{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T22:30:30Z","timestamp":1789425030347,"version":"build-2803163510"},"reference-count":88,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2023,6,22]],"date-time":"2023-06-22T00:00:00Z","timestamp":1687392000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>The course of cystic fibrosis (CF) as a nutritional illness is diverging since the introduction of highly effective modulator therapy, leading to more heterogeneous phenotypes of the disease despite CF genetic mutations that portend worse prognosis. This may become more evident as we follow the pediatric CF population into adulthood as some highly effective modulator therapies (HEMT) are approved for those as young as 1 year old. This review will outline the current research and knowledge available in the evolving nutritional health of people with CF as it relates to the impact of HEMT on anthropometrics, body composition, and energy expenditure, exocrine and endocrine pancreatic insufficiencies (the latter resulting in CF-related diabetes), vitamin and mineral deficiencies, and nutritional health in CF as it relates to pregnancy and lung transplantation.<\/jats:p>","DOI":"10.3390\/life13071431","type":"journal-article","created":{"date-parts":[[2023,6,23]],"date-time":"2023-06-23T01:54:56Z","timestamp":1687485296000},"page":"1431","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Evolving Nutritional Needs in Cystic Fibrosis"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8282-7682","authenticated-orcid":false,"given":"Theresa","family":"Frantzen","sequence":"first","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Medicine, Donald and Barbara Zucker School of Medicine at Hofstra\/Northwell, New Hyde Park, New York, NY 11042, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2364-4493","authenticated-orcid":false,"given":"Sara","family":"Barsky","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, The Steven and Alexandra Cohen Children\u2019s Medical Center, Donald and Barbara Zucker School of Medicine at Hofstra\/Northwell, Lake Success, New York, NY 11042, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geralyn","family":"LaVecchia","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Medicine, Donald and Barbara Zucker School of Medicine at Hofstra\/Northwell, New Hyde Park, New York, NY 11042, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Marowitz","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, The Steven and Alexandra Cohen Children\u2019s Medical Center, Donald and Barbara Zucker School of Medicine at Hofstra\/Northwell, Lake Success, New York, NY 11042, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janice","family":"Wang","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Medicine, Donald and Barbara Zucker School of Medicine at Hofstra\/Northwell, New Hyde Park, New York, NY 11042, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,6,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1001\/archpedi.1946.02020300069004","article-title":"Celiac Syndrome: V. Genetics of Cystic Fibrosis of the Pancreas with a Consideration of Etiology","volume":"72","author":"Andersen","year":"1946","journal-title":"Am. J. Dis. Child."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1055\/s-0029-1238916","article-title":"Nutrition in cystic fibrosis","volume":"30","author":"Matel","year":"2009","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1111\/apm.12915","article-title":"Cystic fibrosis\u2014An example of personalized and precision medicine","volume":"127","author":"Skov","year":"2019","journal-title":"APMIS Acta Pathol. Microbiol. Immunol. Scand."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1364.e2","DOI":"10.1016\/j.jand.2020.03.014","article-title":"Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic Review","volume":"121","author":"Bailey","year":"2021","journal-title":"J. Acad. Nutr. Diet."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Carnovale, V., Iacotucci, P., Terlizzi, V., Colangelo, C., Ferrillo, L., Pepe, A., Francalanci, M., Taccetti, G., Buonaurio, S., and Celardo, A. (2022). Elexacaftor\/Tezacaftor\/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study. J. Clin. Med., 11.","DOI":"10.3390\/jcm11041021"},{"key":"ref_6","unstructured":"Cystic Fibrosis Foundation (2022). Patient Registry 2021 Annual Data Report, Cystic Fibrosis Foundation."},{"key":"ref_7","unstructured":"(2023, April 26). FDA Approves Trikafta for Children Ages 2 through 5 Years with Certain CF Mutations. Available online: https:\/\/www.cff.org\/news\/2023-04\/trikafta-approval-ages-2-5-mutations."