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肺出血肾炎综合征:严重肾功能不全后的恢复情况。

Goodpasture syndrome: recovery after severe renal insufficiency.

作者信息

Cohen L H, Wilson C B, Freeman R M

出版信息

Arch Intern Med. 1976 Jul;136(7):835-7. doi: 10.1001/archinte.136.7.835.

Abstract

A 22-year-old woman developed the sudden onset of cough, dyspnea, blood-tinged sputum, and bilateral fluffy infiltrates on her chest x-ray film, together with severe iron deficiency anemia. Urinalysis initially revealed normal values, but gross hematuria developed on the 12th day. Linear deposits of IgG and C3 were present in the GBM; circulating anti-GBM antibodies were also observed initially but had disappeared 13 months later. Hemodialysis was performed because of oliguria and a rising serum creatinine value. She subsequently had a diuresis; 18 months later, the creatinine clearance was 63 ml/min. The anti-GBM antibody response appears to be transient, lasting only a few months, so that if the patient survives the initial insult, stabilization and even some recovery may ensue. Had this patient undergone immediate nephrectomy as part of her initial therapy, the observed favorable outcome would have been denied.

摘要

一名22岁女性突然出现咳嗽、呼吸困难、痰中带血,胸部X线片显示双侧肺部有模糊浸润影,同时伴有严重缺铁性贫血。尿液分析最初结果正常,但在第12天出现肉眼血尿。肾小球基底膜(GBM)有IgG和C3的线性沉积;最初也观察到循环抗GBM抗体,但13个月后消失。因少尿和血清肌酐值升高进行了血液透析。随后她出现利尿;18个月后,肌酐清除率为63 ml/min。抗GBM抗体反应似乎是短暂的,仅持续几个月,因此如果患者能在初始损伤中存活下来,可能会实现病情稳定甚至部分恢复。如果该患者在初始治疗时就接受了肾切除术,就不会出现观察到的良好结果。

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