This Academy member benefit temporarily has been made public to allow all practitioners access to content that may assist in patient care during the national pandemic response. Click here for information on joining the Academy. 

Cystic Fibrosis

CF: References (2020)

Cystic Fibrosis Guideline 2019 References

References used in this guideline are listed below in alphabetical order. The specific references are available in the Supporting Evidence section of each recommendation under Guideline Major Recommendations & Supporting Evidence

  • Abramowitz MK, Hall CB, Amodu A, Sharma D, Androga L, Hawkins M. Muscle mass, BMI, and mortality among adults in the United States: A population-based cohort study. PLoS One. 2018;13(4):e0194697.
  • Academy of Nutrition and Dietetics. In individuals with CF, what is the longitudinal relationship (at least 3 months) between weight and growth parameters and FEV1?. Evidence Analysis Library Cystic Fibrosis Systematic Review. https://www.andeal.org/topic.cfm?menu=5876&cat=5979. Updated August 2019. Accessed September 10, 2019.
  • Academy of Nutrition and DIetetics. In individuals with CF, what is the relationship between nutrition parameters and hard outcomes? Evidence Analysis Library Cystic Fibrosis Systematic Review web site. https://www.andeal.org/topic.cfm?menu=5876&cat=5979. Published 2019. Accessed September 25, 2019.
  • Academy of Nutrition and Dietetics. In individuals with CF, what is the validity and reliability or nutrition assessment methods compared to reference standards, as measured by validity and/or reliability studies? Evidence Analysis Library Cystic Fibrosis Systematic Review web site. https://www.andeal.org/topic.cfm?menu=5876&cat=5942. Published 2019. Accessed September 26, 2019.
  • Ackland TR, Lohman TG, Sundgot-Borgen J, et al. Current status of body composition assessment in sport: review and position statement on behalf of the ad hoc research working group on body composition health and performance, under the auspices of the I.O.C. Medical Commission. Sports Med. 2012;42(3):227-249.
  • Alvarez JA, Ziegler TR, Millson EC, Stecenko AA. Body composition and lung function in cystic fibrosis and their association with adiposity and normal-weight obesity. Nutrition. 2016;32(4):447-452.
  • Alves C, Lima D, Cardeal M, Santana A. Dyslipidemia in racially admixtured children with cystic fibrosis. Indian J Endocrinol Metab. 2012;16(4):585-588.
  • Amadori A, Antonelli A, Balteri I, Schreiber A, Bugiani M, De Rose V. Recurrent exacerbations affect FEV(1) decline in adult patients with cystic fibrosis. Respir Med. 2009;103(3):407-413.
  • Aris RM, Merkel PA, Bachrach LK, Borowitz DS, et al. Consensus statement: guide to bone health and disease in cystic fibrosis. J. CLin Epidemiol Metab. 2005; 90:1888-1896.
  • Becerra-Tomas N, Blanco Mejia S, Viguiliouk E, et al. Mediterranean diet, cardiovascular disease and mortality in diabetes: A systematic review and meta-analysis of prospective cohort studies and randomized clinical trials. Crit Rev Food Sci Nutr. 2019:1-21.
  • Bellissimo MP, Zhang I, Ivie EA, et al. Visceral adipose tissue is associated with poor diet quality and higher fasting glucose in adults with cystic fibrosis. J Cyst Fibros. 2019;18(3):430-435.
  • Borga M, West J, Bell JD, et al. Advanced body composition assessment: from body mass index to body composition profiling. J Investig Med. 2018;66(5):1-9.
  • Borowitz D, Baker RD, Stallings, VA. Consensus report on nutriiton for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2002; 35:246-259.
  • 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 Sciences. 2016;61(1):198-207
  • Borowitz D, Robinson KA, Rosenfeld M, et al. Cystic Fibrosis FOundation evidence-based guidelines for the management of infants with cystic fibrosis. J Pediatr. 2009; 155:S73-S93.
  • Bryon M, Wallis C. Parents as equal partners in the paediatric cystic fibrosis multidisciplinary team. J R Soc Med. 2011;104 Suppl 1:S30-35.
  • Buckinx F, Landi F, Cesari M, et al. Pitfalls in the measurement of muscle mass: a need for a reference standard. J Cachexia Sarcopenia Muscle .2018;9(2):269-278.
