Emerging Nonpulmonary Complications for Adults With Cystic Fibrosis

Chin, Melanie; Brennan, Amanda; Bell, S. C.

doi:10.1016/j.chest.2021.11.001

Cited by 4 publications

(3 citation statements)

References 127 publications

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“…It directly contributes to the pathophysiology of coronary arteria disease through inflammatory processes and endothelial damage 27 . Thus, EAT could serve as a biomarker for cardiovascular risk in the ageing population of pwCF 28 . We observed a significant decline of muscle and TAT ratios in one patient, who later died due to hemorrhagic shock and acute-on-chronic liver failure.…”

Section: Discussionmentioning

confidence: 99%

Elexacaftor/tezacaftor/ivacaftor influences body composition in adults with cystic fibrosis: a fully automated CT-based analysis

Westhölter,

Haubold,

Welsner

et al. 2024

Sci Rep

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A poor nutritional status is associated with worse pulmonary function and survival in people with cystic fibrosis (pwCF). CF transmembrane conductance regulator modulators can improve pulmonary function and body weight, but more data is needed to evaluate its effects on body composition. In this retrospective study, a pre-trained deep-learning network was used to perform a fully automated body composition analysis on chest CTs from 66 adult pwCF before and after receiving elexacaftor/tezacaftor/ivacaftor (ETI) therapy. Muscle and adipose tissues were quantified and divided by bone volume to obtain body size-adjusted ratios. After receiving ETI therapy, marked increases were observed in all adipose tissue ratios among pwCF, including the total adipose tissue ratio (+ 46.21%, p < 0.001). In contrast, only small, but statistically significant increases of the muscle ratio were measured in the overall study population (+ 1.63%, p = 0.008). Study participants who were initially categorized as underweight experienced more pronounced effects on total adipose tissue ratio (p = 0.002), while gains in muscle ratio were equally distributed across BMI categories (p = 0.832). Our findings suggest that ETI therapy primarily affects adipose tissues, not muscle tissue, in adults with CF. These effects are primarily observed among pwCF who were initially underweight. Our findings may have implications for the future nutritional management of pwCF.

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Section: Discussionmentioning

confidence: 99%

Elexacaftor/tezacaftor/ivacaftor influences body composition in adults with cystic fibrosis: a fully automated CT-based analysis

Westhölter,

Haubold,

Welsner

et al. 2024

Sci Rep

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“…Progressive pulmonary disease remains the dominant cause of morbidity and mortality. Advances in CF care, including but not limited to the advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, have resulted in increased life expectancy and improved quality of life 1,2 . However, alongside these welcome advantages are emerging challenges associated with the rising prevalence and appreciation of extra‐pulmonary CF complications and age‐associated co‐morbidities.…”

Section: Introductionmentioning

confidence: 99%

“…Advances in CF care, including but not limited to the advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, have resulted in increased life expectancy and improved quality of life. 1,2 However, alongside these welcome advantages are emerging challenges associated with the rising prevalence and appreciation of extra-pulmonary CF complications and age-associated co-morbidities. The proportion of patients with cystic fibrosis (pWCF) with extrapulmonary complications is rising, and care will shift from preventing mortality to preventing morbidity.…”

mentioning

confidence: 99%

SHIFTing goals in cystic fibrosis—managing extrapulmonary disease in the era of CFTR modulator therapy; Proceedings of the International Shaping Initiatives and Future Trends (SHIFT) Symposium

O'Donnell,

Hastings,

Briody

et al. 2024

Pediatric Pulmonology

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BackgroundCystic fibrosis (CF) is a life‐shortening multisystem genetic disease. Although progressive pulmonary disease is the predominant cause of morbidity and mortality, improvements in treatment for CF‐related lung disease, with associated increase in longevity, have increased the prevalence of extrapulmonary manifestations1.MethodsTo discuss these issues, a multidisciplinary meeting of international leaders and experts in the field was convened in November 2021 at the Shaping Initiatives and Future Trends Symposium with the goal of highlighting shifting management paradigms in CF. The main topics covered were: (1) nutrition and obesity, (2) exocrine pancreas, (3) CF‐related diabetes, (4) CF liver disease, (5) CF‐related bone disease, and (6) post‐lung transplant care. This document summarizes the proceedings, highlighting the key priorities and important research questions that were discussed.ResultsImproved life expectancy, the advent of cystic fibrosis transmembrane conductance regulator modulators, and the increasing appreciation of the heterogeneity or spectrum of disease are leading to a shift in management for patients with cystic fibrosis. Care should be individualized to ensure that increased longevity is accompanied by improved extra‐pulmonary care and reduced morbidity.

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Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy

Hisert,

Birket,

Clancy

et al. 2023

The Lancet Respiratory Medicine

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Emerging Nonpulmonary Complications for Adults With Cystic Fibrosis

Cited by 4 publications

References 127 publications

Elexacaftor/tezacaftor/ivacaftor influences body composition in adults with cystic fibrosis: a fully automated CT-based analysis

Elexacaftor/tezacaftor/ivacaftor influences body composition in adults with cystic fibrosis: a fully automated CT-based analysis

SHIFTing goals in cystic fibrosis—managing extrapulmonary disease in the era of CFTR modulator therapy; Proceedings of the International Shaping Initiatives and Future Trends (SHIFT) Symposium

Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy

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