosis in 9 cases,renal anemia in 4 cases,renal calcification in 3 cases,Carolis’s syndrome in 1 case,malnutrition and growth retardation in 5 cases,cystinuria in 2 cases,generalized aminoaciduria in 2 cases and vitamin D-resistant rickets in 2 cases.Acute renal failure was found in 1 case and chronic renal failure in 3 cases.The patients all had bilateral pathological changes according to the result of abdominal plain films,renal ultrasonography,intravenous pyelography or computed tomography.IVP showed lesions in kidneys reaching one to three centra,and bilateral lesions were symmetrical.Conclusions MSK was mainly found in adults,rarely in childhood.Childhood onset of MSK was often due to the accompany of renal diseases or extrarenal diseases,which led to prominent calcification or calculus of kidney and resulted in early occurance of symptoms or signs.Therefore,renal ultrasonography,intravenous pyelography or computed tomography should be performed in children manifesting multiple renal calculi,recurrent urinary tract infection and renal failure in order to find MSK at early stage.