{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T01:40:04Z","timestamp":1791337204039,"version":"4.3.1"},"reference-count":192,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2023,1,17]],"date-time":"2023-01-17T00:00:00Z","timestamp":1673913600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Patients with cystic fibrosis (CF) are prone to malnutrition and growth failure, mostly due to malabsorption caused by the derangement in the chloride transport across epithelial surfaces. Thus, optimal nutritional care and support should be an integral part of the management of the disease, with the aim of ameliorating clinical outcomes and life expectancy. In this report, we analyzed the nutrition support across the different ages, in patients with CF, with a focus on the relationships with growth, nutritional status, disease outcomes and the use of the CF transmembrane conductance regulator (CFTR) modulators. The nutrition support goal in CF care should begin as early as possible after diagnosis and include the achievement of an optimal nutritional status to support the growth stages and puberty development in children, that will further support the maintenance of an optimal nutritional status in adult life. The cornerstone of nutrition in patients with CF is a high calorie, high-fat diet, in conjunction with a better control of malabsorption due to pancreatic enzyme replacement therapy, and attention to the adequate supplementation of fat-soluble vitamins. When the oral caloric intake is not enough for reaching the anthropometric nutritional goals, supplemental enteral feeding should be initiated to improve growth and the nutritional status. In the last decade, the therapeutic possibilities towards CF have grown in a consistent way. The positive effects of CFTR modulators on nutritional status mainly consist in the improvement in weight gain and BMI, both in children and adults, and in an amelioration in terms of the pulmonary function and reduction of exacerbations. Several challenges need to be overcome with the development of new drugs, to transform CF from a fatal disease to a treatable chronic disease with specialized multidisciplinary care.<\/jats:p>","DOI":"10.3390\/nu15030479","type":"journal-article","created":{"date-parts":[[2023,1,17]],"date-time":"2023-01-17T02:58:16Z","timestamp":1673924296000},"page":"479","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":49,"title":["Nutritional Care in Children with Cystic Fibrosis"],"prefix":"10.3390","volume":"15","author":[{"given":"Elena","family":"Mariotti Zani","sequence":"first","affiliation":[{"name":"Pediatric Clinic, Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1341-761X","authenticated-orcid":false,"given":"Roberto","family":"Grandinetti","sequence":"additional","affiliation":[{"name":"Pediatric Clinic, Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Cunico","sequence":"additional","affiliation":[{"name":"Pediatric Clinic, Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Torelli","sequence":"additional","affiliation":[{"name":"Pediatric Clinic, Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Fainardi","sequence":"additional","affiliation":[{"name":"Pediatric Clinic, Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanna","family":"Pisi","sequence":"additional","affiliation":[{"name":"Pediatric Clinic, Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanna","family":"Esposito","sequence":"additional","affiliation":[{"name":"Pediatric Clinic, Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,1,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1097\/MCP.0000000000000423","article-title":"Effects of new and emerging therapies on gastrointestinal outcomes in cystic fibrosis","volume":"23","author":"Houwen","year":"2017","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1016\/j.jpeds.2015.02.049","article-title":"Associations between Gut Microbial Colonization in Early Life and Respiratory Outcomes in Cystic Fibrosis","volume":"167","author":"Hoen","year":"2015","journal-title":"J. Pediatr."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.clnesp.2018.06.008","article-title":"Probiotics in cystic fibrosis patients: A double blind crossover placebo controlled study: Pilot study from the ESPGHAN Working Group on Pancreas\/CF","volume":"27","author":"Hauser","year":"2018","journal-title":"Clin. Nutr. ESPEN"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1056\/NEJMoa1105185","article-title":"A CFTR potentiator in patients with cystic fibrosis and the G551D mutation","volume":"365","author":"Ramsey","year":"2011","journal-title":"N. Engl. J. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1186\/s12967-017-1193-9","article-title":"Cystic fibrosis: Current therapeutic targets and future approaches","volume":"15","author":"Rafeeq","year":"2017","journal-title":"J. Transl. Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1016\/j.cgh.2012.11.006","article-title":"Gastrointestinal complications of cystic fibrosis","volume":"11","author":"Gelfond","year":"2013","journal-title":"Clin. Gastroenterol. Hepatol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1016\/j.pcl.2017.01.010","article-title":"Pancreatic Disorders","volume":"64","author":"Uc","year":"2017","journal-title":"Pediatr. Clin. N. Am."},{"key":"ref_8","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_9","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1016\/j.dld.2014.06.011","article-title":"Digestive system dysfunction in cystic fibrosis: Challenges for nutrition therapy","volume":"46","author":"Li","year":"2014","journal-title":"Dig. Liver Dis."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1016\/j.clnu.2016.03.004","article-title":"ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis","volume":"35","author":"Turck","year":"2016","journal-title":"Clin. Nutr."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/j.ccm.2015.11.009","article-title":"Nutritional Issues in Cystic Fibrosis","volume":"37","author":"Solomon","year":"2016","journal-title":"Clin. Chest Med."},{"key":"ref_12","first-page":"503","article-title":"Association of growth and nutritional parameters with pulmonary function in cystic fibrosis: A literature review","volume":"34","author":"Mauch","year":"2016","journal-title":"Rev. Paul. Pediatr."},{"key":"ref_13","first-page":"4","article-title":"Nutritional management of cystic fibrosis\u2014An update for the 21st century","volume":"26","author":"Collins","year":"2018","journal-title":"Paediatr. Respir. Rev."