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抗肾小球基底膜抗体介导疾病的不典型表现与诊断延迟及预后不良相关。

Unusual presentations of anti-glomerular basement membrane antibody mediated disease are associated with delayed diagnosis and poor outcome.

作者信息

Andrews P A, Sheerin N S, Hicks J A, Williams D G, Sacks S H

机构信息

Department of Nephrology, Guy's Hospital, London, UK.

出版信息

Clin Nephrol. 1995 Oct;44(4):262-5.

PMID:8575127
Abstract

Anti-glomerular basement membrane antibody mediated disease (anti-GBM disease) is an uncommon cause of renal failure. Presentation is usually dramatic, with renal failure and alveolar hemorrhage leading to an early diagnosis. In contrast, we here describe 3 cases which presented with atypical features, and where diagnosis was delayed due to the presence of other factors which were initially felt sufficient to explain the clinical status. No patient recovered renal function, and one died from sepsis. We also present data on 15 patients presenting to Guy's Hospital over the past 10 years with anti-GBM disease and creatinine > 500 mumol/l. The median delay from presentation to diagnosis was 7 days, range 1-81 days. Two patients died, and only one recovered renal function. We speculate that earlier referral and renal biopsy might have improved outcome in these patients, and recommend a policy of early biopsy in all cases of acute renal failure with atypical feature, delay in recovery, or where a clearcut cause is not established.

摘要

抗肾小球基底膜抗体介导的疾病(抗GBM病)是肾衰竭的一种罕见病因。其表现通常较为显著,肾衰竭和肺泡出血可导致早期诊断。相比之下,我们在此描述3例具有非典型特征的病例,由于存在其他最初被认为足以解释临床状况的因素,诊断被延迟。所有患者均未恢复肾功能,1例死于败血症。我们还展示了过去10年在盖伊医院就诊的15例抗GBM病且肌酐>500μmol/L患者的数据。从出现症状到确诊的中位延迟时间为7天,范围为1 - 81天。2例患者死亡,仅1例恢复肾功能。我们推测早期转诊和肾活检可能会改善这些患者的预后,并建议对所有具有非典型特征、恢复延迟或病因不明的急性肾衰竭病例采取早期活检策略。

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Unusual presentations of anti-glomerular basement membrane antibody mediated disease are associated with delayed diagnosis and poor outcome.抗肾小球基底膜抗体介导疾病的不典型表现与诊断延迟及预后不良相关。
Clin Nephrol. 1995 Oct;44(4):262-5.
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