Autoimmune Polyendocrine Syndromes in the Pediatric Age

被引:7
|
作者
Paparella, Roberto [1 ]
Menghi, Michela [1 ]
Micangeli, Ginevra [1 ]
Leonardi, Lucia [1 ]
Profeta, Giovanni [1 ]
Tarani, Francesca [2 ]
Petrella, Carla [3 ]
Ferraguti, Giampiero [2 ]
Fiore, Marco [3 ]
Tarani, Luigi [1 ]
机构
[1] Sapienza Univ Rome, Dept Maternal Infantile & Urol Sci, Viale Policlin 155, I-00161 Rome, Italy
[2] Sapienza Univ Rome, Dept Expt Med, Viale Policlin 155, I-00161 Rome, Italy
[3] Inst Biochem & Cell Biol, IBBC CNR, I-00185 Rome, Italy
来源
CHILDREN-BASEL | 2023年 / 10卷 / 03期
关键词
autoimmune; polyendocrinopathy; polyglandular; immunodeficiency; pediatrics; children; autoantibodies; QUALITY-OF-LIFE; POLYGLANDULAR SYNDROME; ADRENAL INSUFFICIENCY; ADDISONS-DISEASE; CANDIDIASIS; MANIFESTATIONS; AUTOANTIBODIES; COMPLICATIONS; ENTEROPATHY; MANAGEMENT;
D O I
10.3390/children10030588
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
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.
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页数:12
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