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1016\/j.jcf.2017.05.006","article-title":"The changing face of nutrition in cystic fibrosis","volume":"16","author":"Wolfe","year":"2017","journal-title":"J. Cyst. Fibros."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1542\/peds.107.1.1","article-title":"Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Wisconsin Cystic Fibrosis Neonatal Screening Study Group","volume":"107","author":"Farrell","year":"2001","journal-title":"Pediatrics"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/S0022-3476(82)80501-5","article-title":"Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption","volume":"100","author":"Gaskin","year":"1982","journal-title":"J. Pediatr."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1111\/j.1651-2227.1978.tb16273.x","article-title":"Relative underweight in cystic fibrosis and its prognostic value","volume":"67","author":"Kraemer","year":"1978","journal-title":"Acta Paediatr Scand."},{"key":"ref_18","first-page":"409","article-title":"Association between nutritional status measurements and pulmonary function in children and adolescents with cystic fibrosis","volume":"35","author":"Gomes","year":"2009","journal-title":"J. Bras. Pneumol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"624","DOI":"10.1067\/mpd.2003.152","article-title":"Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis","volume":"142","author":"Konstan","year":"2003","journal-title":"J. Pediatr."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1016\/j.jpeds.2015.07.044","article-title":"Early Life Growth Trajectories in Cystic Fibrosis are Associated with Pulmonary Function at Age 6 Years","volume":"167","author":"Sanders","year":"2015","journal-title":"J. Pediatr."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1016\/j.jpeds.2012.08.040","article-title":"Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis","volume":"162","author":"Yen","year":"2013","journal-title":"J. Pediatr."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1007\/s00408-019-00218-3","article-title":"Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis","volume":"197","author":"Ashkenazi","year":"2019","journal-title":"Lung"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1136\/adc.64.3.373","article-title":"Energy and nutrient intakes in cystic fibrosis","volume":"64","author":"Buchdahl","year":"1989","journal-title":"Arch. Dis. Child."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1056\/NEJMoa021218","article-title":"Association of cystic fibrosis with abnormalities in fatty acid metabolism","volume":"350","author":"Freedman","year":"2004","journal-title":"N. Engl. J. Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1016\/S0022-3476(05)82478-3","article-title":"Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis","volume":"120","author":"Bronstein","year":"1992","journal-title":"J. Pediatr."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1097\/MPG.0b013e31802c0ceb","article-title":"Risk factors for small bowel bacterial overgrowth in cystic fibrosis","volume":"44","author":"Fridge","year":"2007","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"948367","DOI":"10.1155\/2012\/948367","article-title":"Update of faecal markers of inflammation in children with cystic fibrosis","volume":"2012","author":"Lee","year":"2012","journal-title":"Mediat. Inflamm."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/0016-5085(88)90490-8","article-title":"Impaired chloride secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas","volume":"95","author":"Kopelman","year":"1988","journal-title":"Gastroenterology"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"S70","DOI":"10.1016\/j.jcf.2017.06.011","article-title":"Pancreatic insufficiency in Cystic Fibrosis","volume":"16","author":"Singh","year":"2017","journal-title":"J. Cyst. Fibros."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1097\/MEG.0b013e3282f36d04","article-title":"The changing face of the exocrine pancreas in cystic fibrosis: The correlation between pancreatic status, pancreatitis and cystic fibrosis genotype","volume":"20","author":"Augarten","year":"2008","journal-title":"Eur. J. Gastroenterol. Hepatol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1016\/S0016-5085(00)70358-1","article-title":"Identification of transport abnormalities in duodenal mucosa and duodenal enterocytes from patients with cystic fibrosis","volume":"118","author":"Pratha","year":"2000","journal-title":"Gastroenterology"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"11467","DOI":"10.3748\/wjg.v20.i33.11467","article-title":"Pancreatic enzyme replacement therapy for pancreatic exocrine insufficiency in the 21(st) century","volume":"20","author":"Trang","year":"2014","journal-title":"World J. Gastroenterol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1136\/gut.25.5.491","article-title":"Effect of intrajejunal acidity on lipid digestion and aqueous solubilisation of bile acids and lipids in health, using a new simple method of lipase inactivation","volume":"25","author":"Fine","year":"1984","journal-title":"Gut"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"2275","DOI":"10.1007\/s10620-012-2209-1","article-title":"Intestinal pH and gastrointestinal transit profiles in cystic fibrosis patients measured by wireless motility capsule","volume":"58","author":"Gelfond","year":"2013","journal-title":"Dig. Dis. Sci."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1097\/01.mpg.0000178439.64675.8d","article-title":"Gastrointestinal outcomes and confounders in cystic fibrosis","volume":"41","author":"Borowitz","year":"2005","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"G453","DOI":"10.1152\/ajpgi.00094.2013","article-title":"Characterization of CFTR High Expresser cells in the intestine","volume":"305","author":"Jakab","year":"2013","journal-title":"Am. J. Physiol. Gastrointest. Liver Physiol."},{"key":"ref_37","first-page":"a009753","article-title":"The cystic fibrosis intestine","volume":"3","author":"Borowitz","year":"2013","journal-title":"Cold Spring Harb. Perspect. Med."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"G577","DOI":"10.1152\/ajpgi.00195.2007","article-title":"Effects of laxative and N-acetylcysteine on mucus accumulation, bacterial load, transit, and inflammation in the cystic fibrosis mouse small intestine","volume":"293","author":"Roach","year":"2007","journal-title":"Am. J. Physiol. Gastrointest. Liver Physiol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"S14","DOI":"10.1016\/j.jcf.2017.07.014","article-title":"Bacterial overgrowth, dysbiosis, inflammation, and dysmotility in the Cystic Fibrosis intestine","volume":"16","author":"Dorsey","year":"2017","journal-title":"J. Cyst. Fibros."