Grateau G, Lebrazi H, Valleix S, Hachulla E, Delpech M, Saïle R
Service de médecine interne, L'Hôtel-Dieu de Paris, France.
Rev Med Interne. 2000 Jun;21(6):517-23. doi: 10.1016/s0248-8663(00)89227-9.
The diagnosis of amyloidosis is a four-step strategy requiring: 1) a high degree of suspicion, as the clinical presentation of the disease is very polymorphous; 2) the biochemical nature of the disease; 3) the evaluation of the disease spread and 4) how it evolves.
Simple and noninvasive biopsies usually make it possible to diagnose amyloidosis. Available treatments of generalized amyloidosis require an exact characterization of the nature of amyloid deposits, which is based on the clinical context, immunohistochemical analysis of amyloid deposits, and genetic testing.
Management of amyloidosis should be improved by better characterization of amyloid deposits. This would result in epidemiological data which are currently lacking.
淀粉样变性的诊断是一个四步策略,需要:1)高度怀疑,因为该疾病的临床表现非常多样;2)确定疾病的生化性质;3)评估疾病的扩散情况;4)了解其发展过程。
简单且非侵入性的活检通常能够诊断淀粉样变性。全身性淀粉样变性的现有治疗方法需要基于临床背景、淀粉样沉积物的免疫组织化学分析和基因检测,对淀粉样沉积物的性质进行精确表征。
通过更好地表征淀粉样沉积物,应改进淀粉样变性的管理。这将产生目前缺乏的流行病学数据。