Hemoglobinuria misidentified as hematuria: review of discolored urine and paroxysmal nocturnal hemoglobinuria.

IF 3 Q2 Medicine
Clinical Medicine Insights-Blood Disorders Pub Date : 2013-06-20 eCollection Date: 2013-01-01 DOI:10.4137/CMBD.S11517
Prashant Veerreddy
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引用次数: 32

Abstract

Discolored urine is a common reason for office visits to a primary care physician and urology referral. Early differentiation of the type or cause of discolored urine is necessary for accurate diagnosis and prompt management. Paroxysmal nocturnal hemoglobinuria is a clonal disorder caused by acquired somatic mutations in the PIG-A gene on the X- chromosome of hemopoietic stem cells and leads to deficiency of surface membrane anchor proteins. The deficiency of these proteins leads to an increased risk of hemolysis of erythrocytes and structural damage of platelets, resulting in a clinical syndrome characterized by complement-mediated intravascular hemolytic anemia, bone marrow failure, and venous thrombosis. Patients with this clinical syndrome present with paroxysms of hemolysis, causing hemoglobinuria manifesting as discolored urine. This can be easily confused with other common causes of discolored urine and result in extensive urologic work-up. Three commonly confused entities of discolored urine include hematuria, hemoglobinuria, and myoglobinuria. Specific characteristics in a dipstick test or urinalysis can guide differentiation of these three causes of discolored urine. This article begins with a case summary of a woman presenting with cranberry-colored urine and a final delayed diagnosis of paryxysmal nocturnal hemoglobinuria. Her hemoglobinuria was misdiagnosed as hematuria, leading to extensive urologic work-up. The article also gives an overview of the approach to diagnosing and treating discolored urine.

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血红蛋白尿误诊为血尿:变色尿和阵发性夜间血红蛋白尿的回顾。
尿液变色是去初级保健医生和泌尿科转诊的常见原因。早期鉴别尿液变色的类型或原因是准确诊断和及时处理的必要条件。阵发性夜间血红蛋白尿是一种克隆性疾病,由造血干细胞X染色体上的猪- a基因获得性体细胞突变引起,并导致表面膜锚蛋白缺乏。这些蛋白的缺乏导致红细胞溶血和血小板结构损伤的风险增加,导致补体介导的血管内溶血性贫血、骨髓衰竭和静脉血栓形成为特征的临床综合征。这种临床综合征的患者表现为阵发性溶血,引起血红蛋白尿,表现为尿液变色。这很容易与尿液变色的其他常见原因混淆,并导致广泛的泌尿系统检查。三种常被混淆的变色尿包括血尿、血红蛋白尿和肌红蛋白尿。在试纸试验或尿液分析的具体特征可以指导这三种原因的尿变色的区分。这篇文章开始与一个案例总结的妇女表现为蔓越莓色尿和最后延迟诊断为阵发性夜间血红蛋白尿。她的血红蛋白尿被误诊为血尿,导致广泛的泌尿系统检查。文章还概述了尿液变色的诊断和治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
3.70
自引率
0.00%
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审稿时长
8 weeks
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