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1097\/MPG.0b013e3182638bf4","article-title":"Intestinal smooth muscle dysfunction develops postnatally in cystic fibrosis mice","volume":"55","author":"Meldi","year":"2012","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1111\/j.1365-2982.2009.01418.x","article-title":"Enteric circular muscle dysfunction in the cystic fibrosis mouse small intestine","volume":"22","author":"Sewell","year":"2010","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"582","DOI":"10.1002\/ppul.21598","article-title":"The impact of reflux burden on Pseudomonas positivity in children with cystic fibrosis","volume":"47","author":"Palm","year":"2012","journal-title":"Pediatr. Pulmonol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1080\/17474124.2018.1502663","article-title":"Gastrointestinal pathophysiology and nutrition in cystic fibrosis","volume":"12","author":"Ratchford","year":"2018","journal-title":"Expert Rev. Gastroenterol. Hepatol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1136\/archdischild-2013-304159","article-title":"Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes","volume":"99","author":"Leeuwen","year":"2014","journal-title":"Arch. Dis. Child."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.dld.2021.06.034","article-title":"Serum bile acids in cystic fibrosis patients\u2014Glycodeoxycholic acid as a potential marker of liver disease","volume":"54","author":"Dziedzic","year":"2022","journal-title":"Dig. Liver Dis."},{"key":"ref_46","doi-asserted-by":"crossref","unstructured":"Drzyma\u0142a-Czy\u017c, S., Krzy\u017canowska-Jankowska, P., Dziedzic, K., Lisowska, A., Kurek, S., Go\u017adzik-Spychalska, J., Kononets, V., Wo\u017aniak, D., M\u0105dry, E., and Walkowiak, J. (2021). Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis. Biomolecules, 11.","DOI":"10.3390\/biom11020313"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1725","DOI":"10.1515\/cclm-2019-1112","article-title":"Influence of pancreatic status on circulating plasma sterols in patients with cystic fibrosis","volume":"58","author":"Gelzo","year":"2020","journal-title":"Clin. Chem. Lab. Med."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1021\/acs.jproteome.0c00556","article-title":"Lipidome Alterations Induced by Cystic Fibrosis, CFTR Mutation, and Lung Function","volume":"20","author":"Nizami","year":"2021","journal-title":"J. Proteome Res."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1007\/s00408-017-0029-y","article-title":"Increased Soluble VCAM-1 and Normal P-Selectin in Cystic Fibrosis: A Cross-Sectional Study","volume":"195","author":"Nowak","year":"2017","journal-title":"Lung"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"S36","DOI":"10.1097\/00005176-198811001-00008","article-title":"The management of enzymatic therapy in cystic fibrosis patients by an individualized approach","volume":"7","author":"Costantini","year":"1988","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_51","first-page":"418","article-title":"Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients","volume":"31","author":"Steinkamp","year":"2000","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1093\/jpepsy\/15.3.309","article-title":"A behavioral approach to increasing calorie consumption in children with cystic fibrosis","volume":"15","author":"Stark","year":"1990","journal-title":"J. Pediatr. Psychol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1093\/ajcn\/48.2.235","article-title":"Short-term nutritional supplementation during management of pulmonary exacerbations in cystic fibrosis: A controlled study, including effects of protein turnover","volume":"48","author":"Shepherd","year":"1988","journal-title":"Am. J. Clin. Nutr."},{"key":"ref_54","first-page":"164","article-title":"Supplemental nasogastric feeding in cystic fibrosis patients during treatment for acute exacerbation of chest disease","volume":"25","author":"Daniels","year":"1989","journal-title":"Aust. Paediatr. J."},{"key":"ref_55","first-page":"141","article-title":"Nutritional rehabilitation in cystic fibrosis: A 5 year follow-up study","volume":"15","author":"Dalzell","year":"1992","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1016\/S0022-3476(94)70312-4","article-title":"Improvement of nutritional status and lung function after long-term nocturnal gastrostomy feedings in cystic fibrosis","volume":"124","author":"Steinkamp","year":"1994","journal-title":"J. Pediatr."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1016\/S0022-3476(85)80130-X","article-title":"Effects of long-term nutritional rehabilitation on body composition and clinical status in malnourished children and adolescents with cystic fibrosis","volume":"107","author":"Levy","year":"1985","journal-title":"J. Pediatr."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1016\/S0022-3476(84)80947-6","article-title":"Short-term pulmonary effects of total parenteral nutrition in children with cystic fibrosis","volume":"104","author":"Mansell","year":"1984","journal-title":"J. Pediatr."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/S0022-3476(84)80586-7","article-title":"Short-term clinical, nutritional, and functional effects of continuous elemental enteral alimentation in children with cystic fibrosis","volume":"104","author":"Bertrand","year":"1984","journal-title":"J. Pediatr."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1002\/ppul.10259","article-title":"Comparing the clinical evolution of cystic fibrosis screened neonatally to that of cystic fibrosis diagnosed from clinical symptoms: A 10-year retrospective study in a French region (Brittany)","volume":"35","author":"Siret","year":"2003","journal-title":"Pediatr. Pulmonol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1542\/peds.2006-1498","article-title":"Cystic fibrosis diagnosed after 2 months of age leads to worse outcomes and requires more therapy","volume":"119","author":"Sims","year":"2007","journal-title":"Pediatrics"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1093\/aje\/kwh083","article-title":"Association between initial disease presentation, lung disease outcomes, and survival in patients with cystic fibrosis","volume":"159","author":"Lai","year":"2004","journal-title":"Am. J. Epidemiol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"714","DOI":"10.1542\/peds.2007-3089","article-title":"Recovery of birth weight z score within 2 years of diagnosis is positively associated with pulmonary status at 6 years of age in children with cystic fibrosis","volume":"123","author":"Lai","year":"2009","journal-title":"Pediatrics"},{"key":"ref_64","first-page":"17","article-title":"Does newborn screening improve early lung function in cystic fibrosis?","volume":"42","author":"Davies","year":"2020","journal-title":"Paediatr. Respir. Rev."