Current situation of pediatric cystic fibrosis-related liver disease: results of a Spanish nationwide study.

IF 1.8 4区医学 Q3 GASTROENTEROLOGY & HEPATOLOGY European Journal of Gastroenterology & Hepatology Pub Date : 2025-04-01 Epub Date: 2025-01-21 DOI:10.1097/MEG.0000000000002917

Saioa Vicente-Santamaría, Ana Tabares-González, Celia Gascón-Galindo, Carlos Tutau-Gómez, Marina Álvarez-Beltrán, Rosa Ana Muñoz-Codoceo, María Rubio-Murillo, Marianela De-Los-Santos, Enrique Salcedo-Lobato, Etna Masip-Simó, Ruth García-Romero, Ana Estefanía Fernández-Lorenzo, Ana Moreno-Álvarez, Juliana Serrano-Nieto, Loreto Hierro-Llanillo, Inés Loverdos-Eseverri, Elena Crehuá-Gaudiza, Mercedes Juste-Ruiz, Enrique Blitz-Castro, Ana Morales-Tirado, Concepción Marina López-Cárdenes, Carlos Bousoño-García, David González-Jiménez

{"title":"Current situation of pediatric cystic fibrosis-related liver disease: results of a Spanish nationwide study.","authors":"Saioa Vicente-Santamaría, Ana Tabares-González, Celia Gascón-Galindo, Carlos Tutau-Gómez, Marina Álvarez-Beltrán, Rosa Ana Muñoz-Codoceo, María Rubio-Murillo, Marianela De-Los-Santos, Enrique Salcedo-Lobato, Etna Masip-Simó, Ruth García-Romero, Ana Estefanía Fernández-Lorenzo, Ana Moreno-Álvarez, Juliana Serrano-Nieto, Loreto Hierro-Llanillo, Inés Loverdos-Eseverri, Elena Crehuá-Gaudiza, Mercedes Juste-Ruiz, Enrique Blitz-Castro, Ana Morales-Tirado, Concepción Marina López-Cárdenes, Carlos Bousoño-García, David González-Jiménez","doi":"10.1097/MEG.0000000000002917","DOIUrl":null,"url":null,"abstract":"Background: Cystic fibrosis-related liver disease (CFRLD) is a health problem that can affect as many as 30-40% of cystic fibrosis patients by the age of 12 years. We studied the epidemiology of CFRLD thanks to the first exclusively pediatric CFRLD patient registry to date.Methods: Descriptive cross-sectional study. Information from medical records from January 2018 to December 2020 is collected. CFRLD was classified according to the European Society of Paediatric Gastroenterology, Hepatology and Nutrition 2017 criteria.Results: Data were collected from 168 pediatric patients diagnosed with CFRLD (90.5% liver involvement without cirrhosis and 8.5% multinodular cirrhosis).Conclusion: In this national registry, including exclusively pediatric population, liver disease is diagnosed around 7 years of age. Liver involvement without cirrhosis is the most frequent finding among our patients but about 9% of the patients already had cirrhosis. CFRLD is one of the challenges faced by pediatric gastroenterologists in the future and national registries give us the opportunity to further study and broaden our knowledge.","PeriodicalId":11999,"journal":{"name":"European Journal of Gastroenterology & Hepatology","volume":" ","pages":"505-509"},"PeriodicalIF":1.8000,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Journal of Gastroenterology & Hepatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1097/MEG.0000000000002917","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/21 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Background: Cystic fibrosis-related liver disease (CFRLD) is a health problem that can affect as many as 30-40% of cystic fibrosis patients by the age of 12 years. We studied the epidemiology of CFRLD thanks to the first exclusively pediatric CFRLD patient registry to date.

Methods: Descriptive cross-sectional study. Information from medical records from January 2018 to December 2020 is collected. CFRLD was classified according to the European Society of Paediatric Gastroenterology, Hepatology and Nutrition 2017 criteria.

Results: Data were collected from 168 pediatric patients diagnosed with CFRLD (90.5% liver involvement without cirrhosis and 8.5% multinodular cirrhosis).

Conclusion: In this national registry, including exclusively pediatric population, liver disease is diagnosed around 7 years of age. Liver involvement without cirrhosis is the most frequent finding among our patients but about 9% of the patients already had cirrhosis. CFRLD is one of the challenges faced by pediatric gastroenterologists in the future and national registries give us the opportunity to further study and broaden our knowledge.

查看原文

微信好友朋友圈 QQ好友复制链接

本刊更多论文

儿童囊性纤维化相关肝病的现状：一项西班牙全国性研究的结果

背景：囊性纤维化相关性肝病（CFRLD）是一种健康问题，可影响多达30-40%的12岁囊性纤维化患者。我们研究了CFRLD的流行病学，这要归功于迄今为止第一个专门的儿科CFRLD患者登记。方法：描述性横断面研究。收集2018年1月至2020年12月的病历信息。CFRLD根据欧洲儿科胃肠病学、肝病学和营养学会2017年标准进行分类。结果：收集了168例诊断为CFRLD的儿童患者的数据（90.5%无肝硬化的肝脏受累，8.5%多结节性肝硬化）。结论：在这个国家登记中，包括专门的儿科人群，肝脏疾病在7岁左右被诊断出来。无肝硬化的肝脏受累是我们患者中最常见的发现，但大约9%的患者已经有肝硬化。CFRLD是未来儿科胃肠病学家面临的挑战之一，国家注册给了我们进一步学习和拓宽知识的机会。

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

European Journal of Gastroenterology & Hepatology 医学-胃肠肝病学

CiteScore

4.40

自引率

4.80%

发文量

269

审稿时长

1 months

期刊介绍： European Journal of Gastroenterology & Hepatology publishes papers reporting original clinical and scientific research which are of a high standard and which contribute to the advancement of knowledge in the field of gastroenterology and hepatology. The journal publishes three types of manuscript: in-depth reviews (by invitation only), full papers and case reports. Manuscripts submitted to the journal will be accepted on the understanding that the author has not previously submitted the paper to another journal or had the material published elsewhere. Authors are asked to disclose any affiliations, including financial, consultant, or institutional associations, that might lead to bias or a conflict of interest.