  • Calvo-Lerma J, Hulst J, Boon M, et al. The relative contribution of food groups to macronutrient intake in children with cystic fibrosis: a European Multicenter Assessment. J Acad Nutr Diet. 2019;119(8):1305-1319.
  • Casas R, Castro-Barquero S, Estruch R, Sacanella E. Nutrition and cardiovascular health. Int J Mol Sci. 2018;19(12).
  • Chamnan P, Shine BSF, Haworth CS, Bilton D, Adler A, I. a. Diabetes as a determinant of mortality in cystic fibrosis. Diabetes Care. 2010;33(2):311-316.
  • Charatsi AM, Dusser P, Freund R, et al. Bioelectrical impedance in young patients with cystic fibrosis: Validation of a specific equation and clinical relevance. J Cystic Fibros. 2016;15(6):825-833.
  • Ciofu O, Lykkesfeldt J. Antioxidant supplementation for lung disease in cystic fibrosis. Cochrane Database Syst Rev. 2014:(8)-2014 Aug 2007.
  • Coderre L, Fadainia C, Belson L, et al. LDL-cholesterol and insulin are independently associated with body mass index in adult cystic fibrosis patients. J Cystic Fibros. 2012;11(5):393-397.
  • Colombo C, Bennato V, Costantini D, et al. Dietary and circulating polyunsaturated fatty acids in cystic fibrosis: are they related to clinical outcomes? J Pediatr Gastroenterol Nutr. 2006;43(5):660-665.
  • Colombo C, Costantini D, Zazzeron L, et al. Benefits of breastfeeding in cystic fibrosis: a single-centre follow-up survey. Acta Paediatrica (Oslo, Norway : 1992). 2007;96(8):1228-1232.
  • Corey M, McLaughlin FJ, Williams M, Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 1988;41(6):583-591.
  • Cross CE, Reverri EJ, Morrissey BM. Joining the crowd: cystic fibrosis and cardiovascular disease risk factors. Chest. 2013;143(4):882-884.
  • Cystic Fibrosis Foundation Patient Registry. 2017 Annual Data Report. Available at: https://www.cff.org/Research/Researcher-Resources/Patient-Registry/2017-Patient-Registry-Annual-Data-Report.pdf. Accessed October 15, 2019.
  • Cystic Fibrosis Foundation. Care Centers. Cystic Fibrosis Foundation. https://www.cff.org/Care/Care-Centers/. Published 2019. Accessed October 15, 2019.
  • Cystic Fibrosis Foundation. Chapter 1. Preventive and Maintenance Care for the Patient With Cystic Fibrosis. In. Clicial Practice Guidelines for Cystic Fibrosis "White Binder". 1997; Bethesda, MD.
  • Cystic Fibrosis Foundation. Nutritional Basics. Cystic Fibrosis Foundation. https://www.cff.org/Life-With-CF/Daily-Life/Fitness-and-Nutrition/Nutrition/Getting-Your-Nutrients/Nutritional-Basics/. Accessed September 25, 2019.
  • Cystic Fibrosis Foundation. Our Mission. https://www.cff.org/About-Us/About-the-Cystic-Fibrosis-Foundation/Our-Mission/. Published 2019. Accessed September 25, 2019.
  • Cystic Fibrosis Foundation. Cystic Fibrosis Care Center Administration, Guidelines for Personnel Time Allotments to CF Centers. Cystic Fibrosis Foundation. Accessed 2019.
  • Darrah R, Nelson R, Damato EG, Decker M, Matthews A, Hodges CA. Growth deficiency in cystic fibrosis Is observable at birth and predictive of early pulmonary function. Biol Res Nurs. 2016;18(5):498-504.
  • Davies JC, Wainwright CE, Canny GJ, et al. Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation. Am J Respir Criti Care Med. 2013;187(11):1219-1225.
  • De Boeck K, Munck A, Walker S, et al. Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation. J Cyst Fibros. 2014;13(6):674-680.
  • Declercq D, Van Biervliet S, Robberecht E. Nutrition and pancreatic enzyme intake in patients with cystic fibrosis with distal intestinal obstruction syndrome. Nutr Clin Pract. 2015;30(1):134-137.
