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":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>Data were collected from 168 pediatric patients diagnosed with CFRLD (90.5% liver involvement without cirrhosis and 8.5% multinodular cirrhosis).</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":11999,"journal":{"name":"European Journal of Gastroenterology & Hepatology","volume":" ","pages":""},"PeriodicalIF":2.3000,"publicationDate":"2025-01-21","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":"","PubModel":"","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.
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.
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