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一名患有β2-微球蛋白淀粉样蛋白全身沉积的血液透析患者发生致命性颈椎病性关节病。

Fatal cervical spondyloarthropathy in a hemodialysis patient with systemic deposition of beta2-microglobulin amyloid.

作者信息

Danesh F R, Klinkmann J, Yokoo H, Ivanovich P

机构信息

Northwestern University Medical School and VA Chicago Health Care System, Lakeside Divison, IL 60611, USA.

出版信息

Am J Kidney Dis. 1999 Mar;33(3):563-6. doi: 10.1016/s0272-6386(99)70195-2.

Abstract

Destructive spondyloarthropathy is a serious complication in patients with end-stage renal disease. We report a case of fatal cervical spondyloarthropathy in a patient on hemodialysis who presented with severe pain in the cervical area. Magnetic resonance imaging (MRI) of the cervical spine showed a soft tissue mass at the cervico-occipital hinge with spinal cord compression and destructive lesions of the cervical vertebrae. The patient became quadriplegic during the MRI procedure and died a few days later. Postmortem examination showed deposition of beta2-microglobulin in the cervico-occipital hinge. A unique feature of this case was the documented presence of systemic beta2-microglobulin amyloid deposits involving the spleen that to our knowledge has not been reported previously. Clinical suspicion and early detection of lesions caused by dialysis-related amyloidosis (DRA) may help to prevent significant morbidity and mortality in long-term dialysis patients.

摘要

破坏性脊柱关节病是终末期肾病患者的一种严重并发症。我们报告一例接受血液透析的患者发生致命性颈椎关节病,该患者出现颈部严重疼痛。颈椎磁共振成像(MRI)显示颈枕关节处有一软组织肿块,伴有脊髓受压及颈椎破坏性病变。患者在MRI检查过程中出现四肢瘫痪,数日后死亡。尸检显示颈枕关节处有β2-微球蛋白沉积。该病例的一个独特特征是记录到系统性β2-微球蛋白淀粉样沉积物累及脾脏,据我们所知,此前尚未有过相关报道。临床怀疑并早期发现透析相关淀粉样变性(DRA)所致病变,可能有助于预防长期透析患者出现严重的发病和死亡情况。

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