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Optimizing Nutritional Strategies for Cystic Fibrosis: Mechanisms, Interventions, and Clinical Outcomes

A Special Issue of Nutrients (ISSN 2072-6643) belonging to the section "Clinical Nutrition".

Deadline for manuscript submissions: 15 January 2027 | Viewed by 1574

Editors

Prof. Dr. Maria R. Mascarenhas

E-Mail Website
Guest Editor
Division of Gastroenterology, Hepatology, and Nutrition, Children’s Hospital of Philadelphia, Philadelphia, PA 19104, USA
Interests: cystic fibrosis; gastroenterology; nutrition, irritable bowel syndrome; 22q deletion syndrome, pediatric; integrative health, wellness education
Special Issues, Collections and Topics in MDPI journals
Dr. Jessica Alvarez

E-Mail Website
Guest Editor
Division of Endocrinolog, Liipds, and Metabolism, Department of Medicine, Emory University School of Medicine, Atlanta, GA, USA
Interests: cystic fibrosis; diet; body composition; metabolomics; endocrine; obesity; clinical nutrition; nutrition assessment
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Nutrition in cystic fibrosis (CF) is undergoing a paradigm shift as highly effective CFTR modulator therapies (HEMT) redefine clinical management. Historically, emphasis was placed on hypercaloric, high‑fat diets to mitigate malabsorption and elevated energy expenditure, with pancreatic enzyme replacement therapy (PERT), sodium supplementation, and fat‑soluble vitamins constituting the cornerstone of nutritional care. Malnutrition and impaired growth trajectories were primary concerns. With HEMT improving pulmonary function, reducing exacerbations, and extending survival, the nutritional landscape now includes the rising prevalence of overweight and obesity. Consequently, traditional anthropometric targets, such as weight‑for‑length and body mass index (BMI), require reevaluation. Emerging evidence underscores the prognostic significance of lean body mass, necessitating refined methodologies for body composition assessment and a deeper understanding of the metabolic sequelae of excess adiposity.

Advances in microbiome science highlight the potential role of dietary modulation, including anti‑inflammatory diet, in optimizing physical and mental health outcomes. Enhanced screening for disordered eating behaviors is also warranted to delineate their prevalence in CF populations. Moreover, the impact of HEMT on pancreatic function, nutrient absorption, PERT dosing, electrolyte requirements, micronutrient status, and skeletal health must be systematically investigated. Finally, cystic fibrosis-related diabetes (CFRD) persists as a major complication, requiring nuanced strategies to balance nutritional adequacy with glycemic control.

Prof. Dr. Maria R. Mascarenhas
Dr. Jessica Alvarez
Guest Editors

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Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • cystic fibrosis
  • nutrition
  • gastroenterology
  • eating disorders
  • HEMT
  • CFRD
  • bone health
  • PERT
  • microbiome
  • body composition
  • pancreas
  • vitamins
  • salt
  • diet
  • mental health

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Published Papers (3 papers)

