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Clinical and functional course of patients with cystic fibrosis treated with lumacaftor/ivacaftor at a children's hospital. A case series.

Archivos argentinos de pediatria - 1 Oct 2023

Fernández Sardá M Sol, Báez Mellid Lourdes, Brizuela Paula, Dicembrino Manuela, Giugno Hilda, Castaños Claudio

Abstract excerpt

Cystic fibrosis transmembrane regulator (CFTR) modulators treat defective CFTR protein. Our objective is to describe the course of children with cystic fibrosis treated with lumacaftor/ivacaftor. This is a case series of 13 patients aged 6 to 18 years with ≥ 6 months of treatment. Forced expiratory volume in the first second (FEV1), body mass index (BMI) Z-score, antibiotic therapy/year, before treatment and for...

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Identifiers

PMID: 36883844
DOI: 10.5546/aap.2022-02825.eng

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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