Prinseau J, Baglin A, Benoit J, Leparc J M, Got C, Louis M F, Attar B, Fritel D
Service de Médecine Interne, Néphrologie et Hémodialyse, Hôpital Ambroise Paré, Boulogne-Billancourt.
Ann Med Interne (Paris). 1989;140(2):94-8.
The incidence of beta-2 microglobulin amyloidosis was assessed in two populations of chronic hemodialysis patients. Out of 34 patients who underwent biopsy during orthopedic surgery (33 cases) or autopsy (1 case), 26 had amyloid deposits which fixed anti-beta microglobulin serum. Out of 55 unselected patients treated for over months at the dialysis centre, 14 (25%) had clinical symptoms suggesting amyloidosis and out of 43 patients who had a systematic radiological skeletal survey, 23 (53%) had bone deposits. The plasma beta microglobulin concentrations (about 20 times the normal value) we not significantly different whether or not the patients had histological proven amyloidosis, clinically or radiologically probable amyloidosis, no detectable amyloidosis. However, the duration of hemodialysis was longer in those with proven or highly probable amyloidosis. The finding illustrate the indirect role of elevation of beta-2 microglobulin in the genesis of this pathology and also the necessity of lowering its concentration in order to avoid the long term complications of osteoarticular deposits, the functional consequences of which may be very serious.
在两组慢性血液透析患者群体中评估了β2微球蛋白淀粉样变性的发病率。在34例于骨科手术期间接受活检的患者(33例)或尸检患者(1例)中,26例有可结合抗β微球蛋白血清的淀粉样沉积物。在透析中心接受治疗超过数月的55例未经挑选的患者中,14例(25%)有提示淀粉样变性的临床症状,在43例接受系统性骨骼放射学检查的患者中,23例(53%)有骨沉积物。无论患者是否有组织学证实的淀粉样变性、临床或放射学上可能的淀粉样变性、未检测到淀粉样变性,其血浆β微球蛋白浓度(约为正常值的20倍)均无显著差异。然而,经证实或高度可能有淀粉样变性的患者血液透析时间更长。这些发现说明了β2微球蛋白升高在这种病理发生过程中的间接作用,也说明了降低其浓度以避免骨关节沉积物长期并发症(其功能后果可能非常严重)的必要性。