髓质海绵肾的临床诊断（附6例报告）

Clinical experience in diagnosis of medullary spongy kidney

关键词:  肾  髓质海绵肾  诊断

Key words:  kidney  medullary spongy kidney  diagnosis

摘要:

［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。

Abstract:

［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.