Cystic Fibrosis
Conditions
Keywords
cystic fibrosis, KAFTRIO, renal impact, lithiasis
Brief summary
The objective of this study is to describe the renal impact of Elexacaftor-Teacaftor-Ivacaftor, a triple modulator therapy of CFTR channel, in patients with cystic fibrosis. This new treatment acts on the CFTR channel, which is expressed at the level of the nephrons. The objective is to study the changes in plasma and urinary parameters, including metabolic explorations of urolithiasis, change in volemic parameters, renal function, urinary sediment and nutritional and glycemic parameters, in newly treated patients, through the data collected at introduction of the treatment and during the follow-up.
Interventions
BIOLOGICALEvaluation of the parameters of the lithiasis assessment before and after KAFTRIO initiation
Collection of Uricuria, phosphaturie, calciuria, magnesuria, glycosuria, citraturia, oxaluria, before and after initiation of KAFTRIO
DRUGCollection of Arterial pressure, metabolic data, kidney function, proteinuria and hematuria before and after KATRIO initiation
Arterial pressure, glycemia, glycated hemoglobin, lipid profile, weight, serum and urinary electrolytes, hepatic check, complete blood count, calcium phosphate balance and urinary sediment analysis before and after initiation of KAFTRIO
Sponsors
Hospices Civils de Lyon
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Inclusion criteria
* Patient with cystic fibrosis * More than 18 years old * Eligible for KAFTRIO
Exclusion criteria
* KAFTRIO contraindication * Patient less than 18 years old * Dialysis patient * Pregnant woman * Greffed patient * Patient opposition to data collection
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Modification of the parameters of the lithiasis assessment | 1 month after initiation of KAFTRIO | Change in blood phosphorus and calcium metabolism ; Change in calciuria mmol/d ;Change in urinary pH, urinary specific gravity (g/mL) and crystalluria |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Change in volemic parameter | 1 month after initiation of KAFTRIO | body mass index (BMI) (kg/m²), systolic and diastolic blood pressure (mmHg), presence of orthostatic hypotension, renin and aldosterone levels and ratio (ng/L and pmol/L respectively), natremia (mmol/L), natriuresis (mmol/d), kalemia (mmol/L), kaliuresis (mmol/d), chloremia (mmol/L), bicarbonatemia (mmol/L), brain natriuretic peptid (ng/L), hemoglobin (g/L) and sodium intake estimated by the dietician and calculated thanks to natriuresis (g/d) |
| Change in renal function | 1 month after initiation of KAFTRIO | (measured using creatinine (µmol/L) and cystatin C (mg/L)combined to report 2021 CKD-EPIcreatinineand CKD-EPIcreatinine-cystatin C formulas) and in plasma urea levels (mmol/L). |
| Change in urinary sediment | 1 month after initiation of KAFTRIO | , represented by proteinuria (g/day), albuminuria/creatininuria ratio (mg/g) and urinary beta-2-microglobulin level (mg/day), after collecting 24-hours urine sa |
| Change in glycemic profile | 1 month after initiation of KAFTRIO | (fasting blood glucose level (mmol/L) and glycated hemoglobin (%)) and nutritional profile (albumin and pre-albumin in g/L). |
| Change in lung function | 1 month after initiation of KAFTRIO | predicted forced expiratory volume in 1 second (FEV1) (%) and peak expiratory flow (%)). |
Countries
France
Contacts
Primary ContactEtienne NOVEL CATIN
Outcome results
None listed