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西班牙儿科乳糜泻登记处:21 世纪临床特征的变化。

Spanish National Registry of Paediatric Coeliac Disease: Changes in the Clinical Presentation in the 21st Century.

机构信息

Department of Paediatrics, Hospital Universitario San Agustín, Aviles.

Department of Paediatrics, Hospital Universitario Puerta de Hierro, Majadahonda.

出版信息

J Pediatr Gastroenterol Nutr. 2022 Jun 1;74(6):805-811. doi: 10.1097/MPG.0000000000003424. Epub 2022 Mar 2.

Abstract

OBJECTIVES

Over the last several decades, there has been a tendency towards a predominance of less symptomatic forms of coeliac disease (CD) and an increase in the patient age at diagnosis. This study aimed to assess the clinical presentation and diagnostic process of paediatric CD in Spain.

METHODS

A nationwide prospective, observational, multicentre registry of new paediatric CD cases was conducted from January 2011 to June 2017. The data regarding demographic variables, type of birth, breast-feeding history, family history of CD, symptoms, height and weight, associated conditions, serological markers, human leukocyte antigen (HLA) phenotype, and histopathological findings were collected.

RESULTS

In total, 4838 cases (61% girls) from 73 centres were registered. The median age at diagnosis was 4 years. Gastrointestinal symptoms were detected in 71.4% of the patients, and diarrhoea was the most frequent symptom (45.9%). The most common clinical presentation was the classical form (65.1%) whereas 9.8% ofthe patients were asymptomatic. There was a trend towards an increase in the age at diagnosis, proportion of asymptomatic CD cases, and usage of anti-deamidated gliadin peptide antibodies and HLA typing for CD diagnosis. There was, however, a decreasing trend in the proportion of patients undergoing biopsies. Some of these significant trend changes may reflect the effects of the 2012 ESPGHAN diagnosis guidelines.

CONCLUSIONS

Paediatric CD in Spain is evolving in the same direction as in the rest of Europe, although classical CD remains the most common presentation form, and the age at diagnosis remains relatively low.

摘要

目的

在过去几十年中,乳糜泻(CD)的症状较轻的形式呈上升趋势,且诊断时的患者年龄也有所增加。本研究旨在评估西班牙儿科 CD 的临床表现和诊断过程。

方法

2011 年 1 月至 2017 年 6 月,开展了一项针对新儿科 CD 病例的全国性前瞻性、观察性、多中心登记研究。收集了人口统计学变量、出生类型、母乳喂养史、CD 家族史、症状、身高和体重、合并症、血清标志物、人类白细胞抗原(HLA)表型和组织病理学发现等数据。

结果

共登记了来自 73 个中心的 4838 例(61%为女孩)患者。诊断时的中位年龄为 4 岁。71.4%的患者存在胃肠道症状,腹泻是最常见的症状(45.9%)。最常见的临床表现为经典型(65.1%),而 9.8%的患者无症状。诊断时的年龄、无症状 CD 病例的比例、抗脱酰胺麦胶蛋白肽抗体和 HLA 分型用于 CD 诊断的使用呈上升趋势。然而,活检患者的比例呈下降趋势。这些显著的趋势变化可能反映了 2012 年 ESPGHAN 诊断指南的影响。

结论

西班牙儿科 CD 的发展趋势与欧洲其他地区相同,尽管经典 CD 仍然是最常见的表现形式,且诊断时的年龄仍相对较低。

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