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一名患有中性粒细胞减少症儿童的肾淀粉样变性

Renal amyloidosis in a child with neutropenia.

作者信息

Otukesh Hasan, Hoseini Rozita, Behzadi Ashkan Heshmatzade, Fereshtehnejad Seyed-Mohammad, Mehrazma Mitra, Parvin Mahmoud, Mojtahedzadeh Mona, Razizadeh Nazli, Pourfakharan Mohammad

机构信息

Department of Pediatric Nephrology, Ali Asghar Children Hospital, Tehran, Iran.

出版信息

Saudi J Kidney Dis Transpl. 2011 Sep;22(5):1019-21.

Abstract

Amyloidosis represents a heterogeneous group of disorders of protein metabolism and is characterized by deposition of fibrillar proteins in the intra- and extracellular spaces. Here, a case of generalized amyloidosis associated with neutropenia is presented. She had a medical history of multiple bacterial infections. At the age of 14 years, she developed nephrotic syndrome. An increase of antigenic stimulation during the intermittent bouts of acute infections would have been the main factor responsible for the development of secondary amyloidosis in this case. To the best of our knowledge, coexistence between neutropenic disorders and renal amyloidosis in children has not been reported till date. The purpose of this report is to present a case of secondary amyloidosis associated with neutropenia in pediatric age group, probably for the first time.

摘要

淀粉样变性是一组蛋白质代谢异常的异质性疾病,其特征是纤维状蛋白质在细胞内和细胞外间隙沉积。本文报告一例与中性粒细胞减少相关的全身性淀粉样变性病例。她有多次细菌感染病史。14岁时,她患上了肾病综合征。在急性感染的间歇性发作期间抗原刺激的增加可能是该病例继发性淀粉样变性发展的主要因素。据我们所知,儿童中性粒细胞减少症与肾淀粉样变性并存的情况迄今为止尚未见报道。本报告的目的是首次介绍一例儿童年龄组中与中性粒细胞减少相关的继发性淀粉样变性病例。

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