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1002\/ppul.20842","article-title":"Improved pulmonary and growth outcomes in cystic fibrosis by newborn screening","volume":"43","author":"Collins","year":"2008","journal-title":"Pediatr. Pulmonol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1016\/S2213-2600(16)00053-9","article-title":"Newborn screening for cystic fibrosis","volume":"4","author":"Castellani","year":"2016","journal-title":"Lancet Respir. Med."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"649904","DOI":"10.3389\/fped.2021.649904","article-title":"Sweat Testing and Recent Advances","volume":"9","author":"Gokdemir","year":"2021","journal-title":"Front. Pediatr."},{"key":"ref_68","first-page":"246","article-title":"Consensus report on nutrition for pediatric patients with cystic fibrosis","volume":"35","author":"Borowitz","year":"2002","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_69","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_70","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1097\/MPG.0000000000000610","article-title":"Comparison of WHO and CDC growth charts in predicting pulmonary outcomes in cystic fibrosis","volume":"60","author":"Machogu","year":"2015","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_71","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_72","doi-asserted-by":"crossref","first-page":"S87","DOI":"10.1016\/j.jcf.2017.07.010","article-title":"Nutrition: Prevention and management of nutritional failure in Cystic Fibrosis","volume":"16","author":"Sullivan","year":"2017","journal-title":"J. Cyst. Fibros."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"33","DOI":"10.3389\/fped.2014.00033","article-title":"Body composition and pulmonary function in cystic fibrosis","volume":"2","author":"Sheikh","year":"2014","journal-title":"Front. Pediatr."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1016\/j.jcf.2013.02.008","article-title":"Update on cystic fibrosis-related diabetes","volume":"12","author":"Kelly","year":"2013","journal-title":"J. Cyst. Fibros."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.cccn.2004.11.002","article-title":"Circulating markers to assess nutritional therapy in cystic fibrosis","volume":"353","author":"Wood","year":"2005","journal-title":"Clin. Chim. Acta"},{"key":"ref_76","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_77","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1046\/j.1365-2362.2000.00667.x","article-title":"Metabolic and inflammatory responses to pulmonary exacerbation in adults with cystic fibrosis","volume":"30","author":"Bell","year":"2000","journal-title":"Eur. J. Clin. Investig."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1016\/j.jpeds.2007.10.021","article-title":"Longitudinal analysis of resting energy expenditure in patients with cystic fibrosis","volume":"152","author":"Magoffin","year":"2008","journal-title":"J. Pediatr."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1038\/ejcn.2013.20","article-title":"Nutritional care in children with cystic fibrosis: Are our patients becoming better?","volume":"67","author":"Gaskin","year":"2013","journal-title":"Eur. J. Clin. Nutr."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"60S","DOI":"10.1177\/0148607111420156","article-title":"Nutritional Management of Cystic Fibrosis","volume":"36","author":"Matel","year":"2012","journal-title":"J. Parent. Ent. Nutr."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1016\/j.clnu.2014.04.007","article-title":"Protein intake and exercise for optimal muscle function with aging: Recommendations from the ESPEN Expert Group","volume":"33","author":"Deutz","year":"2014","journal-title":"Clin. Nutr."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1016\/j.clnu.2012.04.012","article-title":"Use of body mass index percentile to identify fat-free mass depletion in children with cystic fibrosis","volume":"31","author":"Engelen","year":"2012","journal-title":"Clin. Nutr."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1097\/MCO.0000000000000100","article-title":"Protein is an important but undervalued macronutrient in the nutritional care of patients with cystic fibrosis","volume":"17","author":"Engelen","year":"2014","journal-title":"Curr. Opin. Clin. Nutr. Metab. Care"},{"key":"ref_84","unstructured":"Matel, J.L., and Kerner, J.A. (2023, January 03). Nutritional Management of Cystic Fibrosis. Available online: https:\/\/aspenjournals.onlinelibrary.wiley.com\/doi\/abs\/10.1177\/0148607111420156."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1097\/MPG.0b013e31817fb76b","article-title":"Serum linoleic acid status as a clinical indicator of essential fatty acid status in children with cystic fibrosis","volume":"47","author":"Maqbool","year":"2008","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"102156","DOI":"10.1016\/j.plefa.2020.102156","article-title":"Abnormal n-6 fatty acid metabolism in cystic fibrosis contributes to pulmonary symptoms","volume":"160","author":"Wheelock","year":"2020","journal-title":"Prostaglandins Leukot. Essent. Fat. Acids"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.plefa.2016.03.014","article-title":"The clinical benefits of long-term supplementation with omega-3 fatty acids in cystic fibrosis patients\u2014A pilot study","volume":"108","author":"Hanssens","year":"2016","journal-title":"Prostaglandins Leukot. Essent. Fat. Acids"},{"key":"ref_88","unstructured":"Ministero della Salute (2023, January 03). Linee Guida per una Corretta Prescrizione di Alimenti a Fini Medici Speciali Erogabili per Soggetti con Fibrosi Cistica, Available online: https:\/\/www.salute.gov.it\/imgs\/C_17_pubblicazioni_1438_allegato.pdf."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1136\/jclinpath-2013-201787","article-title":"Fat-soluble vitamin deficiency in children and adolescents with cystic fibrosis","volume":"67","author":"Rana","year":"2014","journal-title":"J. Clin. Pathol."},{"key":"ref_90","doi-asserted-by":"crossref","unstructured":"Nowak, I.K., Sobkowiak, P., Drzyma\u0142a-Czy\u017c, S., Krzy\u017canowska-Jankowska, P., Sapiejka, E., Skorupa, W., Pogorzelski, A., Nowicka, A., Wojsyk-Banaszak, I., and Kurek, S. (2021). Fat-Soluble Vitamin Supplementation Using Liposomes, Cyclodextrins, or Medium-Chain Triglycerides in Cystic Fibrosis: A Randomized Controlled Trial. Nutrients, 13.","DOI":"10.3390\/nu13124554"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1002\/ped4.12270","article-title":"Association of vitamin A status with lung function in children and adolescents with cystic fibrosis","volume":"5","author":"Loukou","year":"2021","journal-title":"Pediatr. Investig."