  • Demerath EW, Fields DA. Body composition assessment in the infant. Am J Hum Biol. 2014;26(3):291-304.
  • Division of Nutrition, Physical Activity, and Obesity, National Center for Chronic Disease Prevention and Health Promotion. Breastfeeding. Centers for Disease Control and Prevention. https://www.cdc.gov/breastfeeding/about-breastfeeding/why-it-matters.html. Published 2019. Accessed September 11, 2019.
  • Edgeworth D, Keating D, Ellis M, et al. Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: a double-blind, placebo-controlled, randomized, cross-over study with ivacaftor treatment. Clin Sci (Lon). 2017;131(15):2037-2045.
  • Elborn JS, Ramsey BW, Boyle MP, et al. Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis. Lancet Respir Med. 2016;4(8):617-626.
  • Elborn JS. Cystic fibrosis. Lancet. 2016;388(10059):2519-2531.
  • Emerson J, Rosenfeld M, McNamara S, Ramsey B, Gibson RL. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatric Pulmonol. 2002;34(2):91-100.
  • Engelen MPKJ, Schroder R, Van der Hoorn K, Deutz NEP, Com G. Use of body mass index percentile to identify fat-free mass depletion in children with cystic fibrosis. Clin Nutr (Edinburgh, Scotland). 2012;31(6):927-933.
  • Esposito K, Maiorino MI, Bellastella G, Chiodini P, Panagiotakos D, Giugliano D. A journey into a Mediterranean diet and type 2 diabetes: a systematic review with meta-analyses. BMJ Open. 2015;5(8):e008222.
  • 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.
  • Filigno SS, Robson SM, Szczesniak RD, et al. Macronutrient intake in preschoolers with cystic fibrosis and the relationship between macronutrients and growth. J Cystic Fibros. 2017;16(4):519-524.
  • Flume PA, Liou TG, Borowitz DS, et al. Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation. Chest. 2012;142(3):718-724.
  • Forte GC, Pereira JS, Drehmer M, Simon MISdS. Anthropometric and dietary intake indicators as predictors of pulmonary function in cystic fibrosis patients. J Bras Pneumol. 2012;38(4):470-476.
  • Foundation CF. Your CF Care Team. Cystic Fibrosis Foundation. https://www.cff.org/Care/Your-CF-Care-Team/. Accessed October 15, 2019.
  • Frohnert BI, Ode KL, Moran A, et al. Impaired fasting glucose in cystic fibrosis. Diabetes Care. 2010;33(12):2660-2664.
  • George PM, Banya W, Pareek N, et al. Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007. BMJ (Clinical research ed). 2011;342(8900488):d1008.
  • Gonzalez Jimenez D, Munoz-Codoceo R, Garriga-Garcia M, et al. [Excess weight in patients with cystic fibrosis: is it always beneficial?]. Nutricion Hospitalaria. 2017;34(3):578-583.
  • Gordon CM, Anderson EJ, Herlyn K, et al. Nutrient status of adults with cystic fibrosis. J Am Diet Assoc. 2007;107(12_:2114-9.
  • Goss CH, Sykes J, Stanojevic S, et al. Comparison of nutrition and lung function outcomes in patients with cystic fibrosis living in Canada and the United States. Am J Respir Crit Care Med. 2018;197(6):768-775.
  • Grosso G, Bella F, Godos J, et al. Possible role of diet in cancer: systematic review and multiple meta-analyses of dietary patterns, lifestyle factors, and cancer risk. Nutr Rev. 2017;75(6):405-419.
  • Grummer-Strawn LM, Reinold C, Krebs NF. Use of World Health Organization and CDC growth charts for children aged 0-59 months in the United States. MMWR Recomm Rep. 2010;59(Rr-9):1-15.
  • Hadjiliadis D, Khoruts A, Zauber AG, Hempstead SE, Maisonneuve P, Lowenfels AB. Cystic Fibrosis Colorectal Cancer Screening Consensus Recommendations. Gastroenterol. 2018;154(3):736-745.e714.
  • Hanna RM, Weiner DJ. Overweight and obesity in patients with cystic fibrosis: a center-based analysis. Pediatr Pulmonol. 2015;50(1):35-41.
