{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T09:33:56Z","timestamp":1777541636506,"version":"3.51.4"},"reference-count":46,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T00:00:00Z","timestamp":1648512000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJERPH"],"abstract":"<jats:p>Cystic fibrosis (CF) is the most common autosomal recessive inherited monogenic disease in Caucasians. As medical technology progresses and the quality of patient care improves, the survival time of patients with CF has increased, which results in more frequent comorbidities such as cystic fibrosis-related diabetes (CFRD). CFRD is the result of abnormal glucose metabolism characterized primarily by insulin deficiency, exacerbated periodically by insulin resistance. The aim of our study was to analyze the epidemiology of patients with CFRD in Poland on the basis of data collected from six CF treatment centers. Analyses were performed on 1157 CF patients who were treated at one of the six CF care centers. CFRD was diagnosed according to standard criteria. All data including demographics, types of CFTR mutations, CFRD duration, and microorganisms in the sputum were obtained from the patients\u2019 medical history. Our study indicates that the prevalence of CFRD in Poland is 12.9%. CFRD was most often diagnosed between the ages of 11 and 20 (60% of patients), while 23% of patients were diagnosed between 21 and 30 years of age. Furthermore, we observed that approximately 3\u20135% of patients under the age of 10 had CFRD. We found out that the type of mutation did not affect the frequency of CFRD development. Factors that increased the risk of developing CFRD include underweight and chronic Pseudomonas aeruginosa infection. Due to the extended lifespan of CF patients, the number of CFRD patients is currently increasing. We believe that the results of our study may complement information from other studies or may be useful in planning health policy in Poland.<\/jats:p>","DOI":"10.3390\/ijerph19074069","type":"journal-article","created":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T21:44:52Z","timestamp":1648590292000},"page":"4069","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Cystic Fibrosis-Related Diabetes in Poland"],"prefix":"10.3390","volume":"19","author":[{"given":"Marta","family":"Rachel","sequence":"first","affiliation":[{"name":"Institute of Medical Sciences, Medical College, Rzesz\u00f3w University, Warzywna 1a, 35-310 Rzesz\u00f3w, Poland"},{"name":"Department of Allergology and Cystic Fibrosis, State Hospital 2 in Rzeszow, Lwowska 60, 35-301 Rzesz\u00f3w, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marek","family":"Biesiadecki","sequence":"additional","affiliation":[{"name":"Institute of Medical Sciences, Medical College, Rzesz\u00f3w University, Warzywna 1a, 35-310 Rzesz\u00f3w, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1129-3553","authenticated-orcid":false,"given":"Sabina","family":"Galiniak","sequence":"additional","affiliation":[{"name":"Institute of Medical Sciences, Medical College, Rzesz\u00f3w University, Warzywna 1a, 35-310 Rzesz\u00f3w, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,3,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Scotet, V., Gutierrez, H., and Farrell, P.M. (2020). Newborn screening for CF across the globe\u2014Where is it worthwhile?. Int. J. Neonatal Screen., 6.","DOI":"10.3390\/ijns6010018"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"10660","DOI":"10.1038\/s41598-020-67353-3","article-title":"The changing demography of the cystic fibrosis population: Forecasting future numbers of adults in the UK","volume":"10","author":"Keogh","year":"2020","journal-title":"Sci. Rep."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Rachel, M., Topolewicz, S., \u015aliwczy\u0144ski, A., and Galiniak, S. (2020). Managing Cystic Fibrosis in Polish Healthcare. Int. J. Environ. Res. Public Health, 17.","DOI":"10.3390\/ijerph17207630"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1016\/j.jcf.2017.11.013","article-title":"Epidemiology of CF: How registries can be used to advance our understanding of the CF population","volume":"17","author":"Jackson","year":"2018","journal-title":"J. Cyst. Fibros."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1016\/j.rmed.2008.10.011","article-title":"Growing old with cystic fibrosis\u2014The characteristics of long-term survivors of cystic fibrosis","volume":"103","author":"Simmonds","year":"2009","journal-title":"Respir. Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1016\/j.prrv.2013.01.007","article-title":"Ageing in cystic fibrosis and long-term survival","volume":"14","author":"Simmonds","year":"2013","journal-title":"Paediatr. Respir. Rev."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Scotet, V., L\u2019Hostis, C., and F\u00e9rec, C. (2020). The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery. Genes, 11.","DOI":"10.3390\/genes11060589"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.jcf.2019.10.009","article-title":"Cystic fibrosis related diabetes in Europe: Prevalence, risk factors and outcome; Olesen et al","volume":"19","author":"Olesen","year":"2020","journal-title":"J. Cyst. Fibros."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1515\/jpem-2019-0484","article-title":"Cystic fibrosis-related diabetes: An update on pathophysiology, diagnosis, and treatment","volume":"33","author":"Alves","year":"2020","journal-title":"J. Pediatr. Endocrinol. Metab."