Predicting weight gain in patients with cystic fibrosis on triple combination modulator.
Pediatr Pulmonol
; 59(6): 1724-1730, 2024 Jun.
Article
de En
| MEDLINE
| ID: mdl-38607242
ABSTRACT
BACKGROUND:
Cystic fibrosis (CF) is caused by CF transmembrane conductance regulator (CFTR) gene mutations producing dysfunctional CFTR proteins leading to progressive clinical disease. Elexacaftor-tezacaftor-ivacaftor (ETI) remarkably improves lung disease but is associated with substantial weight gain. STUDY DESIGN ANDMETHODS:
We performed a single-center longitudinal study predicting 6-month weight gain after ETI initiation. We used linear mixed effects modeling (LME) to determine association of ETI treatment with changing body mass index (BMI). Using linear regression, we examined BMI prediction models with distinct combinations of main effects to identify a model useful for patient counseling. We used up to eight commonly observed clinical characteristics as input variables (age, sex, percent predicted FEV1 [FEV1%], F508del homozygous state, pancreatic sufficiency, HgbA1c, prior modulator use and prior year number of pulmonary exacerbations).RESULTS:
We evaluated 154 patients (19-73 years old, 54% female, FEV1% = 19-121, 0-6 prior year pulmonary exacerbations). LME demonstrated an association between ETI use and weight increases. Exhaustive testing suggested a parsimonious linear regression model well-fitted to data that is potentially useful for counseling. The two variable model shows that on average, BMI decreases by 0.045 (95% Confidence Interval [CI] = -0.069 to -0.021, p < 0.001) for every year of age and increases by 0.322 (CI = 0.142 to 0.502, p = 0.001) for each additional prior year exacerbation at the time of ETI initiation.INTERPRETATION:
Young patients with many prior year pulmonary exacerbations likely have the largest 6 month weight gain after starting ETI.Mots clés
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Prise de poids
/
Indice de masse corporelle
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Mucoviscidose
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Association médicamenteuse
/
Aminophénols
/
Indoles
Limites:
Adult
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Aged
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Female
/
Humans
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Male
/
Middle aged
Langue:
En
Journal:
Pediatr Pulmonol
Sujet du journal:
PEDIATRIA
Année:
2024
Type de document:
Article
Pays d'affiliation:
États-Unis d'Amérique