  • Hofer M, Schmid C, Benden C, et al. Diabetes mellitus and survival in cystic fibrosis patients after lung transplantation. J Cystic Fibros. 2012;11(2):131-136.
  • Hollander FM, De Roos NM, De Vries JHM, Van Berkhout FT. Assessment of nutritional status in adult patients with cystic fibrosis: whole-body bioimpedance vs body mass index, skinfolds, and leg-to-leg bioimpedance. J Am Diet Assoc. 2005;105(4):549-555.
  • Hollander FM, Kok A, de Roos NM, Belle-van Meerkerk G, van de Graaf EA. Prediction equations underestimate resting energy expenditure in patients with end-stage cystic fibrosis. Nutr Clinical Pract. 2017;32(1):116-121.
  • Hollander FM, van Pierre DD, de Roos NM, van de Graaf EA, Iestra JA. Effects of nutritional status and dietetic interventions on survival in cystic fibrosis patients before and after lung transplantation. J Cystic Fibros. 2014;13(2):212-218.
  • Hosseini B, Berthon BS, Saedisomeolia A, et al. Effects of fruit and vegetable consumption on inflammatory biomarkers and immune cell populations: a systematic literature review and meta-analysis. Am J Clin Nutr. 2018;108(1):136-155.
  • Institute for Clinical and Economic Review. Modulator Treatments for Cystic Fibrosis: Effectiveness and Value: a Evidence Report. 2018:228.
  • Ionescu AA, Evans WD, Pettit RJ, Nixon LS, Stone MD, Shale DJ. Hidden depletion of fat-free mass and bone mineral density in adults with cystic fibrosis. Chest. 2003;124(6):2220-2228.
  • Ionescu AA, Nixon LS, Luzio S, et al. Pulmonary function, body composition, and protein catabolism in adults with cystic fibrosis. Am J Respir Crit Care Med. 2002;165(4):495-500.
  • Jadin SA, Wu GS, Zhang Z, et al. Growth and pulmonary outcomes during the first 2 y of life of breastfed and formula-fed infants diagnosed with cystic fibrosis through the Wisconsin Routine Newborn Screening Program. Am J Clin Nutr. 2011;93(5):1038-1047.
  • Jeppesen PB, Hoy CE, Mortensen PB. Essential fatty acid deficiency in patients receiving home parenteral nutrition. Am J Clin Nutr. 1998;68(1):126-133.
  • Kayani K, Mohammed R, Mohiaddin H. Cystic Fibrosis-related diabetes. Front Endocrinol. 2018;9(101555782):20.
  • Keller BM, Aebischer CC, Kraemer R, Schoni MH. Growth in prepubertal children with cystic fibrosis, homozygous for the Delta F508 mutation. J Cystic Fibros. 2003;2(2):76-83.
  • Keogh RH, Szczesniak R, Taylor-Robinson D, Bilton D. Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data. J Cyst Fibros. 2018;17(2):218-227.
  • King S, Wilson J, Kotsimbos T, Bailey M, Nyulasi I. Body composition assessment in adults with cystic fibrosis: comparison of dual-energy X-ray absorptiometry with skinfolds and bioelectrical impedance analysis. Nutrition. 2005;21(11):1087-1094.
  • King SJ, Nyulasi IB, Strauss BJG, Kotsimbos T, Bailey M, Wilson JW. Fat-free mass depletion in cystic fibrosis: associated with lung disease severity but poorly detected by body mass index. Nutrition. 2010;26(7):753-759.
  • Konstan MW, Morgan WJ, Butler SM, et al. Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis. J Pediatrics. 2007;151(2):134-139.e131.
  • Konstan MW, Plant BJ, Elborn JS, et al. Efficacy response in CF patients treated with ivacaftor: post-hoc analysis. Pediatric Pulmonol. 2015;50(5):447-455.
  • Kopin L, Lowenstein C. Dyslipidemia. Ann Intern Med. 2017;167(11):Itc81-itc96.
  • Lai H, Chin L, Schoff S, Zhang Z, Greer FR. Impact of breastfeeding on the nutritional status of infants with cystic fibrosis (CF) in the first year of life-results of the FIRST STUDY. FASEB J. 2016;30(1_supplement):672.674-672.674.
  • Lai HJ, Shoff SM, Farrell PM, et al. 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.
