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在接受血液透析 27 年后出现的双侧臀部淀粉样变瘤的临床、放射学和生化特征。

Clinical, radiological, and biochemical features of a bilateral buttock amyloidoma emerging after 27 years of hemodialysis.

机构信息

IRCCS Fondazione Salvatore Maugeri, Pavia, Italy.

出版信息

Amyloid. 2009;16(3):115-21. doi: 10.1080/13506120903090783.

Abstract

Deposition of amyloid in the buttock is a rare complication of dialysis related amyloidosis (DRA), but this localization is even rarer in other types of amyloidoses. We report here the clinical, radiological, and biochemical features of a patient who incurred into this complication after 27 years of hemodialysis. Imaging of the amyloid deposition by magnetic resonance imaging (MRI) documents the amyloid infiltration in the muscles of the buttock region and highlights a peculiar feature of amyloid fibrils deposition in the subcutaneous fat. The amyloid deposition is confirmed by biochemical and microscopic analysis of fibrils extracted from a biopsy specimen. Review of literature and the features of this case lead to speculation that the peculiar involvement of the buttock region including muscles and subcutaneous fat in DRA might derive from the propagation of amyloid initially deposited in the hip joint.

摘要

臀部淀粉样蛋白沉积是透析相关淀粉样变性(DRA)的罕见并发症,但在其他类型的淀粉样变性中,这种定位更为罕见。我们在此报告一位患者的临床、放射学和生化特征,他在接受 27 年血液透析后发生了这种并发症。磁共振成像(MRI)对淀粉样蛋白沉积的成像记录了臀部区域肌肉中的淀粉样蛋白浸润,并突出了皮下脂肪中淀粉样纤维沉积的一个特殊特征。通过对活检标本中提取的纤维进行生化和显微镜分析,证实了淀粉样蛋白的沉积。对文献的回顾和对该病例的特征的分析表明,DRA 中臀部区域(包括肌肉和皮下脂肪)的特殊受累可能源于最初沉积在髋关节的淀粉样蛋白的传播。

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