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肾淀粉样变性的临床和生化结果

Clinical and biochemical outcome of renal amyloidosis.

作者信息

Odabas A R, Cetinkaya R, Selcuk Y, Erman Z, Bilen H

机构信息

Department of Nephrology, Ataturk University, School of Medicine, Erzurum, Turkey.

出版信息

Int J Clin Pract. 2002 Jun;56(5):342-4.

Abstract

AA amyloidosis is a relatively rare disease which complicates chronic inflammatory diseases, chronic infections, familial Mediterranean fever (FMF) and malignant diseases. Although amyloid deposition may be found in many organs, renal involvement dominates the clinical picture. We reviewed 63 patients with AA amyloidosis who presented to our nephrology department between 1995 and 2000. Prognostic markers, detailed history, physical examination and laboratory tests were evaluated. The causes of AA amyloidosis were as follows: FMF 42 (66.6%), pulmonary tuberculosis 9 (14.2%), chronic osteomyelitis 4 (6.3%), bronchiectasia 4 (6.3%), rheumatoid arthritis 1 (1.5%), juvenile idiopathic arthritis 1 (1.5%), inflammatory abdominal aortic aneurysm 1 (1.5 %), unknown aetiology 1 (1.5%). The diagnosis was made on renal biopsies in 63.4% of the patients, while the remaining 36.6% were diagnosed as a result of rectal biopsies. Sixteen patients died. A low serum albumin, high creatinine and high 24-hour urine albumin excretion were associated with high mortality.

摘要

AA型淀粉样变性是一种相对罕见的疾病,常并发于慢性炎症性疾病、慢性感染、家族性地中海热(FMF)和恶性疾病。尽管淀粉样蛋白沉积可在多个器官中发现,但肾脏受累在临床表现中占主导地位。我们回顾了1995年至2000年间到我们肾脏病科就诊的63例AA型淀粉样变性患者。对预后标志物、详细病史、体格检查和实验室检查进行了评估。AA型淀粉样变性的病因如下:FMF 42例(66.6%)、肺结核9例(14.2%)、慢性骨髓炎4例(6.3%)、支气管扩张4例(6.3%)、类风湿性关节炎1例(1.5%)、幼年特发性关节炎1例(1.5%)、炎性腹主动脉瘤1例(1.5%)、病因不明1例(1.5%)。63.4%的患者通过肾活检确诊,其余36.6%通过直肠活检确诊。16例患者死亡。血清白蛋白水平低、肌酐水平高和24小时尿白蛋白排泄量高与高死亡率相关。

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