  • Ledger SJ, Owen E, Prasad SA, Goldman A, Willams J, Aurora P. A pilot outreach physiotherapy and dietetic quality improvement initiative reduces IV antibiotic requirements in children with moderate-severe cystic fibrosis. J Cystic Fibros. 2013;12(6):766-772.
  • Linus Pauling Institute MIC. Fiber. Oregon State University. https://lpi.oregonstate.edu/mic/other-nutrients/fiber. Published 2019. Accessed Septemeber 11, 2019.
  • Machogu E, Cao Y, Miller T, et al. Comparison of WHO and CDC growth charts in predicting pulmonary outcomes in cystic fibrosis. J Pediatric Gastroentero lNutri. 2015;60(3):378-383.
  • Maqbool A, Schall JI, Gallagher PR, Zemel BS, Strandvik B, Stallings VA. Relation between dietary fat intake type and serum fatty acid status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2012;55(5):605-611.
  • 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.
  • McColley SA, Schechter MS, Morgan WJ, Pasta DJ, Craib ML, Konstan MW. Risk factors for mortality before age 18 years in cystic fibrosis. Pediatr Pulmonol. 2017;52(7):909-915.
  • McIntosh ID. Health Human Resources Guidelines: Minimum Staffing Standards and Role Descriptions for Canadian Cystic Fibrosis Healthcare Teams. Can Respir J. 2016;2016:6369704.
  • McPhail GL, Acton JD, Fenchel MC, Amin RS, Seid M. Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa use. J Pediatrics. 2008;153(6):752-757.
  • Moen IE, Nilsson K, Andersson A, et al. Dietary intake and nutritional status in a Scandinavian adult cystic fibrosis-population compared with recommendations. Food Nutr Res. 2011;55(101488795).
  • Moran A, Pillay K, Becker D, Granados A, Hameed S, Acerini CL. ISPAD Clinical Practice Consensus Guidelines 2018: Management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes. 2018;19 Suppl 27:64-74.
  • Moss RB, Flume PA, Elborn JS, et al. Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial. Lancet Respir Med. 2015;3(7):524-533.
  • Moudiou T, Galli-Tsinopoulou A, Nousia-Arvanitakis S. Effect of exocrine pancreatic function on resting energy expenditure in cystic fibrosis. Acta Paediatr. 2007;96(10):1521-1525.
  • Moudiou T, Galli-Tsinopoulou A, Vamvakoudis E, Nousia-Arvanitakis S. Resting energy expenditure in cystic fibrosis as an indicator of disease severity. J Cystic Fibros. 2007;6(2):131-136.
  • Moukarzel S, Dyer RA, Innis SM. Complex relation between diet and phospholipid fatty acids in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2017;64(4):598-604.
  • Munck A, Boulkedid R, Weiss L, et al. Nutritional status the first two years of life in cystic fibrosis diagnosed by newborn screening. J Pediatr Gastroenterol Nutr. 2018.
  • National Center for Chronic Disease Prevention and Health Promotion  DfHDaSP. Getting Your Cholesterol Checked. U.S. Department of Health and Human Services. https://www.cdc.gov/cholesterol/cholesterol_screening.htm. Published 2019. Accessed September 2019.
  • Oates GR, Schechter MS. Socioeconomic status and health outcomes: cystic fibrosis as a model. Expert Rev Respir Med. 2016;10(9):967-977.
  • Ollero M, Astarita G, Guerrera IC, et al. Plasma lipidomics reveals potential prognostic signatures within a cohort of cystic fibrosis patients. J Lipid Res. 2011;52(5):1011-1022.
  • Olveira G, Dorado A, Olveira C, et al. Serum phospholipid fatty acid profile and dietary intake in an adult Mediterranean population with cystic fibrosis. BrJ Nutr. 2006;96(2):343-349.
  • Olveira G, Olveira C, Gaspar I, et al. Fat-free mass depletion and inflammation in patients with bronchiectasis. J Acad Nutr Diet. 2012;112(12):1999-2006.
  • Palmeira P, Carneiro-Sampaio M. Immunology of breast milk. Rev Assoc Med Bras (1992). 2016;62(6):584-593.
