{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T09:48:21Z","timestamp":1785923301784,"version":"3.56.0"},"reference-count":33,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T00:00:00Z","timestamp":1785888000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Foundation Council for Pediatric Nutrition Professionals Research Grant","award":["GRT13019"],"award-info":[{"award-number":["GRT13019"]}]},{"award":["GRT13019"],"award-info":[{"award-number":["GRT13019"]}],"id":[{"id":"https:\/\/ror.org\/005vg3k81","id-type":"ROR","asserted-by":"publisher"}]},{"name":"Racha Khalaf receives funding from the Cystic Fibrosis Foundation","award":["005206Q123"],"award-info":[{"award-number":["005206Q123"]}]},{"name":"Racha Khalaf receives funding from the Cystic Fibrosis Foundation","award":["005583Q123"],"award-info":[{"award-number":["005583Q123"]}]},{"name":"Racha Khalaf receives funding from the Cystic Fibrosis Foundation","award":["009319Y125"],"award-info":[{"award-number":["009319Y125"]}]},{"award":["005206Q123"],"award-info":[{"award-number":["005206Q123"]}],"id":[{"id":"https:\/\/ror.org\/00ax59295","id-type":"ROR","asserted-by":"publisher"}]},{"award":["005583Q123"],"award-info":[{"award-number":["005583Q123"]}],"id":[{"id":"https:\/\/ror.org\/00ax59295","id-type":"ROR","asserted-by":"publisher"}]},{"award":["009319Y125"],"award-info":[{"award-number":["009319Y125"]}],"id":[{"id":"https:\/\/ror.org\/00ax59295","id-type":"ROR","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>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\u201318 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\u2013Wilk 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\u2019s Kappa 0.574; p-value 0.005). In age and sex-adjusted linear regression, BIA fat percentage (\u03b2 = \u22120.23; 95% CI, \u22121.09 to 0.64; p = 0.595) and DXA fat percentage (\u03b2 = \u22120.47; 95% CI, \u22122.14 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.<\/jats:p>","DOI":"10.3390\/nu18152557","type":"journal-article","created":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T09:16:22Z","timestamp":1785921382000},"page":"2557","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Body Composition Assessment in Pediatric Cystic Fibrosis in the Highly Effective Modulator Therapy Era\u2014A Pilot Study"],"prefix":"10.3390","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5660-8862","authenticated-orcid":false,"given":"Michelle","family":"Yavelow","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of South Florida, Tampa, FL 33612, USA"},{"name":"Division of Pediatric Pulmonology, Department of Pediatrics, University of South Florida, Tampa, FL 33620, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3818-8283","authenticated-orcid":false,"given":"Athanasios","family":"Tsalatsanis","sequence":"additional","affiliation":[{"name":"Office of Research, Research Methodology and Biostatistics Core, University of South Florida, Tampa, FL 33612, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisa","family":"Couluris","sequence":"additional","affiliation":[{"name":"Division of Pulmonology, Department of Pediatrics, University of South Florida, Tampa, FL 33612, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danielle","family":"Reynolds","sequence":"additional","affiliation":[{"name":"USF Diabetes and Endocrinology Center, Health Informatics Institute, University of South Florida, Tampa, FL 33612, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5010-8149","authenticated-orcid":false,"given":"Racha T.","family":"Khalaf","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of South Florida, Tampa, FL 33612, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,8,5]]},"reference":[{"key":"ref_1","unstructured":"(2026, May 24). Maintaining Healthy Weight with Cystic Fibrosis. Available online: https:\/\/www.cff.org\/sites\/default\/files\/2021-10\/Maintaining-Healthy-Weight-With-Cystic-Fibrosis.pdf."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.ccm.2007.02.006","article-title":"Nutrition and lung disease in cystic fibrosis","volume":"28","author":"Milla","year":"2007","journal-title":"Clin. Chest Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"e910","DOI":"10.1002\/hsr2.910","article-title":"The relationship between weight and pulmonary outcomes in overweight and obese people with cystic fibrosis: A retrospective observational study","volume":"5","author":"Welter","year":"2022","journal-title":"Health Sci. Rep."