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一名患有肾病综合征和“特发性”AA淀粉样变性的21岁男性患者。

[A 21-year-old male patient with nephrotic syndrome and "idiopathic" AA amyloidosis].

作者信息

Saura I Ma, Carretón A, López Guillén E, Alegría M S, Jimeno García L, Ferri B

机构信息

Servicio de Nefrología, Hospital Universitario Virgen de la Arrixaca, Murcia.

出版信息

Nefrologia. 2004;24 Suppl 3:109-12.

Abstract

A case is reported of a 21 year old man who suffered from Castleman's disease and systemic secondary amyloidosis. He was otherwise healthy until development of a history of edema and nocturia over 3 weeks. Physical examination was normal apart from ankle swelling. Renal biopsy showed amyloid deposits in the glomerulus with gentian violet and congo red. Positive staining by antibody against serum amyloid A protein was demonstrated. Nine months later because of persistent nephrotic syndrome, an abdominal tomography was performed and a mesenteric mass was detected. The patient underwent abdominal surgery. The histopathological examination showed plasma cell variant of Castleman disease and AA amyloidosis in the mass. After the removal of mesenteric mass, the proteinuria gradually decreased and disappeared, and no systemic findings were present.

摘要

报告了一例21岁男性患者,患有卡斯尔曼病和系统性继发性淀粉样变性。在出现水肿和夜尿病史超过3周之前,他一直身体健康。除了脚踝肿胀外,体格检查正常。肾活检显示肾小球中有淀粉样沉积物,经龙胆紫和刚果红染色。证实血清淀粉样蛋白A蛋白抗体染色呈阳性。9个月后,由于持续性肾病综合征,进行了腹部断层扫描,发现一个肠系膜肿块。患者接受了腹部手术。组织病理学检查显示肿块为卡斯尔曼病的浆细胞变异型和AA淀粉样变性。切除肠系膜肿块后,蛋白尿逐渐减少并消失,且无全身症状。

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