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1164\/rccm.202011-4153OC","article-title":"Rapid Improvement after Starting Elexacaftor-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease","volume":"204","author":"Burgel","year":"2021","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_9","unstructured":"(2023, April 26). A Prospective Study to Evaluate Biological and Clinical Effects of Significantly Corrected CFTR Function, Available online: https:\/\/clinicaltrials.gov\/ct2\/show\/NCT04038047."},{"key":"ref_10","first-page":"S1569","article-title":"Elexacaftor\/tezacaftor\/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI","volume":"22","author":"Schwarzenberg","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e21","DOI":"10.1016\/S2213-2600(16)30032-7","article-title":"Partial restoration of pancreatic function in a child with cystic fibrosis","volume":"4","author":"Howlett","year":"2016","journal-title":"Lancet Respir. Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1016\/j.jcf.2021.08.032","article-title":"Restoration of exocrine pancreatic function in child with lumacaftor\/ivacaftor therapy in cystic fibrosis","volume":"21","author":"Crowley","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_13","first-page":"99","article-title":"Restoration of exocrine pancreatic function in older children with cystic fibrosis on ivacaftor","volume":"35","author":"Nichols","year":"2020","journal-title":"Paediatr. Respir. Rev."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1164\/rccm.202102-0509OC","article-title":"A Phase 3 Open-Label Study of Elexacaftor\/Tezacaftor\/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One F508del Allele","volume":"203","author":"Zemanick","year":"2021","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/j.jcf.2021.11.012","article-title":"Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis","volume":"21","author":"Petersen","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1164\/rccm.202110-2249OC","article-title":"Effects of Elexacaftor\/Tezacaftor\/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles","volume":"205","author":"Graeber","year":"2022","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1002\/ppul.26261","article-title":"Impact of elexacaftor\u2013Tezacaftor\u2013Ivacaftor on bacterial colonization and inflammatory responses in cystic fibrosis","volume":"58","author":"Sheikh","year":"2022","journal-title":"Pediatr. Pulmonol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1016\/j.jcf.2021.09.001","article-title":"The effect of elexacaftor\/tezacaftor\/ivacaftor (ETI) on glycemia in adults with cystic fibrosis","volume":"21","author":"Scully","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_19","unstructured":"(2023, April 26). Strength and Muscle Related Outcomes for Nutrition and Lung Function in CF, Available online: https:\/\/clinicaltrials.gov\/ct2\/show\/NCT05639556."},{"key":"ref_20","unstructured":"Wang, Y., and Chen, H.J. (2012). Handbook of Anthropometry: Physical Measures of Human Form in Health and Disease, Springer."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1016\/j.jcf.2022.08.018","article-title":"Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor\/tezacaftor\/ivacaftor","volume":"22","author":"Fajac","year":"2023","journal-title":"J. Cyst. Fibros."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1016\/S0140-6736(19)32597-8","article-title":"Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: A double-blind, randomised, phase 3 trial","volume":"394","author":"Heijerman","year":"2019","journal-title":"Lancet"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1164\/rccm.202108-1986OC","article-title":"Clinical Effectiveness of Elexacaftor\/Tezacaftor\/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial","volume":"205","author":"Nichols","year":"2022","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"2003079","DOI":"10.1183\/13993003.03079-2020","article-title":"Efficacy of elexacaftor\/tezacaftor\/ivacaftor in patients with cystic fibrosis and advanced lung disease","volume":"57","author":"Carroll","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1016\/S2213-2600(21)00454-9","article-title":"Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: A 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial","volume":"10","author":"Sutharsan","year":"2022","journal-title":"Lancet Respir. Med."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1002\/ppul.26243","article-title":"Effect of Trikafta on bone density, body composition and exercise capacity in CF: A pilot study","volume":"58","author":"Gur","year":"2023","journal-title":"Pediatr. Pulmonol."},{"key":"ref_28","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_29","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_30","doi-asserted-by":"crossref","first-page":"2652","DOI":"10.1002\/ppul.26078","article-title":"Elexacaftor-Tezacaftor-Ivacaftor improves exercise capacity in adolescents with cystic fibrosis","volume":"57","author":"Causer","year":"2022","journal-title":"Pediatr. Pulmonol."