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表现为先天性肾病综合征的系统性红斑狼疮

Systemic lupus erythematosus presenting as congenital nephrotic syndrome.

作者信息

Dudley J, Fenton T, Unsworth J, Chambers T, MacIver A, Tizard J

机构信息

Department of Paediatric Nephrology, Southmead Hospital, Westbury-on-Trym, Bristol, UK.

出版信息

Pediatr Nephrol. 1996 Dec;10(6):752-5. doi: 10.1007/s004670050209.

DOI:10.1007/s004670050209
PMID:8971898
Abstract

A male Caucasian infant developed nephrotic syndrome at 10 weeks of age. He had high titres of antinuclear antibody (ANA) and anti-double-stranded DNA antibody, with hypocomplementaemia, antiplatelet antibodies and anticardiolipin antibodies. There were no detectable antibodies to extractable nuclear antigens (ENA). His mother was consistently seronegative for anti-ENA (anti-Ro) antibodies and ANA. He developed severe, progressive multisystem involvement, however renal function has remained stable. Immunosuppression has been the mainstay of therapy in this rare presentation of systemic lupus erythematosus.

摘要

一名高加索男婴在10周龄时出现肾病综合征。他有高滴度的抗核抗体(ANA)和抗双链DNA抗体,伴有补体降低、抗血小板抗体和抗心磷脂抗体。未检测到针对可提取核抗原(ENA)的抗体。他的母亲抗ENA(抗Ro)抗体和ANA始终为血清阴性。然而,他出现了严重的、进行性多系统受累,不过肾功能一直保持稳定。在这种罕见的系统性红斑狼疮表现中,免疫抑制一直是主要的治疗方法。

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