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伴有肾衰竭的肺出血肾炎综合征的血浆置换疗法。

Plasmapheresis in Goodpasture's syndrome with renal failure.

作者信息

Walker R G, d'Apice A J, Becker G J, Kincaid-Smith P, Craswell P W

出版信息

Med J Aust. 1977 Jun 11;1(24):875-9. doi: 10.5694/j.1326-5377.1977.tb131209.x.

Abstract

Four patients who presented with severe proliferative glomerulonephritis associated with linear deposition of IgG on the basement membrane have been treated by intermittent massive plasmapheresis. Two patients had all the features of Goodpasture's syndrome. In the other two cases, the characteristic renal lesion was present but there was no pulmonary involvement. In all cases, renal function improved after plasmapheresis while in two, renal function deteriorated after plasmapheresis was stopped. On of these patients responded to a second course of plasmapheresis but failed to respond to a third. This study supports previous reports of the value of plasmapheresis in patients with Goodpasture's syndrome and renal failure in whom recovery without plasmapheresis has not been recorded. The present study emphasizes the importance of continuing treatment for a period of weeks to permit prolonged remission. If treatment is withdrawn too early, rapid deterioration in renal function may result, and unless plasmapheresis is recommenced at once the renal failure may then prove irreversible.

摘要

4例表现为严重增生性肾小球肾炎且免疫球蛋白G(IgG)沿基底膜呈线性沉积的患者,接受了间歇性大剂量血浆置换治疗。2例患者具备肺出血肾炎综合征的所有特征。另外2例患者有典型的肾脏病变,但无肺部受累。所有病例在血浆置换后肾功能均有改善,其中2例在停止血浆置换后肾功能恶化。其中1例患者对第二个疗程的血浆置换有反应,但对第三个疗程无反应。本研究支持了先前关于血浆置换对肺出血肾炎综合征和肾衰竭患者价值的报道,此前尚无未经血浆置换而康复的记录。本研究强调持续治疗数周以实现长期缓解的重要性。如果过早停止治疗,可能导致肾功能迅速恶化,并且除非立即重新开始血浆置换,否则肾衰竭可能会变得不可逆转。

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