作者
Mahnaz Jamee, Majid Zaki-Dizaji, Bernice Lo, Hassan Abolhassani, Fatemeh Aghamahdi, Mehdi Mosavian, Zohreh Nademi, Hamed Mohammadi, Farhad Jadidi-Niaragh, Manuel Rojas, Juan-Manuel Anaya, Gholamreza Azizi
发表日期
2020/9/1
期刊
The Journal of Allergy and Clinical Immunology: In Practice
卷号
8
期号
8
页码范围
2747-2760. e7
出版商
Elsevier
简介
Background
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare inborn error of immunity caused by mutations in the forkhead box P3 (FOXP3) gene.
Objective
In this study, we conducted a systematic review of patients with IPEX and IPEX-like syndrome to delineate differences in these 2 major groups.
Methods
The literature search was performed in PubMed, Web of Science, and Scopus databases, and demographic, clinical, immunologic, and molecular data were compared between the IPEX and IPEX-like groups.
Results
A total of 459 patients were reported in 148 eligible articles. Major clinical differences between patients with IPEX and IPEX-like syndrome were observed in rates of pneumonia (11% vs 31%, P < .001), bronchiectasis (0.3% vs 14%, P < .001), diarrhea (56% vs 42%, P = .020), and organomegaly (10% vs 23%, P = .001), respectively. Eosinophilia (95% vs 100 …
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M Jamee, M Zaki-Dizaji, B Lo, H Abolhassani… - The Journal of Allergy and Clinical Immunology: In …, 2020