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Article

The use of lumacaftor/ivacaftor to treat acute deterioration in paediatric cystic fibrosis.

Paediatric respiratory reviews - 1 Jun 2018

Hammond James A, Connett Gary J

Abstract excerpt

Lumacaftor/ivacaftor is a precision medicine targeting the defective cystic fibrosis transmembrane regulator (CFTR) protein in cystic fibrosis (CF) patients homozygous for Phe508del genotype. Whilst there is evidence for efficacy in children aged 6-11 years who are stable with good lung function, there are little data about the use of this medication for children with acute deterioration in this age group. We...

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Source

Identifiers

PMID: 29914743
DOI: 10.1016/j.prrv.2018.05.008

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026.

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The use of lumacaftor/ivacaftor to treat acute deterioration in paediatric cystic fibrosis.PMID 29914743Read ↗

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Lumacaftor/ivacaftor, a novel agent for the treatment of cystic fibrosis patients who are homozygous for the F580del CFTR mutation.Semantic proximity Read ↗

Managing cystic fibrosis in children aged 6-11yrs: a critical review of elexacaftor/tezacaftor/ivacaftor combination therapy.Semantic proximity Read ↗

Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects.Semantic proximity Read ↗

Lumacaftor/ivacaftor reduces exacerbations in adults homozygous for Phe508del mutation with severe lung disease.Semantic proximity Read ↗

The New and Advanced Cystic fibrosis transmembrane conductance regulators Modulators in Children with Cystic Fibrosis: A Narrative Review on Long-Term Outcomes, Recent Advances, and Comparative Insights.Semantic proximity Read ↗

Ivacaftor for the treatment of cystic fibrosis in children under six years of age.Semantic proximity Read ↗

Clinical Effectiveness of Lumacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for F508del-CFTR. A Clinical Trial.Semantic proximity Read ↗

Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study.Semantic proximity Read ↗

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

Tezacaftor for the treatment of cystic fibrosis.Semantic proximity Read ↗

Real-World Clinical Outcomes of Lumacaftor-Ivacaftor or Tezacaftor-Ivacaftor Therapy in Children With Cystic Fibrosis Homozygous for Phe508del.Semantic proximity Read ↗

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