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"570","DOI":"10.1016\/j.jaip.2017.07.010","article-title":"Overweight, Obesity, and Lung Function in Children and Adults-A Meta-analysis","volume":"6","author":"Forno","year":"2018","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"The Lancet Diabetes Endocrinology (2022). Childhood obesity: A growing pandemic. Lancet Diabetes Endocrinol., 10, 1.","DOI":"10.1016\/S2213-8587(21)00314-4"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1002\/ppul.23033","article-title":"Overweight and obesity in patients with cystic fibrosis: A center-based analysis","volume":"50","author":"Hanna","year":"2015","journal-title":"Pediatr. Pulmonol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"872","DOI":"10.3945\/ajcn.112.051409","article-title":"Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: A population-based cohort study","volume":"97","author":"Stephenson","year":"2013","journal-title":"Am. J. Clin. Nutr."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1591","DOI":"10.1016\/j.jand.2020.03.015","article-title":"Academy of Nutrition and Dietetics: 2020 Cystic Fibrosis Evidence Analysis Center Evidence-Based Nutrition Practice Guideline","volume":"121","author":"McDonald","year":"2021","journal-title":"J. Acad. Nutr. Diet."},{"key":"ref_9","first-page":"100272","article-title":"Body composition in individuals with cystic fibrosis","volume":"26","author":"Soltman","year":"2021","journal-title":"J. Clin. Transl. Endocrinol."},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Achamrah, N., Colange, G., Delay, J., Rimbert, A., Folope, V., Petit, A., Grigioni, S., Dechelotte, P., and Coeffier, M. (2018). Comparison of body composition assessment by DXA and BIA according to the body mass index: A retrospective study on 3655 measures. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0200465"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2523381","DOI":"10.1080\/15502783.2025.2523381","article-title":"Evolution of research trends and emerging hotspots in bioelectrical impedance analysis over the last two decades: A bibliometric analysis","volume":"22","author":"Niu","year":"2025","journal-title":"J. Int. Soc. Sports Nutr."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1002\/ncp.10247","article-title":"Tools and Methods Used for the Assessment of Body Composition in Patients With Cystic Fibrosis: A Systematic Review","volume":"34","author":"Calella","year":"2019","journal-title":"Nutr. Clin. Pract."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"20420188251328210","DOI":"10.1177\/20420188251328210","article-title":"Cystic fibrosis-related bone disease: An update on screening, diagnosis, and treatment","volume":"16","author":"Cobb","year":"2025","journal-title":"Ther. Adv. Endocrinol. Metab."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/j.nut.2018.03.052","article-title":"Cystic fibrosis, body composition, and health outcomes: A systematic review","volume":"55\u201356","author":"Calella","year":"2018","journal-title":"Nutrition"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1002\/ppul.20162","article-title":"Association of body composition and lung function in children with cystic fibrosis","volume":"39","author":"Pedreira","year":"2005","journal-title":"Pediatr. Pulmonol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e37","DOI":"10.1016\/S2213-2600(16)30188-6","article-title":"Classification of CFTR mutation classes","volume":"4","author":"Marson","year":"2016","journal-title":"Lancet Respir. Med."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2101499","DOI":"10.1183\/13993003.01499-2021","article-title":"ERS\/ATS technical standard on interpretive strategies for routine lung function tests","volume":"60","author":"Stanojevic","year":"2022","journal-title":"Eur. Respir. J."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"e70","DOI":"10.1164\/rccm.201908-1590ST","article-title":"Standardization of Spirometry 2019 Update. An Official American Thoracic Society and European Respiratory Society Technical Statement","volume":"200","author":"Graham","year":"2019","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/j.bjae.2023.07.002","article-title":"Interpretation of pulmonary function tests in children","volume":"23","author":"Miskovic","year":"2023","journal-title":"BJA Educ."