},{"key":"ref_31","first-page":"9","article-title":"Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators","volume":"42","author":"Konrad","year":"2020","journal-title":"Paediatr. Respir. Rev."},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Tabori, H., Arnold, C., Jaudszus, A., Mentzel, H.-J., Renz, D.M., Reinsch, S., Lorenz, M., Michl, R., Gerber, A., and Lehmann, T. (2017). Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings. PLoS ONE, 12.","DOI":"10.1371\/journal.pone.0174463"},{"key":"ref_33","first-page":"CD008227","article-title":"Pancreatic enzyme replacement therapy for people with cystic fibrosis","volume":"2020","author":"Somaraju","year":"2020","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/j.1365-2036.2010.04437.x","article-title":"Review article: Enzyme supplementation in cystic fibrosis, chronic pancreatitis, pancreatic and periampullary cancer","volume":"32","author":"Imrie","year":"2010","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/j.jcf.2021.02.003","article-title":"Promise: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy","volume":"20","author":"Nichols","year":"2021","journal-title":"J. Cyst. Fibros."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1016\/j.pan.2019.09.014","article-title":"Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis","volume":"19","author":"Akshintala","year":"2019","journal-title":"Pancreatology"},{"key":"ref_37","doi-asserted-by":"crossref","unstructured":"Timoneda, J., Rodr\u00edguez-Fern\u00e1ndez, L., Zaragoz\u00e1, R., Mar\u00edn, M.P., Cabezuelo, M.T., Torres, L., Vi\u00f1a, J.R., and Barber, T. (2018). Vitamin A Deficiency and the Lung. Nutrients, 10.","DOI":"10.3390\/nu10091132"},{"key":"ref_38","doi-asserted-by":"crossref","unstructured":"Bonifant, C.M., Shevill, E., and Chang, A.B. (2012). Vitamin A supplementation for cystic fibrosis. Cochrane Database Syst. Rev., CD006751.","DOI":"10.1002\/14651858.CD006751.pub3"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1055\/s-0039-1696726","article-title":"Growth and Nutrition in Cystic Fibrosis","volume":"40","author":"Brownell","year":"2019","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1007\/978-3-030-46227-7_2","article-title":"Sunlight, UV Radiation, Vitamin D, and Skin Cancer: How Much Sunlight Do We Need?","volume":"1268","author":"Holick","year":"2020","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_41","first-page":"29","article-title":"Effectiveness of vitamin D2 compared with vitamin D3 replacement therapy in a primary healthcare setting: A retrospective cohort study","volume":"2022","author":"Albarri","year":"2022","journal-title":"Qatar Med. J."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1177\/000456328602300505","article-title":"The use of different lipids to express serum tocopherol: Lipid ratios for the measurement of vitamin E status","volume":"23","author":"Thurnham","year":"1986","journal-title":"Ann. Clin. Biochem."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/S1569-1993(02)00032-2","article-title":"Nutrition in patients with cystic fibrosis: A European Consensus","volume":"1","author":"Sinaasappel","year":"2002","journal-title":"J. Cyst. Fibros."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1S","DOI":"10.1378\/chest.125.1_suppl.1S","article-title":"Cystic fibrosis adult care: Consensus conference report","volume":"125","author":"Yankaskas","year":"2004","journal-title":"Chest"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1080\/21548331.2021.1905413","article-title":"A retrospective evaluation of vitamin K for hemoptysis in adult cystic fibrosis patients","volume":"49","author":"Gavioli","year":"2021","journal-title":"Hosp. Pract."},{"key":"ref_46","doi-asserted-by":"crossref","unstructured":"Sommerburg, O., H\u00e4mmerling, S., Schneider, S.P., Okun, J., Langhans, C.-D., Leutz-Schmidt, P., Wielp\u00fctz, M.O., Siems, W., Gr\u00e4ber, S.Y., and Mall, M.A. (2021). CFTR Modulator Therapy with Lumacaftor\/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis. Antioxidants, 10.","DOI":"10.3390\/antiox10030483"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"e94","DOI":"10.1097\/MPG.0000000000003606","article-title":"Community Perspective on Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis","volume":"75","author":"Borowitz","year":"2022","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1002\/ncp.10899","article-title":"Update on the management of vitamins and minerals in cystic fibrosis","volume":"37","author":"Sankararaman","year":"2022","journal-title":"Nutr. Clin. Pract."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1626","DOI":"10.2337\/dc09-0586","article-title":"Cystic fibrosis-related diabetes: Current trends in prevalence, incidence, and mortality","volume":"32","author":"Moran","year":"2009","journal-title":"Diabetes Care"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/S2213-8587(13)70015-9","article-title":"New insights into cystic fibrosis-related diabetes in children","volume":"1","author":"Ode","year":"2013","journal-title":"Lancet Diabetes Endocrinol."