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Observational Study of Glucose Tolerance Abnormalities in Patient With Cystic Fibrosis Homozygous for Phe 508 Del CFTR Treated by Lumacaftor-Ivacaftor

NCT03512119 · Status: COMPLETED · Type: OBSERVATIONAL · Enrollment: 55

Last updated 2021-11-24

No results posted yet for this study

Summary

Cystic Fibrosis related diabetes (CFRD), a major factor of morbid-mortality in CF, is characterized by a preclinical phase of glucose intolerance particularly long reaching up to 10 years.

At the physiopathology level, insulin secretion is determinant in the glucose tolerance abnormalities in CF. Indeed insulin secretion is dependent of the CFTR activity at the beta cell surface and inhibition of CFTR leads to a decrease in insulin secretion.

Recently, the combination of the lumacaftor, a CFTR corrector, with Ivacaftor, a CFTR potentiator, was studied in patient with CF homozygous for the Phe508 del CFTR mutation patients and showed an improvement of the respiratory state in comparison with the placebo group.

These data suggests that lumacaftor in combination with ivacaftor in targeting CFTR action may have an early impact on the insulin-secretion and consequently on the glucose tolerance.

Conditions

  • Cystic Fibrosis Homozygous for Phe 508 Del CFTR
  • Glucose Intolerance or Newly Diagnosis Diabetes

Interventions

DRUG

Lumacaftor-Ivacaftor treatment

Lumacaftor-Ivacaftor treatment during one year

Sponsors & Collaborators

  • University Hospital, Strasbourg, France

    lead OTHER

Eligibility

Min Age
12 Years
Sex
ALL
Healthy Volunteers
No

Timeline & Regulatory

Start
2016-02-11
Primary Completion
2019-04-10
Completion
2019-04-10

Countries

  • France

Study Locations

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Read the full study record

This page highlights key information. For complete eligibility criteria, study locations, investigator contacts, and the full protocol, visit the original record on ClinicalTrials.gov.

View NCT03512119 on ClinicalTrials.gov