Suwabe Tatsuya, Ubara Yoshifumi, Inoue Masafumi, Kitajima Izuru, Oohashi Kenichi, Nakano Imaharu, Takaichi Kenmei
Clin Nephrol. 2014 Jun;81(6):427-34. doi: 10.5414/CN107686.
We performed autopsy on a 60-year-old Japanese man who had received dialysis for 42 years. He started on intermittent peritoneal dialysis in 1968, which was combined with hemodialysis in 1969. His serum calcium-phosphate balance and his blood pressure had been controlled well. Carpal tunnel syndrome occurred in 1984. Then lumbar spinal canal stenosis (SCS) occurred in 1997, followed by cervical SCS in 2000, destructive lumbar spondyloarthropathy (DSA) in 2002, and pathological fracture of the right femoral neck due to an enlarging bone cyst in 2006. All of his surgical specimens showed dialysis-related deposition of beta2MG amyloid (dialysis-related amyloidosis: DRA). Thereafter, lumbar and cervical spinal palsy progressed. In 2009, he developed severe paralytic ileus with dilatation of the sigmoid colon, and subsequently died of peritonitis due to necrotizing cholecystitis. Autopsy showed massive DRA deposits in his intestinal blood vessels and thickened spinal dura, resulting in the above-mentioned intestinal and spinal complications. However, his arterial tree, including the aorta and coronary arteries, showed very little atheroma. Strict control of the Ca-P balance and blood pressure may have prevented cardiovascular disease, while progress in dialysis technology delayed fatal complications of DRA and allowed this patient to survive on dialysis for 42 years.
我们对一名接受了42年透析治疗的60岁日本男性进行了尸检。他于1968年开始间歇性腹膜透析,并于1969年联合血液透析。他的血清钙磷平衡和血压一直控制良好。1984年出现腕管综合征。1997年发生腰椎管狭窄(SCS),随后2000年出现颈椎SCS,2002年出现破坏性腰椎脊椎关节病(DSA),2006年因骨囊肿增大导致右股骨颈病理性骨折。他所有的手术标本均显示有β2微球蛋白淀粉样蛋白的透析相关沉积(透析相关淀粉样变:DRA)。此后,腰椎和颈椎麻痹病情进展。2009年,他出现严重的麻痹性肠梗阻伴乙状结肠扩张,随后因坏死性胆囊炎引起的腹膜炎死亡。尸检显示其肠道血管和增厚的硬脊膜中有大量DRA沉积,导致上述肠道和脊柱并发症。然而,他的动脉系统,包括主动脉和冠状动脉,显示出极少的动脉粥样硬化。严格控制钙磷平衡和血压可能预防了心血管疾病,而透析技术的进步延缓了DRA的致命并发症,使该患者能够存活42年。