  • Panagopoulou P, Fotoulaki M, Nikolaou A, Nousia-Arvanitakis S,  A. Prevalence of malnutrition and obesity among cystic fibrosis patients. Pediatr Int. 2014;56(1):89-94.
  • Parker EM, O'Sullivan BP, Shea JC, Regan MM, Freedman SD. Survey of breast-feeding practices and outcomes in the cystic fibrosis population. Pediatric pulmonology. 2004;37(4):362-367.
  • Pediatrics AAo. 2016 Recommendations for Preventive Pediatric Health Care. Pediatrics. 2016;137.
  • Pedreira CC, Robert RGD, Dalton V, et al. Association of body composition and lung function in children with cystic fibrosis. Pediatric Pulmonol. 2005;39(3):276-280.
  • Peretti N, Marcil V, Drouin E, Levy E. Mechanisms of lipid malabsorption in cystic fibrosis: the impact of essential fatty acids deficiency. Nutrit Metab (Lond). 2005;2(1):11.
  • Poore S, Berry B, Eidson D, McKie KT, Harris RA. Evidence of vascular endothelial dysfunction in young patients with cystic fibrosis. Chest. 2013;143(4):939-945.
  • Powers SW, Stark LJ, Chamberlin LA, et al. Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial. JAMA Pediatrics. 2015;169(5):e150636.
  • Proesmans M, De Boeck K. Evaluation of dietary fiber intake in Belgian children with cystic fibrosis: is there a link with gastrointestinal complaints? J Pediatr Gastroenterol Nutr. 2002;35(5):610-614.
  • Puiman PJ, Francis P, Buntain H, Wainwright C, Masters B, Davies PSW. Total body water in children with cystic fibrosis using bioelectrical impedance. J Cystic Fibros. 2004;3(4):243-247.
  • Ramirez I, Filbrun A, Hasan A, Kidwell KM, Nasr SZ. Improving nutritional status in a pediatric cystic fibrosis center. Pediatr Pulmonol. 2015;50(6):544-551.
  • Ramos KJ, Quon BS, Heltshe SL, et al. Heterogeneity in survival in adult patients with cystic fibrosis sith FEV1 < 30% of predicted in the United States. Chest. 2017;151(6):1320-1328.
  • Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. New Engl J Med. 2011;365(18):1663-1672.
  • 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.
  • Rees K, Takeda A, Martin N, et al. Mediterranean-style diet for the primary and secondary prevention of cardiovascular disease. Cochrane Database Syst Rev. 2019;3:Cd009825.
  • Rhodes B, Nash EF, Tullis E, et al. Prevalence of dyslipidemia in adults with cystic fibrosis. J Cystic Fibros. 2010;9(1):24-28.
  • Rodriguez-Monforte M, Sanchez E, Barrio F, Costa B, Flores-Mateo G. Metabolic syndrome and dietary patterns: a systematic review and meta-analysis of observational studies. Eur J Nutr. 2017;56(3):925-947.
  • Roesch EA, Nichols DP, Chmiel JF. Inflammation in cystic fibrosis: An update. Pediatr Pulmonol. 2018;53(S3):S30-s50.
  • Rosato V, Temple NJ, La Vecchia C, Castellan G, Tavani A, Guercio V. Mediterranean diet and cardiovascular disease: a systematic review and meta-analysis of observational studies. Eur J Nutr. 2019;58(1):173-191.
  • Rowe SM, McColley SA, Rietschel E, et al. Lumacaftor/Ivacaftor Treatment of patients with cystic fibrosis heterozygous for F508del-CFTR. An Am Thorac Soc. 2017;14(2):213-219.
  • Sagel SD, Khan U, Jain R, et al. Effects of an Antioxidant-enriched multivitamin in cystic fibrosis: randomized, controlled, multicenter trial. Am J Respir Crit Care Med. 2018(9421642).
  • Sagel SD, Sontag MK, Anthony MM, Emmett P, Papas KA. Effect of an antioxidant-rich multivitamin supplement in cystic fibrosis. J Cystic Fibros. 2011;10(1):31-36.
  • Sanders DB, Emerson J, Ren CL, et al. Early childhood risk factors for decreased FEV1 at age six to seven years in young children with cystic fibrosis. An Am Thorac Soc. 2015;12(8):1170-1176.