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"213","DOI":"10.4239\/wjd.v11.i6.213","article-title":"Cystic fibrosis-related diabetes: The unmet need","volume":"11","author":"Pozo","year":"2020","journal-title":"World J. Diabetes"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1016\/j.jpeds.2004.12.039","article-title":"Epidemiology of cystic fibrosis-related diabetes","volume":"146","author":"Marshall","year":"2005","journal-title":"J. Pediatr."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1111\/j.1651-2227.2007.00250.x","article-title":"Liver disease as risk factor for cystic fibrosis-related diabetes development","volume":"96","author":"Minicucci","year":"2007","journal-title":"Acta Paediatr."},{"key":"ref_13","first-page":"100290","article-title":"Cystic fibrosis-related diabetes: Prevalence, screening, and diagnosis","volume":"27","author":"Khare","year":"2021","journal-title":"J. Clin. Transl. Endocrinol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1007\/s00408-020-00396-5","article-title":"Influence of Cystic Fibrosis-Related Diabetes on Mental Health in Adults: A Single-Center Study","volume":"198","author":"Hjelm","year":"2020","journal-title":"Lung"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1016\/j.jcf.2019.12.016","article-title":"Glycemic control and FEV1 recovery during pulmonary exacerbations in pediatric cystic fibrosis-related diabetes","volume":"19","author":"Okoniewski","year":"2020","journal-title":"J. Cyst. Fibros."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"2697","DOI":"10.2337\/dc10-1768","article-title":"Clinical care guidelines for cystic fibrosis-related diabetes: A position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society","volume":"33","author":"Moran","year":"2010","journal-title":"Diabetes Care"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1111\/pedi.12732","article-title":"ISPAD Clinical Practice Consensus Guidelines 2018: Management of cystic fibrosis-related diabetes in children and adolescents","volume":"19","author":"Moran","year":"2018","journal-title":"Pediatr. Diabetes"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1617","DOI":"10.2337\/dci20-0022","article-title":"Precision Medicine in Diabetes: A Consensus Report From the American Diabetes Association (ADA) and the European Association for the Study of Diabetes (EASD)","volume":"43","author":"Chung","year":"2020","journal-title":"Diabetes Care"},{"key":"ref_19","unstructured":"Zolin, A., Orenti, A., Naehrlich, L., and van Rens, J. (2017). ECFSPR Annual Report 2017, European Cystic Fibrosis Society. Available online: https:\/\/www.ecfs.eu\/sites\/default\/files\/general-content-images\/working-groups\/ecfs-patient-registry\/ECFSPR_Report2017_v1.3.pdf."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3310\/hta16240","article-title":"Screening for cystic fibrosis-related diabetes: A systematic review","volume":"16","author":"Waugh","year":"2012","journal-title":"Health Technol. Assess."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"545","DOI":"10.2147\/DMSO.S180597","article-title":"Cystic fibrosis-related diabetes: Optimizing care with a multidisciplinary approach","volume":"12","author":"Frost","year":"2019","journal-title":"Diabetes Metab. Syndr. Obes. Targets Ther."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2413","DOI":"10.1002\/ppul.25463","article-title":"The associations between pediatric weight status and cystic fibrosis-related diabetes status and health-related quality of life among children and young adults with cystic fibrosis: A systematic review","volume":"56","author":"Gomes","year":"2021","journal-title":"Pediatr. Pulmonol."},{"key":"ref_23","first-page":"124","article-title":"Cystic fibrosis-related diabetes (CFRD) and cognitive function in adults with cystic fibrosis","volume":"S1569\u20131993","author":"Chadwick","year":"2021","journal-title":"J. Cyst. Fibros."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1038\/s41436-020-01073-x","article-title":"Cystic fibrosis-related diabetes onset can be predicted using biomarkers measured at birth","volume":"23","author":"Lin","year":"2021","journal-title":"Genet. Med."},{"key":"ref_25","first-page":"dgac020","article-title":"New concepts in the pathogenesis of cystic fibrosis-related diabetes","volume":"2","author":"Moheet","year":"2022","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_26","first-page":"632","article-title":"Ultrasound findings of pubertal development in girls with cystic fibrosis and their association with clinical outcomes and Tanner staging","volume":"65","author":"Machado","year":"2021","journal-title":"Arch. Endocrinol. Metab."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"2631","DOI":"10.1007\/s00125-012-2621-2","article-title":"Prevalence of monogenic diabetes amongst Polish children after a nationwide genetic screening campaign","volume":"55","author":"Fendler","year":"2012","journal-title":"Diabetologia"},{"key":"ref_28","unstructured":"(2021, June 12). Cystic Fibrosis Foundation Patient Registry 2013 Annual Data Report to the Center Directors Bethesda, Maryland. Available online: https:\/\/www.cff.org\/2013_cff_annual_data_report_to_the_center_directors.pdf."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"S32","DOI":"10.1016\/j.jcf.2019.08.011","article-title":"Cystic fibrosis related diabetes: Nutrition and growth considerations","volume":"18","author":"Kaminski","year":"2019","journal-title":"J. Cyst. Fibros."