儿童期自身免疫性多内分泌综合征
Autoimmune Polyendocrine Syndromes in the Pediatric Age.
作者信息
Paparella Roberto, Menghi Michela, Micangeli Ginevra, Leonardi Lucia, Profeta Giovanni, Tarani Francesca, Petrella Carla, Ferraguti Giampiero, Fiore Marco, Tarani Luigi
机构信息
Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
Department of Experimental Medicine, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
出版信息
Children (Basel). 2023 Mar 19;10(3):588. doi: 10.3390/children10030588.
Autoimmune polyendocrine syndromes (APSs) encompass a heterogeneous group of rare diseases characterized by autoimmune activity against two or more endocrine or non-endocrine organs. Three types of APSs are reported, including both monogenic and multifactorial, heterogeneous disorders. The aim of this manuscript is to present the main clinical and epidemiological characteristics of APS-1, APS-2, and IPEX syndrome in the pediatric age, describing the mechanisms of autoimmunity and the currently available treatments for these rare conditions.
自身免疫性多内分泌综合征（APSs）是一组罕见的异质性疾病，其特征是针对两个或更多内分泌或非内分泌器官的自身免疫活动。已报道了三种类型的APSs，包括单基因和多因素的异质性疾病。本文的目的是介绍儿童期APS-1、APS-2和IPEX综合征的主要临床和流行病学特征，描述自身免疫的机制以及目前针对这些罕见疾病的可用治疗方法。
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