  • 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 Pediatrics. 2015;167(5):1081-1088.e1081.
  • Sanders DB, Li Z, Laxova A, et al. Risk factors for the progression of cystic fibrosis lung disease throughout childhood. An Am Thorac Soc. 2014;11(1):63-72.
  • Sanders DB, Zhang Z, Farrell PM, Lai HJ, CF Neonatal screening group W. Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis. J Cystic Fibros. 2018(101128966).
  • Savant AP, Britton LJ, Petren K, McColley SA, Gutierrez HH. Sustained improvement in nutritional outcomes at two paediatric cystic fibrosis centres after quality improvement collaboratives. BMJ Quality & Safety. 2014;23:81-89.
  • Saxby N. PC, Kench A., King S., Crowder T., van der Haak N. and the Australian and New Zealand Cystic Fibrosis Nutrition Guideline Authorship Group. Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand. In. Sydney: Thoracic Society of Australia and New Zealand; 2017.
  • Schwarzenberg SJ, Hempstead SE, McDonald CM, et al. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. J Cystic Fibros. 2016;15(6):724-735.
  • Shakkottai A, O'Brien LM, Nasr SZ, Chervin RD. Sleep disturbances and their impact in pediatric cystic fibrosis. Sleep Med Rev. 2018;42:100-110.
  • Shamseer L, Adams D, Brown N, Johnson JA, Vohra S. Antioxidant micronutrients for lung disease in cystic fibrosis. Cochrane Database Syst Rev. 2010(12):CD007020.
  • Sheikh S, Zemel BS, Stallings VA, Rubenstein RC, Kelly A. Body composition and pulmonary function in cystic fibrosis. Front Pediatr. 2014;2(101615492):33.
  • Shepherd JA, Ng BK, Sommer MJ, Heymsfield SB. Body composition by DXA. Bone. 2017;104:101-105.
  • Skolnik K, Levy RD, Wilcox PG, Quon BS. Coronary artery disease in cystic fibrosis: An emerging concern? J Cyst Fibros. 2016;15(6):e70-e71.
  • Smyth W, Abernethy G, Jessup M, Douglas T, Shields L. Family-centred care in cystic fibrosis: a pilot study in North Queensland, Australia. Nurs Open. 2017;4(3):168-173.
  • Soltani S, Jayedi A, Shab-Bidar S, Becerra-Tomas N, Salas-Salvado J. Adherence to the Mediterranean diet in relation to all-cause mortality: a systematic review and dose-response meta-analysis of prospective cohort studies. Adv Nutr. 2019.
  • Stallings VA, Sainath N, Oberle M, Bertolaso C, Schall JI. Energy balance and mechanisms of weight gain with Ivacaftor treatment of cystic fibrosis gating mutations. J Pediatr. 2018;201:229-237.e224.
  • Stallings VA, Stark LJ, Robinson KA, et al. 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.
  • Stalvey MS, Pace J, Nikniar M, et al. Growth in prepubertal children with cystic fibrosis treated sith Ivacaftor. Pediatrics. 2017;139(2):44-44.
  • 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. Pediatric Pulmonol. 2011;46(1):31-35.
  • Stark LJ, Quittner AL, Powers SW, et al. Randomized clinical trial of behavioral intervention and nutrition education to improve caloric intake and weight in children with cystic fibrosis. Arch Pediatr Adolesc Med. 2009;163(10):915-921.
  • Stephenson AL, Mannik LA, Walsh S, et al. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am J Clin Nutr. 2013;97(4):872-877.
  • Stephenson AL, Tom M, Berthiaume Y, et al. A contemporary survival analysis of individuals with cystic fibrosis: a cohort study. Eur Respir J 2015;45(3):670-679.
  • Sutherland R, Katz T, Liu V, et al. Dietary intake of energy-dense, nutrient-poor and nutrient-dense food sources in children with cystic fibrosis. J Cyst Fibros. 2018;17(6):804-810.
  • Swan WI, Vivanti A, Hakel-Smith NA, et al. Nutrition Care Process and Model Update: Toward Realizing People-Centered Care and Outcomes Management. J Acad Nutr Diet. 2017;117(12):2003-2014.
  • Tangpricha V, Kelly A, Stephenson A, et al. 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.
