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β2微球蛋白所致的全身性淀粉样变,长期血液透析后出现盲肠穿孔。

Generalized amyloidosis from beta 2-microglobulin, with caecal perforation after long-term haemodialysis.

作者信息

Zhou H, Pfeifer U, Linke R

机构信息

Pathologisches Institut der Universität, Bonn, Federal Republic of Germany.

出版信息

Virchows Arch A Pathol Anat Histopathol. 1991;419(4):349-53. doi: 10.1007/BF01606526.

Abstract

A 73-year-old man with chronic renal failure of undetermined aetiology had received haemodialysis for 12 years when he died of acute purulent peritonitis due to caecal perforation. Amyloid deposits detected in a cystic bone lesion in the left hip had caused a pathological fracture 17 days before death. At autopsy, extensive amyloid deposits were found in the osteoarticular system, in the cartilaginous surface and the capsular tissue of joints, ligaments, vertebral discs and bone. In addition, vascular amyloid deposits were diagnosed in the heart, kidneys, testes, lungs, skin and in the gastrointestinal tract. A special feature of this case were interstitial amyloid deposits forming a fine-meshed structure in the myocardium and plate-like deposits in the gastrointestinal tract. Immunohistochemically, all these deposits reacted strongly with antibody to human beta 2-microglobulin but showed no reaction with antibodies to AA, A-lambda, A-kappa and AF. The present case demonstrates that extra-osteoarticular manifestations of AB-amyloidosis can cause serious complications.

摘要

一名病因不明的慢性肾衰竭73岁男性,在因盲肠穿孔导致急性化脓性腹膜炎死亡前已接受血液透析12年。左髋部囊性骨病变中检测到的淀粉样沉积物在死亡前17天导致了病理性骨折。尸检时,在骨关节系统、关节软骨表面、关节囊组织、韧带、椎间盘和骨骼中发现了广泛的淀粉样沉积物。此外,在心脏、肾脏、睾丸、肺、皮肤和胃肠道中诊断出血管淀粉样沉积物。该病例的一个特殊特征是间质淀粉样沉积物在心肌中形成细网状结构,在胃肠道中形成板状沉积物。免疫组织化学分析显示,所有这些沉积物与人β2-微球蛋白抗体反应强烈,但与AA、A-λ、A-κ和AF抗体无反应。本病例表明,AB型淀粉样变性的关节外表现可引起严重并发症。

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