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.jcf.2004.04.003","article-title":"Vitamin A levels in patients with CF are influenced by the inflammatory response","volume":"3","author":"Greer","year":"2004","journal-title":"J. Cyst. Fibros."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1097\/MPG.0b013e31804069e5","article-title":"Vitamins A and E and pulmonary exacerbations in patients with cystic fibrosis","volume":"45","author":"Hakim","year":"2007","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1159\/000012818","article-title":"Long-Term Oral \u03b2-Carotene Supplementation in Patients with Cystic Fibrosis\u2014Effects on Antioxidative Status and Pulmonary Function","volume":"44","author":"Rust","year":"2000","journal-title":"Ann. Nutr. Metab."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1097\/MCP.0b013e3283136787","article-title":"Update on fat-soluble vitamins in cystic fibrosis","volume":"14","author":"Maqbool","year":"2008","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/j.jcf.2007.07.002","article-title":"Vitamin A intake and elevated serum retinol levels in children and young adults with cystic fibrosis","volume":"7","author":"Maqbool","year":"2008","journal-title":"J. Cyst. Fibros."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"244","DOI":"10.3945\/an.111.000398","article-title":"Vitamin D and Chronic Lung Disease: A Review of Molecular Mechanisms and Clinical Studies","volume":"2","author":"Finklea","year":"2011","journal-title":"Adv. Nutr. Int. Rev. J."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"1928","DOI":"10.1097\/CCM.0000000000001148","article-title":"Effect of Cholecalciferol Supplementation on Vitamin D Status and Cathelicidin Levels in Sepsis: A Randomized, Placebo-Controlled Trial","volume":"43","author":"Quraishi","year":"2015","journal-title":"Crit. Care Med."},{"key":"ref_99","unstructured":"Spedding, S. (2019). Vitamin D and Human Health, MDPI."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/j.jsbmb.2015.09.013","article-title":"Treatment of vitamin D deficiency in cystic fibrosis","volume":"164","author":"Chesdachai","year":"2016","journal-title":"J. Steroid Biochem. Mol. Biol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1082","DOI":"10.1210\/jc.2011-3050","article-title":"An Update on the Screening, Diagnosis, Management, and Treatment of Vitamin D Deficiency in Individuals with Cystic Fibrosis: Evidence-Based Recommendations from the Cystic Fibrosis Foundation","volume":"97","author":"Tangpricha","year":"2012","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1002\/ppul.23161","article-title":"Association between serum 25-hydroxyvitamin D level and pulmonary exacerbations in cystic fibrosis","volume":"50","author":"Vanstone","year":"2015","journal-title":"Pediatr. Pulmonol."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1513\/AnnalsATS.201208-068OC","article-title":"Vitamin D Deficiency Is Associated with Pulmonary Exacerbations in Children with Cystic Fibrosis","volume":"11","author":"McCauley","year":"2014","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1038\/ejcn.2016.259","article-title":"Clinical impact of vitamin D treatment in cystic fibrosis: A pilot randomized, controlled trial","volume":"71","author":"Pincikova","year":"2017","journal-title":"Eur. J. Clin. Nutr."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"191","DOI":"10.4161\/derm.20332","article-title":"Pilot study of vitamin D supplementation in adults with cystic fibrosis pulmonary exacerbation: A randomized, controlled trial","volume":"4","author":"Grossmann","year":"2012","journal-title":"Derm.-Endocrinol."},{"key":"ref_106","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."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1016\/j.jpeds.2008.04.058","article-title":"Current Treatment Recommendations for Correcting Vitamin D Deficiency in Pediatric Patients with Cystic Fibrosis Are Inadequate","volume":"153","author":"Green","year":"2008","journal-title":"J. Pediatr."},{"key":"ref_108","first-page":"CD008482","article-title":"Vitamin K supplementation for cystic fibrosis","volume":"1","author":"Jagannath","year":"2015","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"1325","DOI":"10.1542\/peds.2004-1242","article-title":"Vitamin K status among children with cystic fibrosis and its relationship to bone mineral density and bone turnover","volume":"115","author":"Conway","year":"2005","journal-title":"Pediatrics"},{"key":"ref_110","first-page":"1489","article-title":"Vitamin D, K and bone mineral density","volume":"15","author":"Okano","year":"2005","journal-title":"Clin. Calcium"},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"660","DOI":"10.3945\/ajcn.2010.29350","article-title":"Suboptimal vitamin K status despite supplementation in children and young adults with cystic fibrosis","volume":"92","author":"Dougherty","year":"2010","journal-title":"Am. J. Clin. Nutr."},{"key":"ref_112","first-page":"512","article-title":"Effect of vitamin K1 supplementation on vitamin K status in cystic fibrosis patients","volume":"24","author":"Beker","year":"1997","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_113","unstructured":"Shils, M.E., Olson, J.A., and Shike, M. (1994). Book Reviews: Modern Nutrition in Health and Disease, Lea & Febiger. [8th ed.]."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1016\/j.pcl.2009.07.004","article-title":"Nutritional deficiencies during normal growth","volume":"56","author":"Suskind","year":"2009","journal-title":"Pediatr. Clin. N. Am."},{"key":"ref_115","first-page":"341","article-title":"Vitamin E supplementation in cystic fibrosis","volume":"22","author":"Peters","year":"1996","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1016\/j.freeradbiomed.2008.12.007","article-title":"Vitamin E: The shrew waiting to be tamed","volume":"46","year":"2009","journal-title":"Free Radic. Biol. Med."},{"key":"ref_117","first-page":"CD009422","article-title":"Vitamin E supplementation in people with cystic fibrosis","volume":"3","author":"Okebukola","year":"2017","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1016\/j.jns.2009.07.020","article-title":"Correlation between neurological dysfunction with vitamin E deficiency and gastrectomy","volume":"287","author":"Ueda","year":"2009","journal-title":"J. Neurol. Sci."