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目的通过复习文献、结合病例总结儿童髓质海绵肾的临床特点,提高对本病的认识,以达早期诊断和治疗,改善预后。方法对苏州大学附属儿童医院2005年1月至2010年12月收治的11例儿童髓质海绵肾患儿的临床资料进行回顾分析。结果 11例患儿中男3例,女8例。年龄48d至17岁,其中<1岁6例,>3岁5例。临床表现无特异性,均由影像学确诊为双侧髓质海绵肾。表现反复尿路感染5例、远端肾小管酸中毒7例、肾结石1例、高血压2例、发育迟缓6例、肾衰竭2例、肾上腺皮质功能亢进1例。结论髓质海绵肾儿童期发病临床症状无特异性,表现形式多样化,早期诊断依靠辅助检查,影像学检查B超、CT可作首选,存在合并症时合理治疗可改善预后。

Clinical data analysis of 11 cases of medullary sponge kidney in children

Objective To achieve early diagnosis and treatment as well as to improve prognosis through literature review and clinical features of medullary sponge kidney in children.Methods Eleven children with medullary sponge kidney admitted to the Affiliated Children ’ s Hospital from Jan 2005 to Dec 2010 were retrospectively analyzed.Results Among the 11 cases,there were 3 male and 8 female,agied from 48 days to 17 years(6 were within 1 year old and 5 were above 3 years old).None of these cases were clinically specific,and all were image-diagnosed to be bilateral medullary sponge kidney.The clinical demonstrations included:recurrent urinary tract infection(5 cases),distal renal tubular acidosis(7 cases),kidney stones(1 case),hypertension(2 cases),malnutrition and growth retardation(6 cases),renal failure(2 cases),and adrenal cortical hyperfunction(1case).Conclusion Medullary sponge kidney in early childhood is not clini cal specific but with diverse clinical manifestations.The early diagnosis could be based on laboratory examinations;ab dominal ultrasound and CT can be used as first choice.In the occasion of complications,a reasonable treatment can im prove the prognosis.