},{"key":"ref_20","unstructured":"Tanita (2014). Body Composition Analyzer DC-430U Instruction Manual, Remote Display Version, Tanita Corporation."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1038\/sj.ijo.0803232","article-title":"Body fat reference curves for children","volume":"30","author":"McCarthy","year":"2006","journal-title":"Int. J. Obes."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1093\/ajcn\/72.3.694","article-title":"Healthy percentage body fat ranges: An approach for developing guidelines based on body mass index","volume":"72","author":"Gallagher","year":"2000","journal-title":"Am. J. Clin. Nutr."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1016\/j.jcf.2022.01.011","article-title":"Bone mineral density screening by DXA for people with cystic fibrosis: A registry analysis of patient and program factors influencing rates of screening","volume":"21","author":"Ratti","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"Alvarez Merino, M., Cardenes, C.M.L., Cavia, S.M., Rubio, E.T., Rodriguez-Martinez, A., Santamaria, S.V., Torne, C.V., Beltran, M.A., Galindo, C.G., and Garriga Garcia, M. (2025). Impact on Body Composition After Two Years of Elexacaftor-Tezacaftor-Ivacaftor Therapy in Children with Cystic Fibrosis. Children, 12.","DOI":"10.3390\/children12121598"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1016\/j.nut.2015.10.012","article-title":"Body composition and lung function in cystic fibrosis and their association with adiposity and normal-weight obesity","volume":"32","author":"Alvarez","year":"2016","journal-title":"Nutrition"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1016\/j.jcf.2019.01.002","article-title":"Visceral adipose tissue is associated with poor diet quality and higher fasting glucose in adults with cystic fibrosis","volume":"18","author":"Bellissimo","year":"2019","journal-title":"J. Cyst. Fibros."},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Sheikh, S., Zemel, B.S., Stallings, V.A., Rubenstein, R.C., and Kelly, A. (2014). Body composition and pulmonary function in cystic fibrosis. Front. Pediatr., 2.","DOI":"10.3389\/fped.2014.00033"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1016\/j.ajcnut.2025.05.022","article-title":"Methodological standards for body composition-an expert-endorsed guide for research and clinical applications: Levels, models, and terminology","volume":"122","author":"Prado","year":"2025","journal-title":"Am. J. Clin. Nutr."},{"key":"ref_29","unstructured":"Shypailo, R.J. (2026, May 24). Age- and Height-Based Pediatric DXA Reference Charts. Available online: https:\/\/www.bcm.edu\/bodycomplab\/DXAapp\/DXA-calculator-kids.html."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"e13041","DOI":"10.1111\/obr.13041","article-title":"Assessment of body composition in pediatric overweight and obesity: A systematic review of the reliability and validity of common techniques","volume":"21","author":"Orsso","year":"2020","journal-title":"Obes. Rev."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1016\/j.jada.2008.02.020","article-title":"Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: Results of a systematic review","volume":"108","author":"Stallings","year":"2008","journal-title":"J. Am. Diet. Assoc."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1016\/j.jcf.2021.12.014","article-title":"Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis","volume":"21","author":"Madde","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"S16","DOI":"10.1016\/S1569-1993(11)60004-0","article-title":"European cystic fibrosis bone mineralisation guidelines","volume":"10","author":"Bianchi","year":"2011","journal-title":"J. Cyst. Fibros."}],"container-title":["Nutrients"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6643\/18\/15\/2557\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T09:33:13Z","timestamp":1785922393000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6643\/18\/15\/2557"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,5]]},"references-count":33,"journal-issue":{"issue":"15","published-online":{"date-parts":[[2026,8]]}},"alternative-id":["nu18152557"],"URL":"https:\/\/doi.org\/10.3390\/nu18152557","relation":{},"ISSN":["2072-6643"],"issn-type":[{"value":"2072-6643","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,5]]}}}