Modern view on the nutrition of children with cystic fibrosis




cystic fibrosis, children, malnutrition, medical nutrition therapy


Cystic fibrosis (CF) is an autosomal recessive, life-shortening disease in which generalized exocrine gland involvement occurs with predominantly pancreatic insufficiency, airway obstruction, and progressive lung involvement. Due to pancreatic insufficiency, malabsorption and malnutrition are observed in patients. The close attributive association of CF with nutritional status and adverse clinical outcomes determines a thorough assessment of the nutritional status, individual management and monitoring of all children with CF. Attainment of optimal physical development and adequate nutrition in patients with CF is currently possible through early diagnosis and aggressive nutritional medical therapy. Medical dietary interventions are aimed at ensuring normal growth and preventing of malnutrition, thus will upgrade the prognosis and quality of life of pediatric patients with CF.

The reasons for the development of malnutrition in CF, specific screening tools for monitoring the growth of ill children, criteria for stratifying children into nutritional risk groups are reviewed in the lection. The procedure for calculating energy needs, taking into account activity, the severity of the disease and pancreatic insufficiency is presented. Attention is drawn to the ratio of macronutrients and the need to consume energy-dense and nutrient-rich foods.

The prescription of medical nutritional therapy with the inclusion of special foods for medical purposes (clinical nutrition) to meet increased energy requirements and avoid the consumption of energy-dense but nutrient-poor foods also is discussed. It is meaningful to involve into a multidisciplinary team of a psychologist who can on a regular basis correct eating behavior and promote compliance with nutritional recommendations in order to achieve optimal physical development of patients.

The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies.

No conflict of interests was declared by the authors.


Academy of Nutrition and Dietetics. (2020). Nutrition Care Manual. URL:

Academy of Nutrition and Dietetics. (2020). Pediatric Nutrition Care Manual. URL:

Ashkenazi M, Nathan N, Sarouk I, Aluma BEB, Dagan A, Bezalel Y et al. (2019). Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis Lung. 197: 371-376.; PMid:30887107

Baker R, Backer S, Bojczuk G. (2021). Cystic fibrosis: Nutritional issues In: UpToDate. URL:

Balfour-Lynn IM. (2020). Clinical Guidelines: Care of Children with Cystic Fibrosis. 8th edition. London: Royal Brompton Hospital: 311.

Bell SC, Mall MA, Gutierrez H, Macek M, Madge S, Davies JC et al. (2020). The future of cystic fibrosis care: A global perspective. Lancet Respir Med. 8 (1): 65-124.

Brownell JN, Bashaw H, Stallings VA. (2019). Growth and Nutrition in Cystic Fibrosis. Semin Respir Crit Care Med. 40 (6): 775-791.; PMid:31659726

Calvo-Lerma J, Hulst J, Boon M, Martins T, Ruperto M, Colombo C et al. (2019). The Relative Contribution of Food Groups to Macronutrient Intake in Children with Cystic Fibrosis: A European Multicenter Assessment. J Acad Nutr Diet. 119 (8): 1305-1319.; PMid:30862484

Castellani C, Duff AJA, Bell SC, Heijerman HGM, Munck A, Ratjen F et al. (2018). ECFS best practice guidelines: the 2018 revision. J Cyst Fibros. 17 (2): 153-178.; PMid:29506920

Culhane S, George C, Pearo B, Spoede E. (2013). Malnutrition in cystic fibrosis: A review. Nutr Clin Pract. 28 (6): 676-683.; PMid:24170579

Declercq D, Van Meerhaeghe S, Marchand S, Van Braeckel E, van Daele S, De Baets F et al. (2019). The nutritional status in CF: Being certain about the uncertainties, Clinical Nutrition ESPEN. 29: 15-21.; PMid:30661680

Devon Formulary and Referral. (2022). Formulary choice oral nutritional supplements. URL:

Kutney KA, Sandouk Z, Desimone M, Moheet A. (2021). Obesity in cystic fibrosis. J Clin Transl Endocrinol. 26: 100276.; PMid:34868883 PMCid:PMC8626670

Madde A, Okoniewski W, Sanders DB, Ren CL, Weiner DJ, Forno E. (2021). Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis. J Cyst Fibros. URL: S1569199321021731.; PMid:34972650

McDonald CM, Alvarez JA, Bailey J, Bowser EK, Farnham K, Mangus M et al. (2021). Academy of Nutrition and Dietetics: 2020 Cystic Fibrosis Evidence Analysis Center Evidence-Based Nutrition Practice Guideline. J Acad Nutr Diet. 121 (8): 1591-1636.; PMid:32565399

