{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T09:03:25Z","timestamp":1780045405653,"version":"3.53.1"},"reference-count":22,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2024,6,22]],"date-time":"2024-06-22T00:00:00Z","timestamp":1719014400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Over the past decades, efforts have been made to improve the nutritional well-being of people with cystic fibrosis (pwCF). Due to the correlation observed between nutritional indices and lung function, prevailing recommendations consistently advocate for BMI percentile goals at or above the 50th percentile in pwCF. Recent global trends show a notable increase in overweight and obese statuses among pwCF. This study aims to explore the nutritional status of Italian pwCF. Methods: Data from the Italian CF Patient\u2019s Registry were analysed to assess the proportion of individuals categorized as underweight, target weight, overweight, and obese from 2010 to 2021. Patient-level comparison data from 2021 were also examined to identify the potential determinants of overweight and obesity. Results: Analysis spanning 2010 to 2021 reveals a decrease of approximately 40% in underweight status among adults, while the proportion of malnourished patients younger than 18 years remained stable. Conversely, there was a substantial increase of over 70% in overweight status and over 85% in obesity among adults, with minor fluctuations observed among children and adolescents. Patient factors associated with increased obesity incidence included age older than 45 years, male gender, pancreatic sufficiency, possession of at least one CFTR variant conferring residual function, ppFEV1 &gt; 90, and lower prevalence of Pseudomonas aeruginosa colonization. Conclusions: Our study confirms the evolving nutritional status landscape among Italian adult pwCF, with a significant shift towards overweight and obesity over the past decade. These trends highlight the need for proactive measures within CF standards of care to adapt and address the changing needs of patients.<\/jats:p>","DOI":"10.3390\/jcm13133652","type":"journal-article","created":{"date-parts":[[2024,6,24]],"date-time":"2024-06-24T06:59:58Z","timestamp":1719212398000},"page":"3652","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Nutritional Trends in Cystic Fibrosis: Insights from the Italian Cystic Fibrosis Patient Registry"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2432-415X","authenticated-orcid":false,"given":"Donatello","family":"Salvatore","sequence":"first","affiliation":[{"name":"Cystic Fibrosis Centre, Hospital San Carlo, 85100 Potenza, Italy"},{"name":"Scientific Board, Italian CF Registry, 00100 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0624-8125","authenticated-orcid":false,"given":"Rita","family":"Padoan","sequence":"additional","affiliation":[{"name":"Scientific Board, Italian CF Registry, 00100 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annalisa","family":"Amato","sequence":"additional","affiliation":[{"name":"Scientific Board, Italian CF Registry, 00100 Rome, Italy"},{"name":"Italian Cystic Fibrosis League, Charity Programme, 00162 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6402-2610","authenticated-orcid":false,"given":"Marco","family":"Salvatore","sequence":"additional","affiliation":[{"name":"Scientific Board, Italian CF Registry, 00100 Rome, Italy"},{"name":"Undiagnosed Rare Diseases Interdepartmental Unit, Istituto Superiore di Sanit\u00e0, National Center Rare Diseases, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1884-9171","authenticated-orcid":false,"given":"Giuseppe","family":"Campagna","sequence":"additional","affiliation":[{"name":"Scientific Board, Italian CF Registry, 00100 Rome, Italy"},{"name":"Italian Cystic Fibrosis League, Charity Programme, 00162 Rome, Italy"},{"name":"Department of Medical-Surgical Sciences and Translational Medicine, University of Rome Sapienza, 00161 Rome, Italy"},{"name":"Department of Clinical and Molecular Medicine, University of Rome Sapienza, 00189 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"on behalf of the Italian CF Registry Working Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1177\/0884533613507086","article-title":"Malnutrition in cystic fibrosis: A review","volume":"28","author":"Culhane","year":"2013","journal-title":"Nutr. Clin. Pract."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1016\/j.clnu.2023.12.017","article-title":"ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis","volume":"43","author":"Wilschanski","year":"2024","journal-title":"Clin. Nutr."},{"key":"ref_3","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_4","first-page":"1","article-title":"Rapporto 2019\u20132020 [Italian Cystic Fibrosis Registry (ICFR). Report 2019\u20132020]","volume":"46","author":"Campagna","year":"2022","journal-title":"Epidemiol. Prev."