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Phase 3 Completed N=18 Treatment

Evaluation of ELX/TEZ/IVA in Cystic Fibrosis (CF) Subjects 2 Through 5 Years

Source: ClinicalTrials.gov NCT04537793 ↗
Enrolled (actual)
18
Serious AEs
2.2%
Results posted
Jun 2023
Primary outcomePrimary: Part A: Observed Pre-dose Concentration (Ctrough) of ELX,TEZ,IVA, and Relevant Metabolites — 0.00; 3.44; 3.69; 0.00 micrograms per milliliter (mcg/ml)
◆ Published Evidence
Highly cited
129citations · ~43 / year
Phase 3 Open-Label Clinical Trial of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged 2-5 Years with Cystic Fibrosis and at Least One <i>F508del</i> Allele.
American journal of respiratory and critical care medicine · 2023 · Open access · Likely link

Summary

This study will evaluate the safety, tolerability, pharmacokinetics, pharmacodynamics and efficacy of elexacaftor (ELX)/tezacaftor (TEZ)/ivacaftor (IVA) triple combination therapy in CF subjects 2 through 5 years of age.

Linked Publications (3)

  • Phase 3 Open-Label Clinical Trial of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged 2-5 Years with Cystic Fibrosis and at Least One <i>F508del</i> Allele.
    American journal of respiratory and critical care medicine · 2023 · 129 citations · Open access · Likely link
  • Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del).
    The Cochrane database of systematic reviews · 2020 · 71 citations · Open access · Likely link
  • Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del).
    The Cochrane database of systematic reviews · 2023 · 22 citations · Open access · Likely link

Outcome Measures

OutcomeResultp-value
PRIMARY
Part A: Observed Pre-dose Concentration (Ctrough) of ELX,TEZ,IVA, and Relevant Metabolites
0.00; 3.44; 3.69; 0.00; 2.39; 2.47
PRIMARY
Part A: Safety and Tolerability as Assessed by Number of Participants With Treatment- Emergent Adverse Events (TEAEs) and Serious Adverse Events (SAEs)
15; 0
PRIMARY
Part B: Safety and Tolerability as Assessed by Number of Participants With Treatment-Emergent Adverse Events (TEAEs) and Serious Adverse Events (SAEs)
74; 2
SECONDARY
Part B: Observed Pre-dose Plasma Concentration (Ctrough) of ELX,TEZ,IVA and Relevant Metabolites
2.91; 3.87; 3.13; 3.56; 2.48; 3.49
SECONDARY
Part B: Absolute Change in Sweat Chloride (SwCl)
-57.9
SECONDARY
Part B: Absolute Change in Lung Clearance Index 2.5 (LCI 2.5)
-0.83

Eligibility Criteria

Key Inclusion Criteria

  • Homozygous for the F508del mutation or heterozygous for F508del and a minimal function (MF) mutation (F/F or F/MF genotypes)

Key Exclusion Criteria

  • Clinically significant cirrhosis with or without portal hypertension
  • Lung infection with organisms associated with a more rapid decline in pulmonary status
  • Solid organ or hematological transplantation

Other protocol defined Inclusion/Exclusion criteria may apply

View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT04537793) and the linked publication. Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication. Informational only — not medical advice.

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