ePS05.3 Ivacaftor and its effects on body composition in adults with G551D related cystic fibrosis

Tierney, Audrey C.; Edgeworth, D.; Williams, E.; Finlayson, F.; Keating, D.; Clark, D.; Button, Brenda; Kotsimbos, Tom; Wilson, John W.

doi:10.1016/s1569-1993(15)30162-4

Cited by 3 publications

(5 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Ten studies (8.1%) included both children and adults (range: 4–66 years, N = 768). Six studies (published as abstracts) did not report information on the age of the patients (Tables , , and ). Thirty‐one studies (published as abstracts) had a cross‐sectional design, whereas 9 studies were longitudinal with a follow‐up duration between 3 months and 10 years.…”

Section: Resultsmentioning

confidence: 99%

“…Six studies (published as abstracts) did not report information on the age of the patients (Tables , , and ). Thirty‐one studies (published as abstracts) had a cross‐sectional design, whereas 9 studies were longitudinal with a follow‐up duration between 3 months and 10 years. The 84 articles (full texts) included only 9 studies with a longitudinal design with a follow‐up duration between 1 and 4 years (Tables and ).…”

Section: Resultsmentioning

confidence: 99%

“…20,22,[25][26][27][28][29][30][31][32][33][34][35][36][37] Ten studies (8.1%) included both children and adults 20,22,[28][29][30]33,35,[38][39][40] (range: 4-66 years, N = 768). Six studies (published as abstracts) did not report information on the age of the patients 15,[41][42][43][44][45] (Tables 2, 3, and 4). Thirty-one studies 37 Canada CS 34 20-54 a BIA-DXA ADP, air displacement plethysmography; BIA, bioelectric impedance analysis; C, control group; CF, cystic fibrosis; CS, cross-sectional; CT, computed tomography; DD, deuterium dilution; DXA, dual-energy X-ray absorptiometry; H 2 18 O, water isotope; L, longitudinal; MRI, magnetic resonance imaging; NIR, near-infrared interactance; NR, not reported; pQCT, peripheral quantitative computed tomography; SF, skinfolds; TBCl, total body chloride; TBK, total body potassium; TOBEC, total body electrical conductivity; ULT, ultrasound; U, densitometry by underwater weighing.…”

Section: Sample Size and Study Designmentioning

confidence: 99%

“…b Age media. c Age median (published as abstracts) had a cross-sectional design, whereas 9 studies 16,21,34,43,44,[46][47][48][49] were longitudinal with a follow-up duration between 3 months and 10 years. The 84 articles (full texts) included only 9 studies with a longitudinal design with a follow-up duration between 1 and 4 years 23,24,26,[50][51][52][53][54][55] (Tables 2 and 3).…”

Section: Sample Size and Study Designmentioning

confidence: 99%

See 3 more Smart Citations

Tools and Methods Used for the Assessment of Body Composition in Patients With Cystic Fibrosis: A Systematic Review

et al. 2019

View full text Add to dashboard Cite

Background Cystic fibrosis (CF) is characterized by changes in fat mass and lean body mass that may have important prognostic value. We aim to appraise the type and frequency of application of body composition (BC) methods in child and adult patients with CF. Methods We used 4 databases (Embase, PubMed, Scopus, and Web of Science) to perform the literature search. The search was conducted from January 2017 to February 2017. Two independent reviewers selected articles based on titles and abstracts to check eligibility for inclusion. All study designs or types of articles (abstract, full text) were considered. Results Eighty‐four full‐text articles and 40 studies presented only as abstracts were selected. Sixty‐four studies included children and adolescents (age range of 0.1–18 years), and 41 studies recruited adults (range of 18–57 years); 13 studies included both age groups. Dual‐energy X‐ray absorptiometry (DXA) was used in 56 studies (33.9%), and bioelectric impedance analysis (BIA) was used in 12 studies (9.7%), whereas 38 studies (30.6%) combined different methods (up to 5 different methods) to assess BC. Conclusions The results show a large variability in the application of BC methods in patients with CF that makes the comparison between studies difficult. The only methods with a sufficient body of literature are DXA and BIA.

show abstract

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Sample Size and Study Designmentioning

confidence: 99%

Section: Sample Size and Study Designmentioning

confidence: 99%

See 2 more Smart Citations

Tools and Methods Used for the Assessment of Body Composition in Patients With Cystic Fibrosis: A Systematic Review

et al. 2019

View full text Add to dashboard Cite

show abstract

“…Interventions, such as nutritional rehabilitation have been shown to lead to improvements in weight ( Levy et al, 1985 ); however, less is known about the impact of dietary modifications on the body composition of these patients. Similarly, little is known about the long term impact of CFTR modulators, such as ivacaftor on body composition ( Tierney et al, 2015 ; Stallings et al, 2018 ).…”

Section: Introductionmentioning

confidence: 99%

Weight increase in people with cystic fibrosis on CFTR modulator therapy is mainly due to increase in fat mass

et al. 2023

View full text Add to dashboard Cite

Background: Ivacaftor, the first CFTR modulator drug, leads to significant long-term improvement in lung function and weight gain. The mechanism as well as the long-term impact of ivacaftor on weight, resting energy expenditure (REE) and body composition remains to be explored.Methods: This prospective observational study included 18 people with CF (pwCF) (age: median (range) 20 (6–58) years) carrying at least one CFTR gating mutation commencing ivacaftor. Assessments of body composition, REE and laboratory investigations were performed at baseline and 6, 12 and 24 months after treatment initiation.Results: Treatment with ivacaftor was associated with a significantly positive change in BMI z-score at 24 months. Fat mass (mean (95% CL) of 6.5 kg (4.0; 9.0) from baseline, p = 0.0001), but not fat-free mass changed under ivacaftor treatment. There was a significant positive correlation between weight and fat mass change. Overall, there was no significant change in measured REE from baseline (mean (95% CL) of 108 kcal/d (−12; 228), p = 0.07) in our cohort. Pancreatic function and other nutritional markers did not change with treatment, with the exception of an increase in serum vitamin A levels (p = 0.006).Conclusion: The weight gain observed in ivacaftor treated pwCF is predominantly secondary to increases in fat mass warranting early counseling of people starting on CFTR-modulating treatment with respect to healthy diet and physical exercise.

show abstract

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis

Skilton

Krishan

Patel

et al. 2019

Cochrane Database of Systematic Reviews

View full text Add to dashboard Cite

show abstract

ePS05.3 Ivacaftor and its effects on body composition in adults with G551D related cystic fibrosis

Cited by 3 publications

References 0 publications

Tools and Methods Used for the Assessment of Body Composition in Patients With Cystic Fibrosis: A Systematic Review

Tools and Methods Used for the Assessment of Body Composition in Patients With Cystic Fibrosis: A Systematic Review

Weight increase in people with cystic fibrosis on CFTR modulator therapy is mainly due to increase in fat mass

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis

Contact Info

Product

Resources

About