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"S51","DOI":"10.1016\/j.jpeds.2005.08.003","article-title":"Preventing early, prolonged vitamin E deficiency: An opportunity for better cognitive outcomes via early diagnosis through neonatal screening","volume":"147","author":"Koscik","year":"2005","journal-title":"J. Pediatr."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1046\/j.1365-2257.1998.00099.x","article-title":"Anaemia, vitamin E deficiency and failure to thrive in an infant","volume":"20","author":"Swann","year":"1998","journal-title":"Clin. Lab. Haematol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1177\/000992289403300101","article-title":"Severe hemolytic anemia associated with vitamin E deficiency in infants with cystic fibrosis. Implications for neonatal screening","volume":"33","author":"Wilfond","year":"1994","journal-title":"Clin. Pediatr."},{"key":"ref_122","doi-asserted-by":"crossref","unstructured":"Sommerburg, O., H\u00e4mmerling, S., Schneider, S., Okun, J., Langhans, C.-D., Leutz-Schmidt, P., Wielp\u00fctz, M., Siems, W., Gr\u00e4ber, S., and Mall, M. (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_123","doi-asserted-by":"crossref","first-page":"e81","DOI":"10.1038\/ctg.2017.10","article-title":"Impact of CFTR Modulation on Intestinal pH, Motility, and Clinical Outcomes in Patients With Cystic Fibrosis and the G551D Mutation","volume":"8","author":"Gelfond","year":"2017","journal-title":"Clin. Transl. Gastroenterol."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1097\/00075197-200503000-00005","article-title":"Vitamin E: Absorption, plasma transport and cell uptake","volume":"8","author":"Hacquebard","year":"2005","journal-title":"Curr. Opin. Clin. Nutr. Metab. Care"},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1016\/S0006-291X(03)00811-8","article-title":"Vitamin E and drug metabolism","volume":"305","year":"2003","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1016\/S0022-3476(99)70059-4","article-title":"Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen","volume":"135","author":"Feranchak","year":"1999","journal-title":"J. Pediatr."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"1517","DOI":"10.1016\/j.jand.2020.05.011","article-title":"Sodium Status and Replacement in Children and Adults Living with Cystic Fibrosis: A Narrative Review","volume":"120","author":"Declercq","year":"2020","journal-title":"J. Acad. Nutr. Diet."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1016\/j.jcf.2009.08.006","article-title":"Evaluation of salt supplementation in CF infants","volume":"8","author":"Coates","year":"2009","journal-title":"J. Cyst. Fibros."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.jpeds.2012.01.052","article-title":"Prevalence of Hyponatremia at Diagnosis and Factors Associated with the Longitudinal Variation in Serum Sodium Levels in Infants with Cystic Fibrosis","volume":"161","author":"Schettino","year":"2012","journal-title":"J. Pediatr."},{"key":"ref_130","doi-asserted-by":"crossref","unstructured":"Cystic Fibrosis Foundation, Borowitz, D., Robinson, K.A., Rosenfeld, M., Davis, S.D., Sabadosa, K.A., Spear, S.L., Michel, S.H., Parad, R.B., and White, T.B. (2009). Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J. Pediatr., 155, S73\u2013S93.","DOI":"10.1016\/j.jpeds.2009.09.001"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1111\/j.1651-2227.1983.tb09814.x","article-title":"Cystic Fibrosis Mimicking Bartter\u2019s Syndrome","volume":"72","author":"Davison","year":"1983","journal-title":"Acta Paediatr."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1203\/00006450-197111000-00007","article-title":"The Renin-Angiotensin-Aldosterone System in Patients with Cystic Fibrosis of the Pancreas","volume":"5","author":"Simopoulos","year":"1971","journal-title":"Pediatr. Res."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1203\/00006450-198304000-00007","article-title":"Exercise and heat stress in cystic fibrosis patients","volume":"17","author":"Orenstein","year":"1983","journal-title":"Pediatr. Res."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1097\/00005768-199906000-00003","article-title":"Preventing dehydration in children with cystic fibrosis who exercise in the heat","volume":"31","author":"Kriemler","year":"1999","journal-title":"Med. Sci. Sport. Exerc."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"59","DOI":"10.2174\/1874306401206010059","article-title":"Metabolic alkalosis in adults with stable cystic fibrosis","volume":"6","author":"Faughnan","year":"2012","journal-title":"Open Respir. Med. J."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1016\/S0022-3468(88)80370-1","article-title":"Sodium deficit causing decreased weight gain and metabolic acidosis in infants with ileostomy","volume":"23","author":"Bower","year":"1988","journal-title":"J. Pediatr. Surg."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1002\/ppul.22857","article-title":"Iron deficiency occurs frequently in children with cystic fibrosis","volume":"49","author":"Uijterschout","year":"2014","journal-title":"Pediat. Pulmonol."},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1136\/adc.62.2.185","article-title":"Iron deficiency in cystic fibrosis","volume":"62","author":"Ehrhardt","year":"1987","journal-title":"Arch. Dis. Child."},{"key":"ref_139","unstructured":"World Health Organization (2011, November 01). Anaemia and Iron Deficiency. Available online: www.who.int\/nutrition\/publications\/micronutrients\/anaemia_iron_deficiency\/9789241596107\/en\/."},{"key":"ref_140","first-page":"3040346","article-title":"Serum Hepcidin Level as a Marker of Iron Status in Children with Cystic Fibrosis","volume":"2018","year":"2018","journal-title":"Mediat. Inflamm."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1016\/j.jcf.2014.03.007","article-title":"The value of soluble transferrin receptor and hepcidin in the assessment of iron status in children with cystic fibrosis","volume":"13","author":"Uijterschout","year":"2014","journal-title":"J. Cyst. Fibros."},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1016\/j.advms.2015.05.002","article-title":"Evaluation of factors related to bone disease in Polish children and adolescents with cystic fibrosis","volume":"60","author":"Sands","year":"2015","journal-title":"Adv. Med. Sci."