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"e78","DOI":"10.2337\/dc12-0459","article-title":"Glucose derangements in very young children with cystic fibrosis and pancreatic insufficiency","volume":"35","author":"Mozzillo","year":"2012","journal-title":"Diabetes Care"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/1099-0496(200101)31:1<1::AID-PPUL1000>3.0.CO;2-T","article-title":"European Epidemiologic Registry of Cystic Fibrosis (ERCF): Comparison of major disease manifestations between patients with different classes of mutations","volume":"31","author":"Koch","year":"2001","journal-title":"Pediatr. Pulmonol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"2114","DOI":"10.2337\/db13-1464","article-title":"Evidence for a causal relationship between early exocrine pancreatic disease and cystic fibrosis-related diabetes: A Mendelian randomization study","volume":"63","author":"Soave","year":"2014","journal-title":"Diabetes"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"20","DOI":"10.3389\/fendo.2018.00020","article-title":"Cystic Fibrosis-Related Diabetes","volume":"9","author":"Kayani","year":"2018","journal-title":"Front. Endocrinol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1164\/rccm.201403-0576OC","article-title":"Diabetes-related mortality in adults with cystic fibrosis. Role of genotype and sex","volume":"191","author":"Lewis","year":"2015","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1515\/jpem-2017-0005","article-title":"Cystic-fibrosis related-diabetes (CFRD) is preceded by and associated with growth failure and deteriorating lung function","volume":"30","author":"Terliesner","year":"2017","journal-title":"J. Pediatr. Endocrinol. Metab."},{"key":"ref_36","first-page":"422","article-title":"Association of the relative change in weight and body mass index with lung function in teenagers and adults with cystic fibrosis: Influence of gender and diabetes","volume":"62","year":"2015","journal-title":"Endocrinol. Nutr."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"3217","DOI":"10.1002\/ppul.25572","article-title":"Nutritional status between 5-10 years is associated with cystic fibrosis-related diabetes in adolescence","volume":"56","author":"Ararat","year":"2021","journal-title":"Pediatr. Pulmonol."},{"key":"ref_38","unstructured":"(2021, May 20). Consortium of the Cystic Fibrosis Genetic Analysis Consortium. Available online: http:\/\/www.genet.sickkids.on.ca\/cftr."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"P75","DOI":"10.1055\/s-0035-1549581","article-title":"Association of CFTR mutations with Cystic Fibrosis related Diabetes (CFRD) in Germany and Austria: A multicentre analysis from the DPV registry","volume":"10","author":"Laubner","year":"2015","journal-title":"Diabetol. Stoffwechs."},{"key":"ref_40","first-page":"246","article-title":"Insulin Production and Resistance in Cystic Fibrosis: Effect of Age, Disease Activity, and Genotype","volume":"35","author":"Street","year":"2012","journal-title":"J. Endocrinol. Investig."},{"key":"ref_41","doi-asserted-by":"crossref","unstructured":"Iafusco, F., Maione, G., Rosanio, F.M., Mozzillo, E., Franzese, A., and Tinto, N. (2021). Cystic Fibrosis-Related Diabetes (CFRD): Overview of Associated Genetic Factors. Diagnostics, 1.","DOI":"10.3390\/diagnostics11030572"},{"key":"ref_42","first-page":"83","article-title":"Cystic fibrosis-related diabetes in adults","volume":"99","author":"Cawood","year":"2006","journal-title":"Ir. Med. J."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1016\/j.jcf.2008.05.008","article-title":"Microvascular complications in patients with cystic fibrosis-related diabetes (CFRD)","volume":"7","author":"Morton","year":"2008","journal-title":"J. Cyst. Fibros."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"2042018820966428","DOI":"10.1177\/2042018820966428","article-title":"Retinopathy and microalbuminuria are common microvascular complications in cystic fibrosis-related diabetes","volume":"11","author":"Kempegowda","year":"2020","journal-title":"Ther. Adv. Endocrinol. Metab."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1007\/s00592-015-0743-y","article-title":"Screening of glucose metabolism derangements in pediatric cystic fibrosis patients: How, when, why","volume":"52","author":"Franzese","year":"2015","journal-title":"Acta Diabetol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1187","DOI":"10.1007\/s13300-017-0289-9","article-title":"Long-Term Follow-Up in a Girl with Cystic Fibrosis and Diabetes Since the First Year of Life","volume":"8","author":"Fattorusso","year":"2017","journal-title":"Diabetes Ther."}],"container-title":["International Journal of Environmental Research and Public Health"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1660-4601\/19\/7\/4069\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:45:58Z","timestamp":1760136358000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1660-4601\/19\/7\/4069"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3,29]]},"references-count":46,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2022,4]]}},"alternative-id":["ijerph19074069"],"URL":"https:\/\/doi.org\/10.3390\/ijerph19074069","relation":{},"ISSN":["1660-4601"],"issn-type":[{"value":"1660-4601","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,3,29]]}}}