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首页> 外文期刊>Journal of Medical Case Reports >Collapsing glomerulopathy in sickle cell disease: a case report
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Collapsing glomerulopathy in sickle cell disease: a case report

机译:镰状细胞病中的肾小球塌陷症:一例报告

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Introduction Sickle cell disease has been associated with many renal structural and functional abnormalities. Collapsing glomerulopathy or the collapsing variant of focal segmental glomerulosclerosis is a rare clinicopathologic entity in patients with sickle cell disease that requires timely diagnosis and aggressive management. Case presentation In this case report we describe a 21-year-old African-American woman with a medical history of significant sickle cell disease and asthma. She was admitted for pain, decreased urine output, bilateral leg swelling and reported weight gain. During her period of hospitalisation she developed acute renal failure requiring dialysis. Further investigation revealed the collapsing variant of focal segmental glomerulosclerosis. Conclusions Although focal segmental glomerulosclerosis is a common feature of sickle cell nephropathy, the collapsing variant of focal segmental glomerulosclerosis or collapsing glomerulopathy has been rarely documented. Even when other risk factors are controlled, collapsing glomerulopathy has a very poor prognosis. This is a rare case of a patient with massive proteinuria presenting as acute renal failure with a very poor response to corticosteroids and a much faster rate of progression to end-stage renal disease.
机译:简介镰状细胞病与许多肾脏结构和功能异常有关。在镰状细胞病患者中,塌陷的肾小球病变或局灶节段性肾小球硬化的塌陷变体是罕见的临床病理实体,需要及时诊断和积极治疗。病例介绍在此病例报告中,我们描述了一名21岁的非洲裔美国妇女,她有严重的镰状细胞病和哮喘病史。她因疼痛,尿量减少,双腿肿胀和体重增加而入院。在住院期间,她发生了急性肾衰竭,需要透析。进一步的研究显示局灶性节段性肾小球硬化的崩溃变体。结论尽管局灶节段性肾小球硬化是镰状细胞性肾病的常见特征,但局灶节段性肾小球硬化或塌陷型肾小球病变的变体很少见。即使控制了其他危险因素,塌陷性肾小球病的预后也很差。这是罕见的大面积蛋白尿患者,表现为急性肾功能衰竭,对皮质类固醇的反应非常差,进展为晚期肾病的速度更快。

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