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References

  1. Kerem B, Rommens JM, Buchanan JA, et al. Identification of the cystic fibrosis gene: genetic analysis. Science. 1989;245(4922):1073-1080 PMID: 2570460 doi: 10.1126/science.2570460
  2. Kerem E, Corey M, Kerem BS, et al. The relation between genotype and phenotype in cystic fibrosis-analysis of the most common mutation (delta F508). N Engl J Med. 1990;323(22):1517-1522 PMID: 2233932 doi: 10.1056/NEJM199011293232203
  3. Cystic Fibrosis Foundation, Johns Hopkins University, The Hospital for Sick Children. The Clinical and Functional Translation of CFTR (CFTR2); 2022. Available from: http://cftr2.org
  4. Farrell PM, White TB, Ren CL, et al. Diagnosis of cystic fibrosis: consensus guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017;181S:S4-S15 e1
  5. Castellani C, Cuppens H, Macek M Jr, et al. Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice. J Cyst Fibros. 2008;7(3):179-196 PMID: 18456578 doi: 10.1016/j.jcf.2008.03.009
  6. Kristidis P, Bozon D, Corey M, et al. Genetic determination of exocrine pancreatic function in cystic fibrosis. Am J Hum Genet. 1992;50(6):1178-1184 PMID: 1376016
  7. Sosnay PR, Raraigh KS, Gibson RL. Molecular genetics of cystic fibrosis transmembrane conductance regulator: genotype and phenotype. Pediatr Clin North Am. 2016;63(4):585-598 PMID: 27469177 doi: 10.1016/j.pcl.2016.04.002
  8. Walkowiak J, Sands D, Nowakowska A, et al. Early decline of pancreatic function in cystic fibrosis patients with class 1 or 2 CFTR mutations. J Pediatr Gastroenterol Nutr. 2005;40(2):199-201 PMID: 15699697 doi: 10.1097/00005176-200502000-00022
  9. Walkowiak J, Nousia-Arvanitakis S, Agguridaki C, et al. Longitudinal follow-up of exocrine pancreatic function in pancreatic sufficient cystic fibrosis patients using the fecal elastase-1 test. J Pediatr Gastroenterol Nutr. 2003;36(4):474-478 PMID: 12658038
  10. Borowitz D, Parad RB, Sharp JK, et al; Cystic Fibrosis Foundation. Cystic Fibrosis Foundation practice guidelines for the management of infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome during the first two years of life and beyond. J Pediatr. 2009;155(6) (suppl):S106-S116 PMID: 19914443 doi: 10.1016/j.jpeds.2009.09.003
  11. Borowitz D, Robinson KA, Rosenfeld M, et al; Cystic Fibrosis Foundation. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(6)(suppl):S73-S93 PMID: 19914445 doi: 10.1016/j.jpeds.2009.09.001
  12. Li Z, Kosorok MR, Farrell PM, et al. Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis. JAMA. 2005;293(5):581-588 PMID: 15687313 doi: 10.1001/jama.293.5.581
  13. Carlyle BE, Borowitz DS, Glick PL. A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon. J Pediatr Surg. 2012;47(4):772-781 PMID: 22498395 doi: 10.1016/j.jpedsurg.2012.02.019
  14. Doulgeraki A, Petrocheilou A, Petrocheilou G, Chrousos G, Doudounakis SE, Kaditis AG. Body composition and lung function in children with cystic fibrosis and meconium ileus. Eur J Pediatr. 2017;176(6):737-743 PMID: 28409283 doi: 10.1007/s00431-017-2906-z
  15. Padoan R, Cirilli N, Falchetti D, Cesana BM; Meconium Ileus Project Study Group. Risk factors for adverse outcome in infancy in meconium ileus cystic fibrosis infants: A multicentre Italian study. J Cyst Fibros. 2019;18(6):863-868 PMID: 31353045 doi: 10.1016/j.jcf.2019.07.003
