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

IF 0.8 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
{"title":"Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome Manifesting as Lymphocytic Interstitial Pneumonia and Treatment-Resistant Bullous Pemphigoid.","authors":"Stine Maria Andersen,&nbsp;Simon Fage,&nbsp;Sune Leisgaard Rubak,&nbsp;Mette Holm,&nbsp;Jens Magnus Bernth Jensen,&nbsp;Trine Mogensen,&nbsp;Mette Deleuran","doi":"10.1089/ped.2020.1307","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":"34 2","pages":"76-79"},"PeriodicalIF":0.8000,"publicationDate":"2021-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8329723/pdf/ped.2020.1307.pdf","citationCount":"2","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Allergy Immunology and Pulmonology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1089/ped.2020.1307","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"ALLERGY","Score":null,"Total":0}
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.

查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
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。
求助全文
约1分钟内获得全文 去求助
来源期刊
CiteScore
2.00
自引率
0.00%
发文量
23
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
期刊最新文献
Corrigendum to: A Rare Clinical Course in Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome: Lung Involvement. Multiple Organ Involvement in a Boy with Lipopolysaccharide-Responsive Beige-like Anchor Protein Deficiency Detected by F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography. TauroSept-Induced Anaphylaxis: A Pediatric Case Report. Intravenous Lipid Emulsion-Induced Soy Allergy and Atopic Dermatitis in an Infant: A Case Report. Improved Lung Function with Home Oscillation and Lung Expansion Therapy in Children: A Case Series.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1