},{"key":"ref_51","first-page":"100301","article-title":"The effect of cystic fibrosis transmembrane conductance regulator modulators on impaired glucose tolerance and cystic fibrosis related diabetes","volume":"29","author":"Hasan","year":"2022","journal-title":"J. Clin. Transl. Endocrinol."},{"key":"ref_52","first-page":"100286","article-title":"The role of modulators in cystic fibrosis related diabetes","volume":"27","author":"Merjaneh","year":"2022","journal-title":"J. Clin. Transl. Endocrinol."},{"key":"ref_53","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_54","unstructured":"Ballamann, M. (2021). Cystic Fibrosis\u2014Facts, Management and Advances, BoD\u2013Books on Demand."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"852551","DOI":"10.3389\/fped.2022.852551","article-title":"Short-Term Effects of Elexacaftor\/Tezacaftor\/Ivacaftor Combination on Glucose Tolerance in Young People with Cystic Fibrosis\u2014An Observational Pilot Study","volume":"10","author":"Korten","year":"2022","journal-title":"Front. Pediatr."},{"key":"ref_56","first-page":"100311","article-title":"Glycemia and \u03b2-cell function before and after elexacaftor\/tezacaftor\/ivacaftor in youth and adults with cystic fibrosis","volume":"30","author":"Chan","year":"2022","journal-title":"J. Clin. Transl. Endocrinol."},{"key":"ref_57","first-page":"98","article-title":"Dietary interventions for managing glucose abnormalities in people with cystic fibrosis","volume":"7","author":"Birch","year":"2018","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"2495","DOI":"10.1016\/j.clnu.2019.11.004","article-title":"Dietary intake in cystic fibrosis and its role in glucose metab olism","volume":"39","author":"Armaghanian","year":"2020","journal-title":"Clin. Nutr."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1249\/MSS.0000000000001104","article-title":"Combined exercise training improves glycemic control in adult with cystic fibrosis","volume":"49","author":"Beaudoin","year":"2017","journal-title":"Med. Sci. Sports Exerc."},{"key":"ref_60","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_61","unstructured":"Prentice, B., Nicholson, M., and Lam, G.Y. (2022). Cystic fibrosis related diabetes (CFRD) in the era of modulators: A scoping review. Paediatr. Respir. Rev., S1526\u2013S1542."},{"key":"ref_62","doi-asserted-by":"crossref","unstructured":"Patel, D., Shan, A., Mathews, S., and Sathe, M. (2022). Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management. Nutrients, 14.","DOI":"10.3390\/nu14051028"},{"key":"ref_63","doi-asserted-by":"crossref","unstructured":"Staufer, K. (2020). Current Treatment Options for Cystic Fibrosis-Related Liver Disease. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21228586"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"S1","DOI":"10.1097\/00005176-199900001-00001","article-title":"Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group","volume":"28","author":"Sokol","year":"1999","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1002\/hep.26370","article-title":"The nutritional management of hepatic encephalopathy in patients with cirrhosis: International Society for Hepatic Encephalopathy and Nitrogen Metabolism Consensus","volume":"58","author":"Amodio","year":"2013","journal-title":"Hepatology"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"761","DOI":"10.4254\/wjh.v11.i12.761","article-title":"Lumacaftor\/ivacaftor therapy is associated with reduced hepatic steatosis in cystic fibrosis patients","volume":"11","author":"Kutney","year":"2019","journal-title":"World J. Hepatol."},{"key":"ref_67","first-page":"317","article-title":"Cystic fibrosis and calcium oxalate nephrolithiasis","volume":"69","author":"Chidekel","year":"1996","journal-title":"Yale J. Biol. Med."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1693","DOI":"10.1089\/end.2021.0151","article-title":"Systematic Review of the Prevalence of Kidney Stones in Cystic Fibrosis","volume":"35","author":"Moryousef","year":"2021","journal-title":"J. Endourol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1097\/JU.0000000000002331","article-title":"Incidence of Kidney Stones in the United States: The Continuous National Health and Nutrition Examination Survey","volume":"207","author":"Hill","year":"2022","journal-title":"J. Urol."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1870","DOI":"10.1093\/ndt\/gfl067","article-title":"Factors increasing the risk for stone formation in adult patients with cystic fibrosis","volume":"21","author":"Terribile","year":"2006","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"S92","DOI":"10.1016\/S1569-1993(21)01613-1","article-title":"Cystic fibrosis kidney stone disease in the era of CFTR modulator use and the COVID-19 pandemic","volume":"20","author":"Romano","year":"2021","journal-title":"J. Cyst. Fibros."