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阵发性夜间血红蛋白尿患者的肾功能损害

Renal impairment in patients with paroxysmal nocturnal hemoglobinuria.

作者信息

Saito T, Furyama T, Onodera S, Saito H, Shioji R

出版信息

Tohoku J Exp Med. 1975 Jul;116(3):267-75. doi: 10.1620/tjem.116.267.

Abstract

Three patients with paroxysmal nocturnal hemoglobinuria accompanied by chronic renal lesions were studied. All the cases had histories of severe hemolytic anemia and repeated hemoglobinuria. The biopsy specimen of the kidney of two patients (Case 1 and Case 2) showed interstitial nephritis. Renal glucosuria, tubular proteinuria, increased urate clearance (Case 2) and reduced tubular reabsorption of phosphate (Case 3) were revealed in Case 2 and Case 3, suggesting renal tubular impairment. From the nephrological point of view, hemodynamic alteration resulting from intravascular hemolysis and severe persistent chronic anemia may primarily be responsible for the renal impairment.

摘要

对3例伴有慢性肾脏损害的阵发性夜间血红蛋白尿患者进行了研究。所有病例均有严重溶血性贫血和反复血红蛋白尿病史。2例患者(病例1和病例2)的肾脏活检标本显示为间质性肾炎。病例2和病例3出现肾性糖尿、肾小管蛋白尿、尿酸清除率增加(病例2)和肾小管对磷酸盐重吸收减少(病例3),提示肾小管损害。从肾脏病学角度来看,血管内溶血和严重持续性慢性贫血导致的血流动力学改变可能是肾脏损害的主要原因。

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