  16. Lai HC, Kosorok MR, Laxova A, Davis LA, FitzSimmon SC, Farrell PM. Nutritional status of patients with cystic fibrosis with meconium ileus: a comparison with patients without meconium ileus and diagnosed early through neonatal screening. Pediatrics. 2000;105(1 Pt 1):53-61 PMID: 10617704 doi: 10.1542/peds.105.1.53
  17. Borowitz D, Lubarsky B, Wilschanski M, et al. Nutritional status improved in cystic fibrosis patients with the G551D mutation after treatment with ivacaftor. Dig Dis Sci. 2016;61(1):198-207 PMID: 26250833 doi: 10.1007/s10620-015-3834-2
  18. Ramsey BW, Davies J, McElvaney NG, et al; VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365(18):1663-1672 PMID: 22047557 doi: 10.1056/NEJMoa1105185
  19. Nichols DP, Donaldson SH, Frederick CA, et al. PROMISE: working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy. J Cyst Fibros. 2021;20(2):205-212 PMID: 33619012 doi: 10.1016/j.jcf.2021.02.003
  20. Middleton PG, Mall MA, Dřevínek P, et al; VX17-445-102 Study Group. Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele. N Engl J Med. 2019;381(19):1809-1819 PMID: 31697873 doi: 10.1056/NEJMoa1908639
  21. Nichols DP, Paynter AC, Heltshe SL, et al; PROMISE Study group. Clinical effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: a clinical trial. Am J Respir Crit Care Med. 2022;205(5):529-539 PMID: 34784492 doi: 10.1164/rccm.202108-1986OC
  22. Zemanick ET, Taylor-Cousar JL, Davies J, et al. 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. Am J Respir Crit Care Med. 2021;203(12):1522-1532 PMID: 33734030 doi: 10.1164/rccm.202102-0509OC
  23. Goetz DM, Savant AP. Review of CFTR modulators 2020. Pediatr Pulmonol. 2021;56(12):3595-3606 PMID: 34407318 doi: 10.1002/ppul.25627
  24. Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013;162(3):530-535.e1 PMID: 23062247 doi: 10.1016/j.jpeds.2012.08.040
  25. Lai HJ, Shoff SM, Farrell PM; Wisconsin Cystic Fibrosis Neonatal Screening Group. 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. Pediatrics. 2009;123(2):714-722 PMID: 19171643 doi: 10.1542/peds.2007-3089
  26. Sanders DB, Fink A, Mayer-Hamblett N, et al. Early life growth trajectories in cystic fibrosis are associated with pulmonary function at age 6 years. J Pediatr. 2015;167(5):1081-8.e1 PMID: 26340874 doi: 10.1016/j.jpeds.2015.07.044
  27. Scully KJ, Jay LT, Freedman S, et al. The relationship between body composition, dietary intake, physical activity, and pulmonary status in adolescents and adults with cystic fibrosis. Nutrients.2022;14(2):310 PMID: 35057491 doi: 10.3390/nu14020310
  28. Davies PSW, Erskine JM, Hambidge KM, Accurso FJ. Longitudinal investigation of energy expenditure in infants with cystic fibrosis. Eur J Clin Nutr. 2002;56(10): 940-946 PMID: 12373612 doi: 10.1038/sj.ejcn.1601441
  29. Moudiou T, Galli-Tsinopoulou A, Vamvakoudis E, Nousia-Arvanitakis S. Resting energy expenditure in cystic fibrosis as an indicator of disease severity. J Cyst Fibros. 2007;6(2):131-136 PMID: 16844432 doi: 10.1016/j.jcf.2006.06.001
  30. Thomson MA, Wilmott RW, Wainwright C, Masters B, Francis PJ, Shepherd RW. Resting energy expenditure, pulmonary inflammation, and genotype in the early course of cystic fibrosis. J Pediatr. 1996;129(3):367-373 PMID: 8804325 doi: 10.1016/S0022-3476(96)70068-9
