Ram R, Adiraju K P, Gudithi S, Dakshinamurty K V
Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India.
Department of General Medicine, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
Indian J Nephrol. 2017 Jul-Aug;27(4):289-293. doi: 10.4103/0971-4065.205201.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired chronic disorder characterized by a triad of clinical features - hemolytic anemia, pancytopenia, and thrombosis. Not many reports of renal involvement in PNH are available in literature. We present a case series of PNH with renal involvement. We present the data of PNH patients who attended to Departments of General Medicine and Nephrology at a government-run tertiary care institute in South India. The diagnosis of PNH in these patients during initial phase, between 1998 and 2004 was based on sucrose lysis and Ham's test. After 2004, the diagnosis was based on flow cytometry to detect CD59 (membrane inhibitor of reactive lysis), a glycoprotein, and CD55 (decay accelerating factor) in regulation of complement action. The patient data were collected from 1998 to 2014. There were 14 patients of PNH in this period. The mean age was 37 years and the range was 16-68 years. There were eight females. Acute kidney injury (AKI) was noted in six patients. Dialysis was performed in four of them. The mean serum creatinine and urea at the initiation of dialysis were 5.4 ± 0.6 and 64.1 ± 6.1 mg/dl, respectively. The median number of hemodialysis sessions done was four. Renal biopsy was done in four patients. In three patients, the urinalysis and serum chemistry were suggestive of Fanconi syndrome. In our patients, three renal manifestations of PNH were identified. They were AKI, renal vessel thrombosis, and Fanconi syndrome. Chronic renal failure was not identified.
阵发性睡眠性血红蛋白尿(PNH)是一种获得性慢性疾病,其临床特征为三联征——溶血性贫血、全血细胞减少和血栓形成。关于PNH肾脏受累的文献报道并不多。我们报告一组伴有肾脏受累的PNH病例系列。我们展示了在印度南部一家政府运营的三级医疗机构的普通内科和肾内科就诊的PNH患者的数据。这些患者在1998年至2004年初始阶段的PNH诊断基于蔗糖溶血试验和酸溶血试验(Ham试验)。2004年后,诊断基于流式细胞术检测调节补体作用的糖蛋白CD59(反应性溶血膜抑制剂)和CD55(衰变加速因子)。患者数据收集于1998年至2014年。在此期间有14例PNH患者。平均年龄为37岁,范围为16至68岁。有8名女性。6例患者出现急性肾损伤(AKI)。其中4例进行了透析。透析开始时的平均血清肌酐和尿素分别为5.4±0.6和64.1±6.1mg/dl。血液透析的中位数次数为4次。4例患者进行了肾活检。3例患者的尿液分析和血清化学检查提示范科尼综合征。在我们的患者中,确定了PNH的三种肾脏表现。它们是AKI、肾血管血栓形成和范科尼综合征。未发现慢性肾衰竭。