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Effect of One Year Elexacaftor-tezacaftor-ivacaftor Treatment

Completed
Conditions
Cystic Fibrosis
Interventions
Drug: elexacaftor-tezacaftor-ivacaftor treatment
Registration Number
NCT06331000
Lead Sponsor
University Hospital, Strasbourg, France
Brief Summary

The development of CFTR (cystic fibrosis conductance transmembrane regulator) modulators for people with cystic fibrosis (pwCF) and eligible for these treatments is a true therapeutic revolution. The major beneficial effect of CFTR modulators (CFTRm) on pulmonary function and the reduction of pulmonary exacerbations should have a considerable impact on the quality of life and patient's life expectancy. Data on the impact of CFTRm on glucose tolerance abnormalities are still very fragmentary. The investigators can think that their use, earlier and earlier in the history of the disease, will transform the evolutionary trajectories of patients on the respiratory, nutritional and metabolic levels.

Diabetes represents a major challenge in the management of pwCF because it is a factor in morbidity and mortality at all stages of the disease, from children to patients with terminal respiratory failure requiring lung transplantation. Early abnormalities in glucose tolerance observed in childhood, before the stage of diabetes, are also associated with poor pulmonary and nutritional outcomes. Experimental data suggest a positive effect of CFTRm on insulin secretion. However, investigators do not currently know the impact of CFTRm in patients with very early glucose disorders or at the stage of diabetes treated with insulin. Recently continuous glucose measurement (CGM) devices represent very effective tools for assessing abnormalities in glucose tolerance before the stage of diabetes and for monitoring patients treated with insulin.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
184
Inclusion Criteria
  • Patients with cystic fibrosis aged over 18
  • Patients treated for 1 year by elexacaftortezacaftor-ivacaftor
  • Normal tolerant or intolerant to glucose or presenting with cystic fibrosis related diabetes treated or not with insulin
  • Patient who had a continuous glucose monitoring in the 6 months before and 6 months after one year of treatment with elexacaftor-tezacaftor-ivacaftor
Exclusion Criteria
  • Patient not eligible for triple CFTR modulator
  • Patient intolerant to triple corrector
  • Pregnant woman

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Arm && Interventions
GroupInterventionDescription
Adult patients with cystic fibrosiselexacaftor-tezacaftor-ivacaftor treatment-
Primary Outcome Measures
NameTimeMethod
Evaluate time in range of blood glucose variation >140 mg/dl during 2 weeks in a cohort of adult patients treated for one year with elexacaftor-tezacaftor-ivacaftortime in range of blood glucose variation >140 mg/dl one year before and one year after the begining of elexacaftor-tezacaftor-ivacaftor treatment
Secondary Outcome Measures
NameTimeMethod
Assessment of respiratory function parameters : number of IV antibioticsOne year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment
Evaluate other CGM parameters : time in range of blood glucose >180mg/dl ; >140mg/dl ; <70mg/dl ; 70-180 mg/dl ; 70-140mg/dlOne year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment
Marker of metabolic status: C-Peptid (µg/L)One year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment
Marker of metabolic status: C-Peptid (nmol/L)One year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment
Markers of nutritional status: Evaluation of weight monitoring (kg)One year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment
Marker of metabolic status: HbA1c (mmol/l)One year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment
Marker of metabolic status: HbA1c (%)One year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment
Markers of nutritional status: Body Mass Index (BMI)One year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment
Diabetes treatment (assess insulin needs) : mean glucose value per day (mg/dl)One year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment
Number patients with severe hypoglycaemia and serious adverse eventsOne year before and one year after the start of elexacaftor-tezacaftor-ivacaftor treatment

Trial Locations

Locations (1)

Hopitaux Universitaires de Strasbourg

🇫🇷

Strasbourg, France

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