  31. Munck A. Cystic fibrosis: evidence for gut inflammation. Int J Biochem Cell Biol. 2014;52:180-183 PMID: 24548777 doi: 10.1016/j.biocel.2014.02.005
  32. Nichols DP, Chmiel JF. Inflammation and its genesis in cystic fibrosis. Pediatr Pulmonol. 2015;50(S40)(suppl 40):S39-S56 PMID: 26335954 doi: 10.1002/ppul.23242
  33. Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H; Clinical Practice Guidelines on Growth and Nutrition Subcommittee; Ad Hoc Working Group. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108(5):832-839 PMID: 18442507 doi: 10.1016/j.jada.2008.02.020
  34. Beswick DM, Humphries SM, Balkissoon CD, et al. Olfactory dysfunction in people with cystic fibrosis with at least one copy of F508del. Int Forum Allergy Rhinol. 2022;12(7):963-966 PMID: 34908251
  35. Beswick DM, Humphries SM, Balkissoon CD, et al. Olfactory dysfunction in cystic fibrosis: impact of CFTR modulator therapy. J Cyst Fibros. 2022;21(2): e141-e147 PMID: 34598881 doi: 10.1016/j.jcf.2021.09.014
  36. Gelfond D, Borowitz D. Gastrointestinal complications of cystic fibrosis. Clin Gastroenterol Hepatol. 2013;11(4):333-342 PMID: 23142604 doi: 10.1016/j.cgh.2012.11.006
  37. Jaudszus A, Zeman E, Jans T, et al. Validity and reliability of a novel multimodal questionnaire for the assessment of abdominal symptoms in people with cystic fibrosis (CFAbd-Score). Patient. 2019;12(4):419-428 PMID: 30887269 doi: 10.1007/s40271-019-00361-2
  38. Wouthuyzen-Bakker M, Bodewes FAJA, Verkade HJ. Persistent fat malabsorption in cystic fibrosis; lessons from patients and mice. J Cyst Fibros. 2011;10(3):150-158 PMID: 21459688 doi: 10.1016/j.jcf.2011.03.008
  39. Gelfond D, Ma C, Semler J, Borowitz D. Intestinal pH and gastrointestinal transit profiles in cystic fibrosis patients measured by wireless motility capsule. Dig Dis Sci. 2013;58(8):2275-2281 PMID: 22592630 doi: 10.1007/s10620-012-2209-1
  40. Gelfond D, Heltshe S, Ma C, et al. Impact of CFTR modulation on intestinal pH, motility, and clinical outcomes in patients with cystic fibrosis and the G551D mutation. Clin Transl Gastroenterol. 2017;8(3):e81 PMID: 28300821 doi: 10.1038/ctg.2017.10
  41. Moheet A, Moran A. New concepts in the pathogenesis of cystic fibrosis-related diabetes. J Clin Endocrinol Metab. 2022;107(6):1503-1509 PMID: 35106591 doi: 10.1210/clinem/dgac020
  42. Nielsen BU, Faurholt-Jepsen D, Oturai PS, et al. Associations between glucose tolerance, insulin secretion, muscle and fat mass in cystic fibrosis. Clin Med Insights Endocrinol Diabetes. 2021;14:11795514211038259 PMID: 34413690 doi: 10.1177/11795514211038259
  43. Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al; Cystic Fibrosis Foundation Pulmonary Clinical Practice Guidelines Committee. Cystic Fibrosis Foundation pulmonary guideline. pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection. Ann Am Thorac Soc. 2014;11(10): 1640-1650 PMID: 25549030 doi: 10.1513/AnnalsATS.201404-166OC
  44. Ramsey BW, Farrell PM, Pencharz P; The Consensus Committee. Nutritional assessment and management in cystic fibrosis: a consensus report. Am J Clin Nutr. 1992;55(1):108-116 PMID: 1728810 doi: 10.1093/ajcn/55.1.108
  45. Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2002;35(3):246-259 PMID: 12352509
  46. Lahiri T, Hempstead SE, Brady C, et al. Clinical practice guidelines from the Cystic Fibrosis Foundation for preschoolers with cystic fibrosis. Pediatrics. 2016;137(4):e20151784 PMID: 27009033 doi: 10.1542/peds.2015-1784
