Exocrine Pancreatic Function in Pediatric Patients After Two Years of Triple CFTR Modulator Therapy: Is It Possible to Restore Pancreatic Function?

Pediatric Pulmonology 2026

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Authors: Concepción Marina López Cárdenes, S. Vicente Santamaría, R. García Romero, A. Tabares González, C. Gascón Galindo, E. Blitz Castro, J. Quaresma, A. Morales Tirado, M Gómez García, Martínez, M. Álvarez Merino, E. Torcuato Rubio
+13 more P. Ortiz Pérez, L. Peña-Quintana, A. Reyes-Dominguez, S Sierra San Nicolás, M. Murray Hurtado, Rodrigo del Brío Castillo, M. de los Santos Mercedes, C. García Volpe, M. Álvarez Beltrán, S.Mesonero Cavia, C.Viñas Torne, D. González Jiménez, y Grupo de trabajo de FQ y páncreas de la SEGHNP
DOI: 10.1002/ppul.71625
Cited by: 1
References: 30

Abstract

BACKGROUND: Improvement in exocrine pancreatic function in people with Cystic fibrosis (PwCF) after ivacaftor and lumacaftor/ivacaftor has been documented. Data on triple therapy with elexacaftor/tezacaftor/ivacaftor (ETI) are limited. METHODS: In this multicenter, prospective, observational study, we reviewed changes in fecal elastase-1 (FE-1) in the pediatric population with exocrine pancreatic insufficiency (EPI) after 2 years of ETI. Patients were classified according to FE-1: mild-moderate EPI as 100-199 μg/g and severe EPI as

Fields of Study & Subjects

Cystic Fibrosis Research Advances Gastrointestinal motility and disorders Body Composition Measurement Techniques

Publication details

Journal / source
Pediatric Pulmonology
Publisher
Wiley
Volume
61
Issue
4
Pages
e71625–e71625
Publication date
1 April 2026
Language
EN