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儿童嗜酸性粒细胞性食管炎的实用最新进展

A Practical Update on Pediatric Eosinophilic Esophagitis.

作者信息

Votto Martina, De Filippo Maria, Caimmi Silvia, Indolfi Cristiana, Raffaele Alessandro, Tosca Maria Angela, Marseglia Gian Luigi, Licari Amelia

机构信息

Pediatric Unit, Department of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, 27100 Pavia, Italy.

Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy.

出版信息

Children (Basel). 2023 Sep 28;10(10):1620. doi: 10.3390/children10101620.

DOI:10.3390/children10101620
PMID:37892285
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10605219/
Abstract

Eosinophilic esophagitis (EoE) is an emerging atopic disease of unknown etiology limited to the esophagus. The pathogenesis is still understood and is likely characterized by type 2 inflammation. Food allergens are the primary triggers of EoE that stimulate inflammatory cells through an impaired esophageal barrier. In children and adolescents, clinical presentation varies with age and mainly includes food refusal, recurrent vomiting, failure to thrive, abdominal/epigastric pain, dysphagia, and food impaction. Upper-gastrointestinal endoscopy is the gold standard for diagnosing and monitoring EoE. EoE therapy aims to achieve clinical, endoscopic, and histological ("deep") remission; prevent esophageal fibrosis; and improve quality of life. In pediatrics, the cornerstones of therapy are proton pump inhibitors, topical steroids (swallowed fluticasone and viscous budesonide), and food elimination diets. In recent years, much progress has been made in understanding EoE pathogenesis, characterizing the clinical and molecular heterogeneity, and identifying new therapeutic approaches. Notably, clinical, molecular, endoscopic, and histological features reflect and influence the evolution of inflammation over time and the response to currently available treatments. Therefore, different EoE phenotypes and endotypes have recently been recognized. Dupilumab recently was approved by FDA and EMA as the first biological therapy for adolescents (≥12 years) and adults with active EoE, but other biologics are still under consideration. Due to its chronic course, EoE management requires long-term therapy, a multidisciplinary approach, and regular follow-ups.

摘要

嗜酸性食管炎(EoE)是一种病因不明的新发特应性疾病,局限于食管。其发病机制仍不明确,可能以2型炎症为特征。食物过敏原是EoE的主要触发因素,通过受损的食管屏障刺激炎症细胞。在儿童和青少年中,临床表现因年龄而异,主要包括拒食、反复呕吐、生长发育迟缓、腹部/上腹部疼痛、吞咽困难和食物嵌塞。上消化道内镜检查是诊断和监测EoE的金标准。EoE治疗旨在实现临床、内镜和组织学(“深度”)缓解;预防食管纤维化;并改善生活质量。在儿科,治疗的基石是质子泵抑制剂、局部类固醇(吞咽氟替卡松和粘性布地奈德)以及食物排除饮食。近年来,在理解EoE发病机制、表征临床和分子异质性以及确定新的治疗方法方面取得了很大进展。值得注意的是,临床、分子、内镜和组织学特征反映并影响炎症随时间的演变以及对现有治疗的反应。因此,最近人们认识到了不同的EoE表型和内型。度普利尤单抗最近被美国食品药品监督管理局(FDA)和欧洲药品管理局(EMA)批准为治疗青少年(≥​12岁)和成人活动性EoE的首个生物疗法,但其他生物制剂仍在考虑之中。由于其病程呈慢性,EoE的管理需要长期治疗、多学科方法和定期随访。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b98/10605219/7a18c3d46b84/children-10-01620-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b98/10605219/82f0a11aad68/children-10-01620-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b98/10605219/0cb9de6968f5/children-10-01620-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b98/10605219/7a18c3d46b84/children-10-01620-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b98/10605219/82f0a11aad68/children-10-01620-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b98/10605219/0cb9de6968f5/children-10-01620-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b98/10605219/7a18c3d46b84/children-10-01620-g003.jpg

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Eosinophilic Gastrointestinal Diseases in Inborn Errors of Immunity.免疫缺陷病中的嗜酸性胃肠道疾病
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