  47. McDonald CM, Alvarez JA, Bailey J, et al. Academy of Nutrition and Dietetics: 2020 cystic fibrosis evidence analysis center evidence-based nutrition practice guideline. J Acad Nutr Diet. 2021;121(8):1591-1636.e3 PMID: 32565399 doi: 10.1016/j.jand.2020.03.015
  48. Vanga RR, Tansel A, Sidiq S, El-Serag HB, Othman MO. Diagnostic performance of measurement of fecal elastase-1 in detection of exocrine pancreatic insufficiency: systematic review and meta-analysis. Clin Gastroenterol Hepatol. 2018;16(8):1220-1228.e4 PMID: 29374614 doi: 10.1016/j.cgh.2018.01.027
  49. Carroccio A, Pardo F, Montalto G, et al. Effectiveness of enteric-coated preparations on nutritional parameters in cystic fibrosis. A long-term study. Digestion. 1988;41(4):201-206 PMID: 3243379 doi: 10.1159/000199788
  50. Durie P, Kalnins D, Ellis L. Uses and abuses of enzyme therapy in cystic fibrosis. J R Soc Med. 1998;91(Suppl 34)(suppl 34):2-13 PMID: 9709382 doi: 10.1177/014107689809134S02
  51. Ng C, Major G, Smyth AR. A systematic Cochrane Review of the timing of pancreatic enzyme replacement therapy (PERT) in cystic fibrosis. Paediatr Respir Rev. 2021;40:44-45 PMID: 34635420 doi: 10.1016/j.prrv.2021.09.002
  52. Taylor CJ, Thieroff-Ekerdt R, Shiff S, Magnus L, Fleming R, Gommoll C. Comparison of two pancreatic enzyme products for exocrine insufficiency in patients with cystic fibrosis. J Cyst Fibros. 2016;15(5):675-680 PMID: 27013382 doi: 10.1016/j.jcf.2016.02.010
  53. Borowitz D, Durie PR, Clarke LL, et al. Gastrointestinal outcomes and confounders in cystic fibrosis. J Pediatr Gastroenterol Nutr. 2005;41(3):273-285 PMID: 16131979 doi: 10.1097/01.mpg.0000178439.64675.8d
  54. Heijerman HG, Lamers CB, Bakker W. Omeprazole enhances the efficacy of pancreatin (pancrease) in cystic fibrosis. Ann Intern Med. 1991;114(3):200-201 PMID: 1984743 doi: 10.7326/0003-4819-114-3-200
  55. Sander-Struckmeier S, Beckmann K, Janssen-van Solingen G, Pollack P. Retrospective analysis to investigate the effect of concomitant use of gastric acid-suppressing drugs on the efficacy and safety of pancrelipase/ pancreatin (CREON®) in patients with pancreatic exocrine insufficiency. Pancreas. 2013;42(6):983-989 PMID: 23587850 doi: 10.1097/MPA.0b013e31828784ef
  56. Dorsey J, Gonska T. Bacterial overgrowth, dysbiosis, inflammation, and dysmotility in the Cystic Fibrosis intestine. J Cyst Fibros. 2017;16(suppl 2): S14-S23 PMID: 28986022 doi: 10.1016/j.jcf.2017.07.014
  57. Davies JC, Wainwright CE, Sawicki GS, et al. Ivacaftor in infants aged 4 to <12 months with cystic fibrosis and a gating mutation. results of a two-part phase 3 clinical trial. Am J Respir Crit Care Med. 2021;203(5):585-593 PMID: 33023304 doi: 10.1164/rccm.202008-3177OC
  58. Munce D, Lim M, Akong K. Persistent recovery of pancreatic function in patients with cystic fibrosis after ivacaftor. Pediatr Pulmonol. 2020;55(12): 3381-3383 PMID: 32910556 doi: 10.1002/ppul.25065
  59. Nichols AL, Davies JC, Jones D, Carr SB. Restoration of exocrine pancreatic function in older children with cystic fibrosis on ivacaftor. Paediatr Respir Rev. 2020;35:99-102 PMID: 32386958 doi: 10.1016/j.prrv.2020.04.003