},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"1014","DOI":"10.1542\/peds.2007-2336","article-title":"Prevalence of Low Bone Mass and Deficiencies of Vitamins D and K in Pediatric Patients with Cystic Fibrosis from 3 Canadian Centers","volume":"122","author":"Grey","year":"2008","journal-title":"Pediatrics"},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1136\/thorax.2003.006726","article-title":"Bone mineral density in Australian children, adolescents and adults with cystic fibrosis: A controlled cross sectional study","volume":"59","author":"Buntain","year":"2004","journal-title":"Thorax"},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"1888","DOI":"10.1210\/jc.2004-1629","article-title":"Guide to bone health and disease in cystic fibrosis","volume":"90","author":"Aris","year":"2005","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_146","first-page":"926045","article-title":"A roadmap to the brittle bones of cystic fibrosis","volume":"2011","author":"Gore","year":"2010","journal-title":"J. Osteoporos."},{"key":"ref_147","first-page":"616","article-title":"Impact of cystic fibrosis on bone health","volume":"16","author":"Haworth","year":"2010","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1016\/j.prrv.2009.05.001","article-title":"Update on cystic fibrosis-related bone disease: A special focus on children","volume":"10","author":"Castanet","year":"2009","journal-title":"Paediatr. Respir. Rev."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"1816S","DOI":"10.3945\/jn.115.229708","article-title":"Biomarkers of Nutrition for Development (BOND)-Vitamin A Review","volume":"146","author":"Tanumihardjo","year":"2016","journal-title":"J. Nutr."},{"key":"ref_150","doi-asserted-by":"crossref","unstructured":"Lin, P.-H., Sermersheim, M., Li, H., Lee, P., Steinberg, S., and Ma, J. (2017). Zinc in Wound Healing Modulation. Nutrients, 10.","DOI":"10.3390\/nu10010016"},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"1198","DOI":"10.3945\/ajcn.113.075036","article-title":"Zinc deficiency in children with environmental enteropathy-development of new strategies: Report from an expert workshop","volume":"100","author":"Young","year":"2014","journal-title":"Am. J. Clin. Nutr."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.jval.2017.07.007","article-title":"An Economic Evaluation of the Impact, Cost, and Medicare Policy Implications of Chronic Nonhealing Wounds","volume":"21","author":"Nussbaum","year":"2018","journal-title":"Value Health"},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"1452S","DOI":"10.1093\/jn\/133.5.1452S","article-title":"Zinc-altered immune function","volume":"133","author":"Ibs","year":"2003","journal-title":"J. Nutr."},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"510","DOI":"10.1016\/S0899-9007(01)00812-7","article-title":"The role of zinc in the growth and development of children","volume":"18","author":"Salgueiro","year":"2002","journal-title":"Nutrition"},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1146\/annurev.nu.05.070185.002013","article-title":"Clinical manifestations of zinc deficiency","volume":"5","author":"Prasad","year":"1985","journal-title":"Annu. Rev. Nutr."},{"key":"ref_156","first-page":"127","article-title":"Effect of supplementary zinc on body mass index, pulmonary function and hospitalization in children with cystic fibrosis","volume":"56","author":"Ataee","year":"2014","journal-title":"Turk. J. Pediatr."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1097\/01.mpg.0000228093.36089.35","article-title":"Plasma Zinc and Growth Status in Preadolescent Children with Cystic Fibrosis","volume":"43","author":"Maqbool","year":"2006","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"184","DOI":"10.2174\/157339609789007222","article-title":"Importance of zinc in cystic fibrosis patients","volume":"5","author":"Robberecht","year":"2009","journal-title":"Curr. Pediatr. Rev."},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1159\/000129650","article-title":"The effect of zinc supplements in cystic fibrosis patients","volume":"52","author":"Robberecht","year":"2008","journal-title":"Ann. Nutr. Metab."},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1002\/ppul.20771","article-title":"Effect of zinc supplementation on respiratory tract infections in children with cystic fibrosis","volume":"43","author":"Abdulhamid","year":"2008","journal-title":"Pediatr. Pulmonol."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1177\/0884533614527773","article-title":"Pancreatic enzyme replacement therapy during pancreatic insufficiency","volume":"29","author":"Berry","year":"2014","journal-title":"Nutr. Clin. Pract."},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/j.jcf.2010.04.008","article-title":"Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening","volume":"9","author":"Mayell","year":"2010","journal-title":"J. Cyst. Fibros."},{"key":"ref_163","doi-asserted-by":"crossref","first-page":"1110","DOI":"10.1016\/j.jpeds.2014.01.022","article-title":"Pancreatic enzyme replacement therapy dosing and nutritional outcomes in children with cystic fibrosis","volume":"164","author":"Haupt","year":"2014","journal-title":"J. Pediatr."},{"key":"ref_164","unstructured":"(2022, November 01). Clinical Guidelines: Care of Children with Cystic Fibrosis. Available online: https:\/\/www.rbht.nhs.uk\/childrencf."},{"key":"ref_165","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1016\/j.jcf.2014.09.005","article-title":"Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation","volume":"13","author":"Munck","year":"2014","journal-title":"J. Cyst. Fibros."},{"key":"ref_166","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1136\/archdischild-2015-310420","article-title":"The impact of 12 months treatment with ivacaftor on Scottish paediatric patients with cystic fibrosis with the G551D mutation: A review","volume":"103","author":"Dryden","year":"2018","journal-title":"Arch. Dis. Child."},{"key":"ref_167","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.jpeds.2018.05.018","article-title":"Energy Balance and Mechanisms of Weight Gain with Ivacaftor Treatment of Cystic Fibrosis Gating Mutations","volume":"201","author":"Stallings","year":"2018","journal-title":"J. Pediatr."},{"key":"ref_168","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1002\/ppul.24745","article-title":"Long-term effects of ivacaftor on nonpulmonary outcomes in individuals with cystic fibrosis, heterozygous for a S1251N mutation","volume":"55","author":"Burghard","year":"2020","journal-title":"Pediatr. Pulmonol."},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1007\/s11894-021-00817-2","article-title":"The Intestinal Microbiome and Cystic Fibrosis Transmembrane Conductance Regulator Modulators: Emerging Themes in the Management of Gastrointestinal Manifestations of Cystic Fibrosis","volume":"23","author":"Karb","year":"2021","journal-title":"Curr. Gastroenterol. Rep."