小儿抗肾小球基底膜病伴快速进行性肾小球肾炎和出血性肺泡炎(文献综述)

N. Savenkova, K. A. Papayan
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引用次数: 0

摘要

本文致力于自身免疫性小血管炎伴肾、肺损害的争议性问题。关于AT致GBM的发病机制、临床表现、治疗、结局和预后的现代资料显示:针对IV型GBM (Goodpasture自体抗原)的非胶原结构域-1 (NC1) α3链,具有IgG类AT的特发性疾病(IgG1和IgG3);随着AT对GBM和ANCA的同时增加;Alport综合征患儿移植肾中α5(IV)和α3(IV)链发生AT的“新生”疾病
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Anti–glomerular basement membrane disease with rapidly progressive glomerulonephritis and hemorrhagic alveolitis in pediatric patients (reviev of literature)
   The article is devoted to controversial issues of autoimmune small vessel vasculitis with kidney and lung damage. Modern data on the features of pathogenesis, clinical manifestation, treatment, outcome, and prognosis of the disease with AT to GBM have presented: an idiopathic disease with AT of class IgG (IgG1 and IgG3) against non-collagen domain-1 (NC1) α3-chain ofcollagen type IV GBM (Goodpasture autoantigen); with the simultaneous increase in AT to GBM and ANCA; "de novo" diseases with AT to the α5(IV) and α3(IV) chains of GBM type IV collagen in renal graft with Alport syndrome in pediatric patients.
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