  60. Rosenfeld M, Cunningham S, Harris WT, et al; KLIMB study group. An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2-5 years (KLIMB). J Cyst Fibros. 2019;18(6):838-843 PMID: 31053538 doi: 10.1016/j.jcf.2019.03.009
  61. Borowitz DS, Grand RJ, Durie PR; Consensus Committee. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. J Pediatr. 1995;127(5):681-684 PMID: 7472816 doi: 10.1016/S0022-3476(95)70153-2
  62. Borowitz D, Gelfond D, Maguiness K, Heubi JE, Ramsey B. Maximal daily dose of pancreatic enzyme replacement therapy in infants with cystic fibrosis: a reconsideration. J Cyst Fibros. 2013;12(6):784-785 PMID: 23809508 doi: 10.1016/j.jcf.2013.05.011
  63. Brady MS, Rickard K, Yu PL, Eigen H. Effectiveness of enteric coated pancreatic enzymes given before meals in reducing steatorrhea in children with cystic fibrosis. J Am Diet Assoc. 1992;92(7):813-817 PMID: 1624649 doi: 10.1016/S0002-8223(21)00735-5
  64. Lai HC, Corey M, FitzSimmons S, Kosorok MR, Farrell PM. Comparison of growth status of patients with cystic fibrosis between the United States and Canada. Am J Clin Nutr. 1999;69(3):531-538 PMID: 10075341 doi: 10.1093/ajcn/69.3.531
  65. Lai HC, Kosorok MR, Sondel SA, et al. Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: evaluation of various criteria used to identify malnutrition. J Pediatr. 1998;132(3 Pt 1): 478-485 PMID: 9544905 doi: 10.1016/S0022-3476(98)70024-1
  66. Rhodes B, Nash EF, Tullis E, et al. Prevalence of dyslipidemia in adults with cystic fibrosis. J Cyst Fibros. 2010;9(1):24-28 PMID: 19875344 doi: 10.1016/j.jcf.2009.09.002
  67. Nowak JK, Wykrętowicz A, Mądry E, et al. Preclinical atherosclerosis in cystic fibrosis: two distinct presentations are related to pancreatic status. J Cyst Fibros. 2022;21(1):26-33 PMID: 34253491 doi: 10.1016/j.jcf.2021.06.010
  68. Filigno SS, Robson SM, Szczesniak RD, et al. Macronutrient intake in preschoolers with cystic fibrosis and the relationship between macronutrients and growth. J Cyst Fibros. 2017;16(4):519-524 PMID: 28185886 doi: 10.1016/j.jcf.2017.01.010
  69. White H, Morton AM, Peckham DG, Conway SP. Dietary intakes in adult patients with cystic fibrosis-do they achieve guidelines? J Cyst Fibros. 2004;3(1):1-7 PMID: 15463880 doi: 10.1016/j.jcf.2003.12.002
  70. Woestenenk JW, Castelijns SJ, van der Ent CK, Houwen RH. Dietary intake in children and adolescents with cystic fibrosis. Clin Nutr. 2014;33(3):528-532 PMID: 23920501 doi: 10.1016/j.clnu.2013.07.011
  71. Benabdeslam H, Garcia I, Bellon G, Gilly R, Revol A. Biochemical assessment of the nutritional status of cystic fibrosis patients treated with pancreatic enzyme extracts. Am J Clin Nutr. 1998;67(5):912-918 PMID: 9583849 doi: 10.1093/ajcn/67.5.912
  72. Marcus MS, Sondel SA, Farrell PM, et al. Nutritional status of infants with cystic fibrosis associated with early diagnosis and intervention. Am J Clin Nutr. 1991;54(3):578-585 PMID: 1877513 doi: 10.1093/ajcn/54.3.578
  73. Cystic Fibrosis Foundation. Cystic Fibrosis Foundation Patient Registry: 2020 Annual Data Report. Cystic Fibrosis Foundation; 2020
  74. Figueroa V, Milla C, Parks EJ, Schwarzenberg SJ, Moran A. Abnormal lipid concentrations in cystic fibrosis. Am J Clin Nutr. 2002;75(6):1005-1011 PMID: 12036806 doi: 10.1093/ajcn/75.6.1005
  75. Poore TS, Taylor-Cousar JL, Zemanick ET. Cardiovascular complications in cystic fibrosis: A review of the literature. J Cyst Fibros. 2022;21(1):18-25 PMID: 34140249 doi: 10.1016/j.jcf.2021.04.016
