Kanai H, Kashiwagi M, Hirakata H, Nagashima A, Tada S, Yao T, Nakamoto M, Nakamura S, Fujishima M
The Second Department of Internal Medicine, Faculty of Medicine, Kyushu University, Japan.
Clin Nephrol. 2000 May;53(5):394-9.
Dialysis-related amyloidosis (DRA) is one of the most serious complications interfering with rehabilitation in dialysis patients. Here, we report a case of beta2-microglobulin (beta2M)-related amyloidosis, in which the patient developed a severe intestinal pseudo-obstruction. The patient was a 42-year-old male who had been undergoing hemodialysis for 13 years, and who had no history of osteoarticular involvement of DRA. The first symptoms of the disease were severe abdominal fullness and nausea after meals. The whole intestinal wall biopsy revealed massive amyloid deposition in the propria muscularis. The patient became malnourished and died of acute subendocardial infarction 3 years after the onset. An autopsical examination revealed a massive deposition of amyloid, which was positively stained with anti-beta2M antibody but not AA amyloid, predominantly in the gastrointestinal muscular layer, including the tongue, esophagus, stomach, small intestines, colon, and rectum. These results suggest that the gastrointestinal involvement of beta2M-related amyloidosis might occur during the course of hemodialysis treatment, and that this possibility should be considered if patients suffer from intestinal pseudo-obstruction without osteoarticular symptoms.
透析相关淀粉样变性(DRA)是影响透析患者康复的最严重并发症之一。在此,我们报告一例β2微球蛋白(β2M)相关淀粉样变性病例,该患者出现了严重的肠道假性梗阻。患者为一名42岁男性,已接受血液透析13年,无DRA骨关节受累病史。该病的首发症状是餐后严重腹胀和恶心。全肠壁活检显示固有肌层有大量淀粉样沉积。患者出现营养不良,并在发病3年后死于急性心内膜下梗死。尸检显示有大量淀粉样沉积,用抗β2M抗体呈阳性染色,但不是AA淀粉样蛋白,主要沉积在包括舌、食管、胃、小肠、结肠和直肠在内的胃肠道肌层。这些结果表明,β2M相关淀粉样变性的胃肠道受累可能发生在血液透析治疗过程中,如果患者出现无骨关节症状的肠道假性梗阻,应考虑这种可能性。