},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1016\/j.jcf.2016.08.004","article-title":"Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines","volume":"15","author":"Schwarzenberg","year":"2016","journal-title":"J. Cyst. Fibros."},{"key":"ref_171","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1177\/0115426507022002223","article-title":"Update on enteral nutrition support for cystic fibrosis","volume":"22","author":"Erskine","year":"2007","journal-title":"Nutr. Clin. Pract."},{"key":"ref_172","first-page":"1662","article-title":"CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine","volume":"10","year":"2019","journal-title":"Front. Pharmacol."},{"key":"ref_173","doi-asserted-by":"crossref","unstructured":"Meoli, A., Eickmeier, O., Pisi, G., Fainardi, V., Zielen, S., and Esposito, S. (2022). Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms232012421"},{"key":"ref_174","unstructured":"Food and Drug Administration (2023, January 03). Highlights of Prescribing Information. Kalydeco, Available online: https:\/\/www.accessdata.fda.gov\/drugsatfda_docs\/label\/2020\/203188s034,207925s013lbl.pdf."},{"key":"ref_175","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1007\/s10620-015-3834-2","article-title":"Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation after Treatment with Ivacaftor","volume":"61","author":"Borowitz","year":"2016","journal-title":"Dig. Dis. Sci."},{"key":"ref_176","doi-asserted-by":"crossref","first-page":"902","DOI":"10.1016\/S2213-2600(14)70218-8","article-title":"Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: A phase 3, open-label extension study (PERSIST)","volume":"2","author":"McKone","year":"2014","journal-title":"Lancet Respir. Med."},{"key":"ref_177","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1164\/rccm.201404-0703OC","article-title":"Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis","volume":"190","author":"Rowe","year":"2014","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_178","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1111\/pedi.12026","article-title":"Insulin secretion improves in cystic fibrosis following ivacaftor correction of CFTR: A small pilot study","volume":"14","author":"Bellin","year":"2013","journal-title":"Pediatr. Diabetes"},{"key":"ref_179","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1164\/rccm.201405-0882LE","article-title":"Resolution of cystic fibrosis-related diabetes with ivacaftor therapy","volume":"190","author":"Hayes","year":"2014","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_180","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/S2213-2600(16)30427-1","article-title":"Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): A phase 3, extension study","volume":"5","author":"Konstan","year":"2017","journal-title":"Lancet Resp. Med."},{"key":"ref_181","doi-asserted-by":"crossref","first-page":"2024","DOI":"10.1056\/NEJMoa1709847","article-title":"Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis","volume":"377","author":"Rowe","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_182","doi-asserted-by":"crossref","first-page":"1612","DOI":"10.1056\/NEJMoa1807120","article-title":"VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles","volume":"379","author":"Keating","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_183","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_184","doi-asserted-by":"crossref","first-page":"957","DOI":"10.3389\/fimmu.2020.00957","article-title":"Gut Microbiota in Celiac Disease: Is There Any Role for Probiotics?","volume":"11","author":"Pecora","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_185","doi-asserted-by":"crossref","unstructured":"Frati, F., Salvatori, C., Incorvaia, C., Bellucci, A., Di Cara, G., Marcucci, F., and Esposito, S. (2018). The Role of the Microbiome in Asthma: The Gut\u2013Lung Axis. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20010123"},{"key":"ref_186","doi-asserted-by":"crossref","unstructured":"Fattorusso, A., Di Genova, L., Dell\u2019Isola, G., Mencaroni, E., and Esposito, S. (2019). Autism Spectrum Disorders and the Gut Microbiota. Nutrients, 11.","DOI":"10.3390\/nu11030521"},{"key":"ref_187","doi-asserted-by":"crossref","unstructured":"Esposito, S., Testa, I., Mariotti Zani, E., Cunico, D., Torelli, L., Grandinetti, R., Fainardi, V., Pisi, G., and Principi, N. (2022). Probiotics Administration in Cystic Fibrosis: What Is the Evidence?. Nutrients, 14.","DOI":"10.3390\/nu14153160"},{"key":"ref_188","doi-asserted-by":"crossref","first-page":"17834","DOI":"10.1038\/s41598-018-36364-6","article-title":"Impact of CFTR modulation with Ivacaftor on Gut Microbiota and Intestinal Inflammation","volume":"8","author":"Ooi","year":"2018","journal-title":"Sci. Rep."},{"key":"ref_189","doi-asserted-by":"crossref","unstructured":"Bernarde, C., Keravec, M., Mounier, J., Gouriou, S., Rault, G., F\u00e9rec, C., Barbier, G., and H\u00e9ry-Arnaud, G. (2015). Impact of the CFTR-potentiator ivacaftor on airway microbiota in cystic fibrosis patients carrying a G551D mutation. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0124124"},{"key":"ref_190","doi-asserted-by":"crossref","first-page":"00731","DOI":"10.1183\/23120541.00731-2020","article-title":"Lumacaftor\/ivacaftor changes the lung microbiome and metabolome in cystic fibrosis patients","volume":"7","author":"Neerincx","year":"2021","journal-title":"ERJ Open. Res."},{"key":"ref_191","doi-asserted-by":"crossref","unstructured":"Maule, G., Arosio, D., and Cereseto, A. (2020). Gene Therapy for Cystic Fibrosis: Progress and Challenges of Genome Editing. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21113903"},{"key":"ref_192","doi-asserted-by":"crossref","first-page":"962","DOI":"10.1136\/thoraxjnl-2014-205835","article-title":"Delivery of genes into the CF airway","volume":"69","author":"Gill","year":"2014","journal-title":"Thorax"}],"container-title":["Nutrients"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6643\/15\/3\/479\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T18:07:59Z","timestamp":1760119679000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6643\/15\/3\/479"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1,17]]},"references-count":192,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2023,2]]}},"alternative-id":["nu15030479"],"URL":"https:\/\/doi.org\/10.3390\/nu15030479","relation":{},"ISSN":["2072-6643"],"issn-type":[{"value":"2072-6643","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,1,17]]}}}