  76. Christophe AB, Warwick WJ, Holman RT. Serum fatty acid profiles in cystic fibrosis patients and their parents. Lipids. 1994;29(8):569-575 PMID: 7990664 doi: 10.1007/BF02536629
  77. Strandvik B. Fatty acid metabolism in cystic fibrosis. Prostaglandins Leukot Essent Fatty Acids. 2010;83(3):121-129 PMID: 20673710 doi: 10.1016/j.plefa.2010.07.002
  78. Maqbool A, Schall JI, Garcia-Espana JF, Zemel BS, Strandvik B, Stallings VA. Serum linoleic acid status as a clinical indicator of essential fatty acid status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2008;47(5):635-644 PMID: 18955866 doi: 10.1097/MPG.0b013e31817fb76b
  79. Shoff SM, Ahn HY, Davis L, Lai H; Wisconsin CF Neonatal Screening Group. Temporal associations among energy intake, plasma linoleic acid, and growth improvement in response to treatment initiation after diagnosis of cystic fibrosis. Pediatrics. 2006;117(2):391-400 PMID: 16452358 doi: 10.1542/peds.2004-2832
  80. Strandvik B. Nutrition in cystic fibrosis-some notes on the fat recommendations. Nutrients.2022;14(4):853 PMID: 35215502 doi: 10.3390/nu14040853
  81. Feranchak AP, Sontag MK, Wagener JS, Hammond KB, Accurso FJ, Sokol RJ. Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen. J Pediatr. 1999;135(5):601-610 PMID: 10547249 doi: 10.1016/S0022-3476(99)70059-4
  82. Rana M, Wong-See D, Katz T, et al. Fat-soluble vitamin deficiency in children and adolescents with cystic fibrosis. J Clin Pathol. 2014;67(7):605-608 PMID: 24711511 doi: 10.1136/jclinpath-2013-201787
  83. Anabtawi A, Le T, Putman M, Tangpricha V, Bianchi ML. Cystic fibrosis bone disease: Pathophysiology, assessment and prognostic implications. J Cyst Fibros. 2019;18(suppl 2):S48-S55 PMID: 31679729 doi: 10.1016/j.jcf.2019.08.018
  84. Wilfond BS, Farrell PM, Laxova A, Mischler E. Severe hemolytic anemia associated with vitamin E deficiency in infants with cystic fibrosis. Implications for neonatal screening. Clin Pediatr (Phila). 1994;33(1):2-7 PMID: 8156723 doi: 10.1177/000992289403300101
  85. Ozçelik U, Göçmen A, Kiper N, Coşkun T, Yilmaz E, Ozgüç M. Sodium chloride deficiency in cystic fibrosis patients. Eur J Pediatr. 1994;153(11):829-831 PMID: 7843198 doi: 10.1007/BF01972892
  86. Krebs NF, Sontag M, Accurso FJ, Hambidge KM. Low plasma zinc concentrations in young infants with cystic fibrosis. J Pediatr. 1998;133(6):761-764 PMID: 9842040 doi: 10.1016/S0022-3476(98)70147-7
  87. Krebs NF, Westcott JE, Arnold TD, et al. Abnormalities in zinc homeostasis in young infants with cystic fibrosis. Pediatr Res. 2000;48(2):256-261 PMID: 10926304 doi: 10.1203/00006450-200008000-00022
  88. von Drygalski A, Biller J. Anemia in cystic fibrosis: incidence, mechanisms, and association with pulmonary function and vitamin deficiency. Nutr Clin Pract. 2008;23(5):557-563 PMID: 18849562 doi: 10.1177/0884533608323426
  89. Evans DC, Corkins MR, Malone A, et al; ASPEN Malnutrition Committee. The use of visceral proteins as nutrition markers: an ASPEN position paper. Nutr Clin Pract. 2021;36(1):22-28 PMID: 33125793 doi: 10.1002/ncp.10588
  90. Turck D, Braegger CP, Colombo C, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016;35(3):557-577 PMID: 27068495 doi: 10.1016/j.clnu.2016.03.004