},{"key":"ref_5","unstructured":"Cystic Fibrosis Foundation (2022). Patient Registry 2021 Annual Data Report, Cystic Fibrosis Foundation."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1016\/j.jcf.2022.09.001","article-title":"Trajectories of early growth and subsequent lung function in cystic fibrosis: An observational study using UK and Canadian registry data","volume":"22","author":"Macdougall","year":"2023","journal-title":"J. Cyst. Fibros."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"596","DOI":"10.1136\/thorax.57.7.596","article-title":"Relationship between nutritional status and lung function in cystic fibrosis: Cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project","volume":"57","author":"Steinkamp","year":"2002","journal-title":"Thorax"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1016\/j.jcf.2022.03.010","article-title":"Obesity in cystic fibrosis: Prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry","volume":"21","author":"Szentpetery","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.jcf.2021.05.002","article-title":"Overweight and obesity in adults with cystic fibrosis: An Italian multicenter cohort study","volume":"21","author":"Gramegna","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1016\/S0140-6736(23)01609-4","article-title":"CFTR modulator therapy: Transforming the landscape of clinical care in cystic fibrosis","volume":"402","author":"Robinson","year":"2023","journal-title":"Lancet"},{"key":"ref_11","unstructured":"Body Mass Index (BMI) (2023, October 11). Centers for Disease Control and Prevention. CDC 2022, Available online: https:\/\/www.cdc.gov\/healthyweight\/assessing\/bmi\/index.html."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1002\/ppul.24215","article-title":"Patients with cystic fibrosis having a residual function mutation: Data from the Italian registry","volume":"54","author":"Salvatore","year":"2019","journal-title":"Pediatr. Pulmonol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"234","DOI":"10.1016\/j.jcf.2022.07.015","article-title":"Disease severity of people with cystic fibrosis carrying residual function mutations: Data from the ECFS Patient Registry","volume":"22","author":"Orenti","year":"2023","journal-title":"J. Cyst. Fibros."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Bass, R., Brownell, J.N., and Stallings, V.A. (2021). The impact of highly effective CFTR modulators on growth and nutrition status. Nutrients, 13.","DOI":"10.3390\/nu13092907"},{"key":"ref_15","first-page":"1","article-title":"Inequality in overweight and obesity among adults in Italy: Trends and geographic disparities with a focus on Campania Region","volume":"4","author":"Masocco","year":"2023","journal-title":"Boll. Epidemiol. Naz."},{"key":"ref_16","unstructured":"Cystic Fibrosis Canada (2023). The Canadian Cystic Fibrosis Registry 2021 Annual Data Report, Cystic Fibrosis Canada."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"100690","DOI":"10.1016\/j.lanepe.2023.100690","article-title":"Impact of elexacaftor\/tezacaftor\/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: Real-world evidence from the German CF Registry","volume":"32","author":"Sutharsan","year":"2023","journal-title":"Lancet Reg. Health Eur."},{"key":"ref_18","unstructured":"Zolin, A., Orenti, A., Jung, A., and van Rens, J. (2023). ECFSPR Annual Report 2021, ECFSPR."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1016\/j.jcf.2021.11.008","article-title":"Forewarned is forearmed: The cardiovascular time bomb in Cystic Fibrosis","volume":"21","author":"Duckers","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1038\/s44161-022-00030-y","article-title":"Identifying and preventing cardiovascular disease in patients with cystic fibrosis","volume":"1","author":"Saunders","year":"2022","journal-title":"Nat. Cardiovasc. Res."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1591","DOI":"10.1016\/j.jand.2020.03.015","article-title":"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_22","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.jcf.2023.12.002","article-title":"Standards for the care of people with cystic fibrosis; establishing and maintaining health","volume":"23","author":"Southern","year":"2024","journal-title":"J. Cyst. Fibros."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/13\/3652\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T15:03:00Z","timestamp":1760108580000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/13\/3652"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,22]]},"references-count":22,"journal-issue":{"issue":"13","published-online":{"date-parts":[[2024,7]]}},"alternative-id":["jcm13133652"],"URL":"https:\/\/doi.org\/10.3390\/jcm13133652","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,22]]}}}