ESPghan 欧洲儿科嗜酸性食管炎注册研究(pEEr)对嗜酸性食管炎的特征描述。

Characterization of Eosinophilic Esophagitis From the European Pediatric Eosinophilic Esophagitis Registry (pEEr) of ESPGHAN.

机构信息

From the Maternal and Child Department, Pediatric Gastroenterology and Liver Unit, Sapienza - University of Rome, Rome, Italy.

the Pediatric Gastroenterology Unit, Centro Hospitalar São João, Porto, Portugal.

出版信息

J Pediatr Gastroenterol Nutr. 2022 Sep 1;75(3):325-333. doi: 10.1097/MPG.0000000000003530. Epub 2022 Aug 9.

Abstract

OBJECTIVES

Few pediatric data on phenotypic aspects of eosinophilic esophagitis (EoE) are available. The pEEr registry was developed to prospectively characterize children with EoE from Europe and Israel.

METHODS

pEEr is an ongoing prospective registry enrolling children with esophageal eosinophilia (≥15 eos/HPF). Anonymized data were collected from 19 pediatric centers. Data regarding demographics, clinical manifestations, endoscopy, histology, and therapies were collected.

RESULTS

A total of 582 subjects (61% male) were analyzed. The median age at diagnosis was 10.5 years [interquartile range (IQR): 5.7-17.7], whereas the age at symptom onset was 9.2 years (IQR: 4.3-16.4), resulting in a median diagnostic delay of 1.2 years (IQR: 0.7-2.3). The diagnostic delay was longer below age <6 years. Shorter diagnostic delays were associated with the presence of food allergy or a family history for EoE. Symptoms varied by age with dysphagia and food impaction more common in adolescents, while vomiting and failure to thrive more common in younger children ( P < 0.001). Among endoscopic findings, esophageal rings were more common in adolescents, whereas exudates were more frequent in younger children( P < 0.001). Patients who responded to proton pump inhibitors (PPIs) were more likely to be older, males, and less often presented severe endoscopic findings. Patients unresponsive to PPIs received topical steroids (40%), elimination diet (41%), or a combined therapy (19%).

CONCLUSIONS

EoE findings vary according to age in pediatric EoE. Young children are commonly characterized by non-specific symptoms, atopic dermatitis, food allergy, and inflammatory endoscopic lesions. Adolescents usually have dysphagia or food impaction, fibrostenotic lesions, and a better PPI response.

摘要

目的

有关嗜酸性粒细胞性食管炎(EoE)表型方面的儿科数据很少。pEEr 登记册是为了前瞻性地描述来自欧洲和以色列的 EoE 患儿而开发的。

方法

pEEr 是一项正在进行的前瞻性登记,纳入食管嗜酸性粒细胞增多(≥15 个 eos/HPF)的患儿。从 19 个儿科中心收集了匿名数据。收集了人口统计学、临床表现、内镜、组织学和治疗的数据。

结果

共分析了 582 名患儿(61%为男性)。诊断时的中位年龄为 10.5 岁[四分位距(IQR):5.7-17.7],而症状发作时的中位年龄为 9.2 岁(IQR:4.3-16.4),导致中位诊断延迟 1.2 年(IQR:0.7-2.3)。<6 岁以下儿童的诊断延迟时间更长。较短的诊断延迟与食物过敏或 EoE 家族史有关。症状随年龄而变化,青少年更常见吞咽困难和食物嵌塞,而幼儿更常见呕吐和生长不良(P<0.001)。在内镜表现中,食管环在青少年中更常见,而渗出物在幼儿中更常见(P<0.001)。对质子泵抑制剂(PPIs)有反应的患者更可能是年龄较大的男性,且较少表现出严重的内镜表现。对 PPI 无反应的患者接受了局部类固醇(40%)、排除饮食(41%)或联合治疗(19%)。

结论

儿科 EoE 中,EoE 的发现随年龄而变化。幼儿通常表现为非特异性症状、特应性皮炎、食物过敏和炎症性内镜病变。青少年通常有吞咽困难或食物嵌塞、纤维性狭窄病变和更好的 PPI 反应。

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