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1016\/S0022-5347(01)69068-2","article-title":"Metabolic risk factors for stone formation in patients with cystic fibrosis","volume":"167","author":"Caplan","year":"2002","journal-title":"J. Urol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"160","DOI":"10.5414\/CNP59160","article-title":"Which factors account for renal stone formation in cystic fibrosis?","volume":"59","author":"Balestra","year":"2003","journal-title":"Clin. Nephrol."},{"key":"ref_74","unstructured":"(2023, April 28). Prospective Study of Pregnancy in Women with Cystic Fibrosis\u2014Full Text View, Available online: https:\/\/clinicaltrials.gov\/ct2\/show\/NCT04828382."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1016\/j.jcf.2021.07.019","article-title":"Pregnancy in cystic fibrosis: Review of the literature and expert recommendations","volume":"21","author":"Jain","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1095\/biolreprod.110.084517","article-title":"Maternal Undernutrition Influences Placental-Fetal Development","volume":"83","author":"Belkacemi","year":"2010","journal-title":"Biol. Reprod."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1097\/GRF.0b013e3181deb448","article-title":"Cystic fibrosis in pregnancy","volume":"53","author":"Whitty","year":"2010","journal-title":"Clin. Obstet. Gynecol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1177\/1753495X14554022","article-title":"Pregnancy and cystic fibrosis: Approach to contemporary management","volume":"7","author":"Geake","year":"2014","journal-title":"Obstet. Med."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1349","DOI":"10.1016\/j.healun.2021.07.005","article-title":"Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation","volume":"40","author":"Leard","year":"2021","journal-title":"J. Heart Lung Transplant."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"898","DOI":"10.1016\/j.chest.2019.11.043","article-title":"Underweight Patients with Cystic Fibrosis Have Acceptable Survival Following Lung Transplantation","volume":"157","author":"Ramos","year":"2020","journal-title":"Chest"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.jcf.2019.03.002","article-title":"Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines","volume":"18","author":"Ramos","year":"2019","journal-title":"J. Cyst. Fibros."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1177\/0884533615591604","article-title":"Nutrition Management of Cystic Fibrosis in the 21st Century","volume":"30","author":"Schindler","year":"2015","journal-title":"Nutr. Clin. Pract."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1016\/j.nut.2009.06.026","article-title":"Fat-free mass depletion in cystic fibrosis: Associated with lung disease severity but poorly detected by body mass index","volume":"26","author":"King","year":"2010","journal-title":"Nutrition"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1016\/j.jcf.2013.08.009","article-title":"Effects of nutritional status and dietetic interventions on survival in Cystic Fibrosis patients before and after lung transplantation","volume":"13","author":"Hollander","year":"2014","journal-title":"J. Cyst. Fibros."},{"key":"ref_85","first-page":"100276","article-title":"Obesity in cystic fibrosis","volume":"26","author":"Kutney","year":"2021","journal-title":"J. Clin. Transl. Endocrinol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1055\/s-0042-1758731","article-title":"Advanced Cystic Fibrosis Lung Disease and Lung Transplantation in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulators","volume":"44","year":"2023","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_87","first-page":"CD011737","article-title":"Reduction in saturated fat intake for cardiovascular disease","volume":"5","author":"Hooper","year":"2020","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_88","doi-asserted-by":"crossref","unstructured":"Wang, Y.-J., Yeh, T.-L., Shih, M.-C., Tu, Y.-K., and Chien, K.-L. (2020). Dietary Sodium Intake and Risk of Cardiovascular Disease: A Systematic Review and Dose-Response Meta-Analysis. Nutrients, 12.","DOI":"10.3390\/nu12102934"}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/13\/7\/1431\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T19:58:56Z","timestamp":1760126336000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/13\/7\/1431"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,6,22]]},"references-count":88,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2023,7]]}},"alternative-id":["life13071431"],"URL":"https:\/\/doi.org\/10.3390\/life13071431","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,6,22]]}}}