  • Taylor-Cousar JL, Munck A, McKone EF, et al. Tezacaftor-Ivacaftor in patients with cystic fibrosis homozygous for Phe508del. New Engl J Med. 2017;377(21):2013-2023.
  • Trabulsi J, Ittenbach RF, Schall JI, et al. Evaluation of formulas for calculating total energy requirements of preadolescent children with cystic fibrosis. Amer J Clini Nutr. 2007;85(1):144-151.
  • 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 (Edinburgh, Scotland). 2016;35(3):557-577.
  • United States Department of Agriculture. 2015 – 2020 Dietary Guidelines for Americans.2015.
  • Usatin D, Yen EH, McDonald C, Asfour F, Pohl J, Robson J. Differences between WHO and CDC early growth measurements in the assessment of cystic fibrosis clinical outcomes. J Cystic Fibros. 2017;16(4):503-509.
  • van der Doef HPJ, Kokke FTM, Beek FJA, Woestenenk JW, Froeling SP, Houwen RHJ. Constipation in pediatric cystic fibrosis patients: an underestimated medical condition. J Cystic Fibros. 2010;9(1):59-63.
  • van Gool K, Norman R, Delatycki MB, Hall J, Massie J. Understanding the costs of care for cystic fibrosis: an analysis by age and health state. Value Health. 2013;16(2):345-355.
  • Vieni G, Faraci S, Collura M, et al. Stunting is an independent predictor of mortality in patients with cystic fibrosis. Clin Nutr. 2013;32(3):382-385.
  • Wainwright CE, Elborn JS, Ramsey BW, et al. Lumacaftor-Ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. New Engl J Med. 2015;373(3):220-231.
  • Wells GD, Heale L, Schneiderman JE, et al. Assessment of body composition in pediatric patients with cystic fibrosis. Pediatric Pulmonol. 2008;43(10):1025-1032.
  • Welsh L, Robertson CF, Ranganathan SC. Increased rate of lung function decline in Australian adolescents with cystic fibrosis. Pediatric Pulmonol. 2014;49(9):873-877.
  • White H, Wolfe SP, Foy J, Morton A, Conway SP, Brownlee KB. Nutritional intake and status in children with cystic fibrosis: does age matter? J Pediatr Gastroenterol Nutr. 2007;44(1):116-123.
  • Widmer RJ, Flammer AJ, Lerman LO, Lerman A. The Mediterranean diet, its components, and cardiovascular disease. Am J Med. 2015;128(3):229-238.
  • Williams JE, Wells JC, Benden C, et al. Body composition assessed by the 4-component model and association with lung function in 6-12-y-old children with cystic fibrosis. Am J Clin Nutr. 2010;92(6):1332-1343.
  • Woestenenk JW, Schulkes DA, Schipper HS, van der Ent CK, Houwen RHJ. Dietary intake and lipid profile in children and adolescents with cystic fibrosis. J Cystic Fibros. 2017;16(3):410-417.
  • Woestenenk JW, Stellato RK, Terheggen-Lagro SW, van der Ent CK, Houwen RHJ. The relationship between body growth and pulmonary function in children with cystic fibrosis. Acta Paediatrica (Oslo, Norway : 1992). 2014;103(2):162-167.
  • World Health Organization. Breastfeeding. World Health Organization. https://www.who.int/topics/breastfeeding/en/. Published 2019. Accessed September 11, 2019.
  • World Health Organization. Obesity and overweight. World Health Organization. https://www.who.int/news-room/fact-sheets/detail/obesity-and-overweight. Published 2018. Accessed September 11, 2019.
  • Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004; 125(1 Suppl):1S-39S.
  • 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 Pediatrics. 2013;162(3):530-535.e531.
  • Zhang Z, Shoff SM, Lai HJ. Comparing the use of Centers for Disease Control and Prevention and World Health Organization growth charts in children with cystic fibrosis through 2 years of age. J Pediatrics. 2015;167(5):1089-1095.
  • Ziai S, Coriati A, St-Pierre D, et al. Glucose fluctuations are not modulated by the proportion of calories from macronutrients or spontaneous total energy expenditure in adults with cystic fibrosis. CJ Diabetes. 2016;40(5):389-392.

 

Proceed to Recommendations and Supporting Information  Return to CF Home Page