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作 者:张伟[1] 邵乐平[1] Zhang Wei Shao Leping(Department of Nephrology , the Affiliated Hospital of Qingdao University, Qingdao 266000 , Chin)
出 处:《中华老年医学杂志》2017年第10期1156-1160,共5页Chinese Journal of Geriatrics
基 金:国家自然科学基金面上项目(81170653)
摘 要:腹膜后纤维化(RPF)是一种罕见病,以腹膜后异常增生的纤维炎性组织包绕腹主动脉和髂动脉及其相邻结构为特点,分为特发性和继发性两种,既往诊断的部分特发性腹膜后纤维化属于IgG4相关疾病范畴。本文基于对国内外腹膜后纤维化研究现状的分析,简述了RPF的流行病学特点,分析其发病机制,总结其临床特点、病理及影像学特征,以提高临床工作者对该病的诊断率,并阐述了最新诊疗进展。Retroperitoneal fibrosis (RPF) is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs. RPF can be divided into two types: idiopathic and secondary. Some of the previously diagnosed idiopathic retroperitoneal fibrosis cases belong to the IgG4 related disease category. In order to help clinicians to achieve better diagnostic accuracy, the article reviews current national and international research on this disorder, and summarizes the epidemiological characteristics, the pathogenesis, the clinical manifestations, the rnieroscopicand imaging features, and the most recent advances in the management of RPF.
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