  91. Ellis L, Kalnins D, Corey M, Brennan J, Pencharz P, Durie P. Do infants with cystic fibrosis need a protein hydrolysate formula? A prospective, randomized, comparative study. J Pediatr. 1998;132(2):270-276 PMID: 9506640 doi: 10.1016/S0022-3476(98)70444-5
  92. Farrell PM, Mischler EH, Sondel SA, Palta M. Predigested formula for infants with cystic fibrosis. J Am Diet Assoc. 1987;87(10):1353-1356 PMID: 3309012 doi: 10.1016/S0002-8223(21)03321-6
  93. Baker RD, Greer FR; Committee on Nutrition American Academy of Pediatrics. Diagnosis and prevention of iron deficiency and iron-deficiency anemia in infants and young children (0-3 years of age). Pediatrics. 2010;126(5):1040-1050 PMID: 20923825 doi: 10.1542/peds.2010-2576
  94. Smyth RL. Fibrosing colonopathy in cystic fibrosis. Arch Dis Child. 1996;74(5): 464-468 PMID: 8669970 doi: 10.1136/adc.74.5.464
  95. Powers SW, Patton SR, Byars KC, et al. Caloric intake and eating behavior in infants and toddlers with cystic fibrosis. Pediatrics. 2002;109(5):E75-E5 PMID: 11986481 doi: 10.1542/peds.109.5.e75
  96. Stark LJ, Opipari-Arrigan L, Quittner AL, Bean J, Powers SW. The effects of an intensive behavior and nutrition intervention compared to standard of care on weight outcomes in CF. Pediatr Pulmonol. 2011;46(1):31-35 PMID: 20812240 doi: 10.1002/ppul.21322
  97. Burton JS, Hachem C, Abraham JM. Luminal gastrointestinal manifestations of cystic fibrosis. Curr Gastroenterol Rep.2021;23(3):4 PMID: 33758994 doi: 10.1007/s11894-021-00806-5
  98. Dana J, Debray D, Beaufrère A, et al. Cystic fibrosis-related liver disease: clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies. J Hepatol. 2022;76(2):420-434 PMID: 34678405 doi: 10.1016/j.jhep.2021.09.042
  99. Barker DH, Quittner AL. Parental depression and pancreatic enzymes adherence in children with cystic fibrosis. Pediatrics. 2016;137(2):e20152296 PMID: 26738883 doi: 10.1542/peds.2015-2296
  100. Quittner AL, Saez-Flores E, Barton JD. The psychological burden of cystic fibrosis. Curr Opin Pulm Med. 2016;22(2):187-191 PMID: 26814144 doi: 10.1097/MCP.0000000000000244
  101. Schwarzenberg SJ, Hempstead SE, McDonald CM, et al. Enteral tube feeding for individuals with cystic fibrosis: cystic Fibrosis Foundation evidence-informed guidelines. J Cyst Fibros. 2016;15(6):724-735 PMID: 27599607 doi: 10.1016/j.jcf.2016.08.004
  102. Freedman S, Orenstein D, Black P, et al. Increased fat absorption from enteral formula through an in-line digestive cartridge in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2017;65(1):97-101 PMID: 28471913 doi: 10.1097/MPG.0000000000001617
  103. Stevens J, Wyatt C, Brown P, Patel D, Grujic D, Freedman SD. Absorption and safety with sustained use of Relizorb evaluation (ASSURE) study in patients with cystic fibrosis receiving enteral feeding. J Pediatr Gastroenterol Nutr. 2018;67(4):527-532 PMID: 30074573 doi: 10.1097/MPG.0000000000002110
  104. Powers SW, Stark LJ, Chamberlin LA, et al. Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial. JAMA Pediatr. 2015;169(5):e150636 PMID: 25938655 doi: 10.1001/jamapediatrics.2015.0636
  105. Tangpricha V, Kelly A, Stephenson A, et al; Cystic Fibrosis Foundation Vitamin D Evidence-Based Review Committee. 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. J Clin Endocrinol Metab. 2012;97(4):1082-1093 PMID: 22399505 doi: 10.1210/jc.2011-3050