Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome Manifesting as Lymphocytic Interstitial Pneumonia and Treatment-Resistant Bullous Pemphigoid.

IF 1.1 4区医学 Q4 ALLERGY

Pediatric Allergy Immunology and Pulmonology Pub Date : 2021-06-01 DOI:10.1089/ped.2020.1307

Stine Maria Andersen, Simon Fage, Sune Leisgaard Rubak, Mette Holm, Jens Magnus Bernth Jensen, Trine Mogensen, Mette Deleuran

引用次数: 2

Abstract

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare immune deficiency with a broad clinical presentation. IPEX syndrome causes dysfunctional regulatory T cells, increasing the risk of autoimmune diseases. In this case report, we describe a 7-year-old boy with lymphocytic interstitial pneumonia and bullous pemphigoid who was recently diagnosed with IPEX syndrome.

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以淋巴细胞间质性肺炎和难治性大疱性类天疱疮为表现的免疫失调、多内分泌病、肠病、x连锁综合征。

免疫失调、多内分泌病、肠病、x连锁综合征是一种罕见的免疫缺陷，具有广泛的临床表现。IPEX综合征导致调节性T细胞功能失调，增加自身免疫性疾病的风险。在这个病例报告中，我们描述了一个患有淋巴细胞间质性肺炎和大疱性类天疱疮的7岁男孩，他最近被诊断为IPEX综合征。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Allergy Immunology and Pulmonology ALLERGY-IMMUNOLOGY

CiteScore

2.00

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： Pediatric Allergy, Immunology, and Pulmonology is a peer-reviewed journal designed to promote understanding and advance the treatment of respiratory, allergic, and immunologic diseases in children. The Journal delivers original translational, clinical, and epidemiologic research on the most common chronic illnesses of children—asthma and allergies—as well as many less common and rare diseases. It emphasizes the developmental implications of the morphological, physiological, pharmacological, and sociological components of these problems, as well as the impact of disease processes on families. Pediatric Allergy, Immunology, and Pulmonology coverage includes: -Functional and genetic immune deficiencies- Interstitial lung diseases- Both common and rare respiratory, allergic, and immunologic diseases- Patient care- Patient education research- Public health policy- International health studies