Hedeshi A R, Brenez D, Benaddia L, Vandervelde D, Khodadadi E, Ardichvili D, Malarme M
Service de Médecine, H.I.S., Site Joseph Bracops, U.L.B.
Rev Med Brux. 2001 Feb;22(1):9-14.
AL amyloidosis is a systemic disease caused by the deposition of fibrillar proteins in organs and tissues. These deposits are responsible for organic dysfunctions and may be rapidly lethal. Current therapy is unsatisfactory. We describe three clinical cases of multisystemic AL amyloidosis but with different initial presentation: nephrotic syndrome, cardiac failure and hepatic insufficiency. We review the literature about the clinical features, diagnostic methods and treatment of the disease.
AL淀粉样变性是一种由纤维状蛋白在器官和组织中沉积引起的全身性疾病。这些沉积物导致器官功能障碍,可能迅速致命。目前的治疗方法并不令人满意。我们描述了三例多系统AL淀粉样变性的临床病例,但初始表现不同:肾病综合征、心力衰竭和肝功能不全。我们回顾了有关该疾病临床特征、诊断方法和治疗的文献。