髓质海绵肾的临床诊断(附6例报告)

目的]总结髓质海绵肾（MSK）的临床特点，加深对MSK的认识并提高其诊断的准确性。方法]对大连医科大学第一临床学院肾内科收治的6例MSK患者的临床表现及辅助检查结果进行回顾分析。结果]6例MSK患者中，男4例，女2例，均表现有肾小管功能的损害，尤其表现为远端肾小管浓缩功能的障碍。其中2例存在尿酸化功能不良。3例出现不同程度的肾小球滤过功能异常，1例经肝活检确诊合并先天性肝纤维化。影像学检查结果各有不同。1例肾活检病理显示集合管扩张，上皮变扁、变性，可见灶状钙盐沉着，周围纤维化明显。结论]MSK临床表现多变，诊断时不仅要想到本病，也要尽量完善各种影像学检查，在诊断不清的情况下可考虑肾活检。

Clinical experience in diagnosis of medullary spongy kidney

Objective] To summary the clinical features of Medullary Sponge Kidney(MSK),get a clearer recognition and improve the accuracy of diagnosis of MSK.Methods] The clinical findings,imaging examinations and pathological results of 6 cases with MSK admitted into our hospital were analyzed retrospectively.Results] All the 6 cases,4 male and 2 female,presented with tubular dysfunction,mainly in distal renal tubule impairment.Two of six cases were accompanied with impaired urine acidification and 3 cases were with decreased Glomerular Filtration Rate(GFR).One case was accompanied with Congenital Hepatic Fibrosis(CHF) confirmed by hepatic biopsy.There were different results in B-mode US,CT scan and IVP.Renal biopsy of one case showed dilatation of collecting duct,degeneration of epithelium and focal deposition of calcium.Conclusions] The clinical manifestations of MSK are variable.It is necessary to combine all the imaging tests in diagnosing of MSK.Renal biopsy is final way to estabolish diagnosis when it is suspected.