Abstract
髓质海绵肾是少见的肾脏先天性发育异常疾病,发病率约为1/20000～ 1/5000,男性多见.该病以肾小管扩张及发育不良所致的肾集合管囊状扩张为病变特点,因外观似海绵而得名,在1939年由Lenarduzzi首先报道.但其合并肾小管酸中毒和低钾性麻痹极为少见,现报道一例。
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