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

Fecha de publicación:

Autores de IIS La Fe

Participantes ajenos a IIS La Fe

  • Vicente-Santamaría S.
  • Tabares-González A.
  • Gascón-Galindo C.
  • Tutau-Gómez C.
  • Álvarez-Beltrán M.
  • Muñoz-Codoceo R.A.
  • Rubio-Murillo M.
  • De-Los-Santos M.
  • Salcedo-Lobato E.
  • García-Romero R.
  • Fernández-Lorenzo A.E.
  • Moreno-Álvarez A.
  • Serrano-Nieto J.
  • Hierro-Llanillo L.
  • Loverdos-Eseverri I.
  • Crehuá-Gaudiza E.
  • Juste-Ruiz M.
  • Blitz-Castro E.
  • Morales-Tirado A.
  • López-Cárdenes C.M.
  • Bousoño-García C.
  • González-Jiménez D.

Grupos

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. Copyright © 2025 Wolters Kluwer Health, Inc.

Datos de la publicación

ISSN/ISSNe:
0954-691X, 1473-5687

EUROPEAN JOURNAL OF GASTROENTEROLOGY & HEPATOLOGY  LIPPINCOTT WILLIAMS & WILKINS

Tipo:
Article
Páginas:
505-509
PubMed:
39976013
Enlace a otro recurso:
www.scopus.com
Factor de Impacto:
0,685 SCImago ℠
Cuartil:
Q2 SCImago ℠

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Keywords

  • Adolescent; Child; Child, Preschool; Cross-Sectional Studies; Cystic Fibrosis; Female; Humans; Infant; Liver Cirrhosis; Liver Diseases; Male; Registries; Spain; ursodeoxycholic acid; adolescent; Article; ascites; child; cohort analysis; controlled study; cross-sectional study; cystic fibrosis; descriptive research; esophagus varices; female; human; hypersplenism; liver cirrhosis; liver disease; liver transplantation; major clinical study; male; medical record review; patient registry; portal hypertension; retrospective study; variceal ligation; complication; cystic fibrosis; diagnosis; epidemiology; etiology; infant; liver cirrhosis; liver disease; preschool child; register; Spain

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