Overweight and obesity in adults with cystic fibrosis: An Italian multicenter cohort study

Gramegna, Andrea; Aliberti, Stefano; Contarini, Martina; Savi, Daniela; Sotgiu, Giovanni; Saderi, Laura; Lucidi, V.; Amati, Francesco; Pappalettera, Maria; Palange, Paolo; Blasi, Francesco

doi:10.1016/j.jcf.2021.05.002

Cited by 29 publications

(35 citation statements)

References 16 publications

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“…A similar increase in BMI after using CFTR modulators in severely affected pwCF was described in patients with the F/MF genotype [22] and in patients with mutations determining CFTR residual function [23]. Although over the last decades an emphasis has been on promoting BMI gain to optimise pulmonary outcomes, the risk of overweightness/obesity in an adult population should be monitored in the long-term [24][25][26].…”

Section: Discussionmentioning

confidence: 55%

Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study

Carnovale

Iacotucci

Terlizzi

et al. 2022

JCM

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Background: Elexacaftor/tezacaftor/ivacaftor (ETI) is the newest cystic fibrosis transmembrane conductance regulator (CFTR) modulator drug approved for the treatment of patients with cystic fibrosis (pwCF) aged ≥6 years with at least one copy of the F508del mutation (F) in the CFTR gene or another mutation that is responsive to treatment with ETI. This study determined the effectiveness and safety of ETI in a cohort of severely affected pwCF with an F/F genotype. Methods: Retrospective observational study in F/F pwCF treated for 48 weeks, enrolled in an ETI managed access program available to subjects with advanced lung disease (ppFEV1 < 40). Twenty-six patients from three centres were included. The main outcomes included lung function, sweat chloride concentration (SCC), nutrition, frequency of pulmonary exacerbations (PEx), CFQ-R, and safety. Results: ppFEV1 improved by 12.06 (95%CI 8.54, 15.57) from baseline after 4 weeks of treatment with ETI, 15.32 (11.3, 19.34) after 24 weeks, and 14.48 (10.64, 18.32) after 48 weeks. The increase in FEV1 was accompanied by a decrease in SCC, improvement of BMI, and noticeable reduction in PEx. An overall good safety profile was observed. Conclusions: In F/F pwCF with advanced lung disease with an F/F genotype, ETI was safe and associated with clinical improvement.

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Section: Discussionmentioning

confidence: 55%

Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study

Carnovale

Iacotucci

Terlizzi

et al. 2022

JCM

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“…The proportion of patients with CF who are overweight or obese is increasing. While patients who are overweight or obese are reported to have better lung function than their normal weight or underweight counterparts in some studies, this finding may be confounded by the fact that these patients are also more likely to be pancreatic sufficient and have milder disease genotypes [ 118 , 119 , 120 , 121 , 122 ]. Additionally, weight gain and increased BMI, fat mass, and fat-free mass are reported outcomes of CFTR modulator therapies that need to be taken into account as modulator therapies gradually become the cornerstone of CF treatment [ 123 , 124 , 125 , 126 ].…”

Section: Nutritional Management In Cfmentioning

confidence: 99%

What Do We Know about the Microbiome in Cystic Fibrosis? Is There a Role for Probiotics and Prebiotics?

2022

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Cystic fibrosis (CF) is a life-shortening genetic disorder that affects the cystic fibrosis transmembrane conductance regulator (CFTR) protein. In the gastrointestinal (GI) tract, CFTR dysfunction results in low intestinal pH, thick and inspissated mucus, a lack of endogenous pancreatic enzymes, and reduced motility. These mechanisms, combined with antibiotic therapies, drive GI inflammation and significant alteration of the GI microbiota (dysbiosis). Dysbiosis and inflammation are key factors in systemic inflammation and GI complications including malignancy. The following review examines the potential for probiotic and prebiotic therapies to provide clinical benefits through modulation of the microbiome. Evidence from randomised control trials suggest probiotics are likely to improve GI inflammation and reduce the incidence of CF pulmonary exacerbations. However, the highly variable, low-quality data is a barrier to the implementation of probiotics into routine CF care. Epidemiological studies and clinical trials support the potential of dietary fibre and prebiotic supplements to beneficially modulate the microbiome in gastrointestinal conditions. To date, limited evidence is available on their safety and efficacy in CF. Variable responses to probiotics and prebiotics highlight the need for personalised approaches that consider an individual’s underlying microbiota, diet, and existing medications against the backdrop of the complex nutritional needs in CF.

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“…Increasing age is associated with rising BMI among the general population and has been found to predict obesity in some CF studies as well [8] , [9] , [14] . Interestingly this was not found in the Italian cohort, suggesting cultural factors may influence obesity risk factors across the globe [15] .…”

Section: Epidemiologymentioning

confidence: 73%

“…Pancreatic insufficiency impacts approximately 85% of people with CF and has long been associated with malnutrition, so may also result in lower risk for obesity [5] . In fact, multiple studies find higher rates of exocrine pancreatic sufficiency and mild genotypes among overweight and obese cohorts [8] , [9] , [11] , [14] , [15] . Nevertheless, pancreatic insufficient individuals with CF are not immune to overnutrition.…”

Section: Epidemiologymentioning

confidence: 99%

Obesity in cystic fibrosis

Kutney

Sandouk

Desimone

et al. 2021

Journal of Clinical & Translational Endocrinology

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The prevalence of obesity in patients with cystic fibrosis (CF) is increasing and around one-third of adults with CF are now overweight or obese. The causes of excess weight gain in CF are likely multifactorial, including: adherence to the high-fat legacy diet, reduced exercise tolerance, therapeutic advances, and general population trends. Increased weight has generally been considered favorable in CF, correlating with improved pulmonary function and survival. While the optimal BMI for overall health in CF is unknown, most studies demonstrate minimal improvement in pulmonary function when BMI exceeds 30 kg/m 2 . Dyslipidemia and cardiovascular disease are important co-morbidities of obesity in the general population, but are uncommon in CF. In people with CF, obesity is associated with hypertension and higher cholesterol levels. With longer life expectancy and rising obesity rates, there may be an increase in cardiovascular disease among people with CF in coming years. Overweight CF patients are more likely to be insulin resistant, taking on features of type 2 diabetes. Treating obesity in people with CF requires carefully weighing the metabolic risks of overnutrition with the impact of low or falling BMI on lung function. This article describes current knowledge on the epidemiology, causes, consequence, and treatment of obesity in people with CF.

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Overweight and obesity in adults with cystic fibrosis: An Italian multicenter cohort study

Cited by 29 publications

References 16 publications

Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study

Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study

What Do We Know about the Microbiome in Cystic Fibrosis? Is There a Role for Probiotics and Prebiotics?

Obesity in cystic fibrosis

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