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Research

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18 pages, 460 KB  
Article
Evaluating Dietary Assessment Methods in Adults with Cystic Fibrosis in the Modulator Era: A Comparison of Food Frequency Questionnaires and Three-Day Food Diaries
by Cian Greaney, Caoimhe Egan, Deanna Kenny, Sarah Tecklenborg, Ciara Howlett, Karen Cronin, Clodagh Landers, Mary Connolly, Derbhla O’Sullivan, Katie Robinson and Audrey Tierney
Nutrients 2026, 18(18), 3002; https://doi.org/10.3390/nu18183002 - 14 Sep 2026
Abstract
Background/Objectives: With advances in treatment, nutritional priorities in Cystic Fibrosis (CF) have shifted towards long-term metabolic health. However, uncertainty remains regarding whether commonly used dietary assessment methods provide comparable estimates of dietary intake during this transition. This study aims to compare and evaluate [...] Read more.
Background/Objectives: With advances in treatment, nutritional priorities in Cystic Fibrosis (CF) have shifted towards long-term metabolic health. However, uncertainty remains regarding whether commonly used dietary assessment methods provide comparable estimates of dietary intake during this transition. This study aims to compare and evaluate agreement between dietary intakes derived from food frequency questionnaires (FFQs) and three-day food diaries in adults with CF. Methods: Cross-sectional study of Irish adults with CF. Semi-quantitative FFQs and three-day food diaries gathered dietary data. Results: Forty-four participants were included (variant-specific therapies: 81.8%). No significant differences were observed between methods for energy, carbohydrate, protein, or total fat intake (p > 0.05). However, significant differences were identified for several nutrients and food groups. Compared with the three-day food diary, the FFQ reported higher intakes of total sugar, percentage energy from saturated fat, monounsaturated fat, polyunsaturated fat, sodium, zinc, and vitamin E, but lower intakes of fibre and vitamin D (p < 0.05). Associations between methods ranged from weak to strong, with the strongest observed for fibre (r = 0.701). There were wide limits of agreement for many dietary variables (e.g., energy: −1335.3 to 1242.3 kcal/day), despite comparable group-level estimates. Both methods identified poor adherence to dietary recommendations. Conclusions: The FFQ and three-day food diary yielded broadly similar estimates in adults with CF. However, substantial individual-level variability and poor agreement for several nutrients and food groups suggest these methods are not interchangeable for individual dietary assessment. While three-day food diaries may be preferable when detailed assessment of current intake is required, FFQs remain useful for characterising habitual dietary intake in epidemiological and population-based research. Full article
(This article belongs to the Special Issue Optimizing Nutritional Strategies for Cystic Fibrosis: Mechanisms, Interventions, and Clinical Outcomes)
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15 pages, 2332 KB  
Article
Body Composition Assessment in Pediatric Cystic Fibrosis in the Highly Effective Modulator Therapy Era—A Pilot Study
by Michelle Yavelow, Athanasios Tsalatsanis, Marisa Couluris, Danielle Reynolds and Racha T. Khalaf
Nutrients 2026, 18(15), 2557; https://doi.org/10.3390/nu18152557 - 5 Aug 2026
Viewed by 438
Abstract
Background/Objectives: The prevalence of obesity in cystic fibrosis (CF) has increased following the introduction of highly effective modulator therapy (HEMT). However, the impact of increased adiposity on the lung function of pediatric patients with CF (ppwCF) in the era of HEMT remains incompletely [...] Read more.
Background/Objectives: The prevalence of obesity in cystic fibrosis (CF) has increased following the introduction of highly effective modulator therapy (HEMT). However, the impact of increased adiposity on the lung function of pediatric patients with CF (ppwCF) in the era of HEMT remains incompletely understood. Furthermore, while the body mass index (BMI) is the most commonly used nutritional marker in CF care, it has important limitations and may not accurately reflect body composition. Methods: In this cross-sectional pilot study, ppwCF aged 5–18 years completed Bioelectrical Impedance Analysis (BIA) while a subset completed a dual-energy X-ray absorptiometry (DXA) scan and spirometry. Normality was assessed using Shapiro–Wilk tests. Correlations, categorical association, agreement tests, Firth penalized logistic regression, and exploratory linear regression of continuous FEV1% predicted were used to examine relationships among anthropometric measurements, body compositions, and pulmonary function. Results: Thirty-four individuals completed study procedures (19 female (56%); mean age 9.85 (SD 3.58)). BIA and DXA-derived body fat percentages were strongly correlated, and categorical agreement was moderate (Cohen’s Kappa 0.574; p-value 0.005). In age and sex-adjusted linear regression, BIA fat percentage (β = −0.23; 95% CI, −1.09 to 0.64; p = 0.595) and DXA fat percentage (β = −0.47; 95% CI, −2.14 to 1.21; p = 0.552) were not significantly associated with FEV1% predicted. Similarly, Firth logistic models showed no statistically significant association between the adiposity category and reduced lung function. Conclusions: BIA-derived body fat measurements correlate with DXA-derived measurements in ppwCF and demonstrate feasibility for use in routine CF clinic visits. This pilot cohort did not show a statistically significant relationship between higher adiposity and poorer pulmonary function. However, the wide confidence intervals indicate substantial uncertainty. Larger prospective studies are needed to evaluate longitudinal relationships between body composition and lung function in the HEMT era. Full article
(This article belongs to the Special Issue Optimizing Nutritional Strategies for Cystic Fibrosis: Mechanisms, Interventions, and Clinical Outcomes)
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Review

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21 pages, 1261 KB  
Review
Vitamin A Status in Cystic Fibrosis in the Current Era of CFTR-Directed Therapies
by Senthilkumar Sankararaman, Terri Schindler, Kay Vavrina and Maria Mascarenhas
Nutrients 2026, 18(16), 2639; https://doi.org/10.3390/nu18162639 - 12 Aug 2026
Viewed by 642
Abstract
Vitamin A plays an important role in multiple homeostatic functions such as vision, immunity, epithelial cell integrity and differentiation, cell signaling, pulmonary function, reproduction, growth, and development. Vitamin A deficiency was described in people with cystic fibrosis (pwCF) due to a multitude of [...] Read more.
Vitamin A plays an important role in multiple homeostatic functions such as vision, immunity, epithelial cell integrity and differentiation, cell signaling, pulmonary function, reproduction, growth, and development. Vitamin A deficiency was described in people with cystic fibrosis (pwCF) due to a multitude of causes, such as suboptimally managed exocrine pancreatic insufficiency, advanced cystic fibrosis-related liver disease (aCFLD), and a history of intestinal resection, and is generally rare in contemporary practice. Apart from true vitamin A deficiency, low vitamin A levels may also be noted in various inflammatory states, as vitamin A is a negative acute-phase reactant. In the current era of cystic fibrosis (CF) transmembrane-conductance regulator (CFTR)-directed therapies, there is a paradigm shift in vitamin A status, with deficiency statuses becoming rarer, and instead higher serum vitamin levels (in some cases, even in the hypervitaminosis range) are increasingly reported. People with aCFLD, renal insufficiency, post-lung transplantation, and pregnancy are prone to vitamin A toxicity. Hence, CF clinicians should be proactive in evaluating these abnormalities and proficient in managing both deficiency and toxicity, as both these conditions can be associated with adverse outcomes. In this review, we detailed the basics of vitamin A metabolism, manifestations of both vitamin A deficiency and excess, and their clinical implications in pwCF. Full article
(This article belongs to the Special Issue Optimizing Nutritional Strategies for Cystic Fibrosis: Mechanisms, Interventions, and Clinical Outcomes)
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