McDonald CM, Bowser EK, Farnham K, Alvarez JA, Padula L, Rozga M. (2021). Dietary Macronutrient Distribution and Nutrition Outcomes in Persons with Cystic Fibrosis: An Evidence Analysis Center Systematic Review. J Acad Nutr Diet. 121 (8): 1574-1590.; PMid:32532674

McDonald CM. (2009). Validation of a nutrition risk screening tool for children and adolescents with cystic fibrosis ages 2-20 years. J Pediatr Gastroenterol Nutr. 46 (4): 438-446.; PMid:18367958

Ministry of Healthcare of Ukraine. (2016). The unified clinical protocol of primary, secondary (specialized) and tertiary (highly specialized) medical care «Cystic fibrosis». Order No. 723, 15.07.2016. URL:

Poulimeneas D, Grammatikopoulou MG, Devetzi P, Petrocheilou A, Kaditis AG, Papamitsou T, et al. (2020). Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study. Nutrients. 12 (10): 3126.; PMid:33066268 PMCid:PMC7602117

Poulimeneas D, Grammatikopoulou MG, Petrocheilou A, Kaditis AG, Troupi E, Doudounakis SE et al. (2020). Comparison of International Growth Standards for Assessing Nutritional Status in Cystic Fibrosis: The GreeCF Study. J Pediatr Gastroenterol Nutr. 71 (1): 35-39.; PMid:32102088

Poulimeneas D, Grammatikopoulou MG, Petrocheilou A, Kaditis AG, Vassilakou T. (2020). Triage for Malnutrition Risk among Pediatric and Adolescent Outpatients with Cystic Fibrosis, Using a Disease-Specific Tool. Children (Basel, Switzerland). 7 (12): 269.; PMid:33291524 PMCid:PMC7761802

Poulimeneas D, Petrocheilou A, Grammatikopoulou MG, Kaditis AG, Loukou I, Doudounakis SE et al. (2017). High attainment of optimal nutritional and growth status observed among Greek pediatric cystic fibrosis patients: results from the GreeCF study. J Pediatr Endocrinol Metab. 26; 30 (11): 1169-1176.; PMid:29087958

RBHT. (2020). Clinical Guidelines: Care of Children with Cystic Fibrosis. Chapter 7 - Gastrointestinal & nutritional care. 8th editions. Balfour-Lynn I. Royal Brompton&Harefield NHS Foundation Trust: 331. URL:

Ritchie H, Nahikian-Nelms M, Roberts K, Gemma S, Shaikhkhalil A. (2021). The prevalence of aberrations in body composition in pediatric cystic fibrosis patients and relationships with pulmonary function, bone mineral density, and hospitalizations. J Cyst Fibros. 20 (5): 837-842.; PMid:33933345

Rozga M, Handu D. (2019). Nutrition Care for Patients with Cystic Fibrosis: An Evidence Analysis Center Scoping Review. J Acad Nutr Diet. 119 (1): 137-151.; PMid:29804871

Schofield, WN. (1985). Predicting basal metabolic rate, new standards and review of previous work. Hum Nutr Clin Nutr. 39 (1): 5-41.

Slae M, Wilschanski M. (2019). Prevention of malnutrition in cystic fibrosis. Curr Opin Pulm Med. 25 (6): 674-679.; PMid:31567328

Solomon M, Bozic M, Mascarenhas MR. (2016). Nutritional Issues in Cystic Fibrosis. Clin Chest Med. 37 (1): 97-107.; PMid:26857771

Souza Dos Santos Simon MI, Forte GC, da Silva Pereira J, da Fonseca Andrade Procianoy E, Drehmer M. (2016). Validation of a Nutrition Screening Tool for Pediatric Patients with Cystic Fibrosis. J Acad Nutr Diet. 116 (5): 813-818.; PMid:27126153

Sutherland R, Katz T, Liu V, Quintano J, Brunner R, Tong ChW et al. (2018). Dietary intake of energy-dense, nutrient-poor and nutrient-dense food sources in children with cystic fibrosis. J Cyst Fibros. 17 (6): 804-810.; PMid:29724576

Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R et al. (2016). ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 35 (3): 557-577.; PMid:27068495

Van der Haak N, King SJ, Crowder T, Kench A, Painter C, Saxby N. (2020). Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand Authorship Group and Interdisciplinary Steering Committee. Highlights from the nutrition guidelines for cystic fibrosis in Australia and New Zealand. J Cyst Fibros. 19 (1): 16-25.; PMid:31175004

Watson H, Stackhouse C. (2020). Omega-3 fatty acid supplementation for cystic fibrosis. Cochrane Database Syst Rev. 4 (4): CD002201.

Zhang Zh, Chin LH, Lai HCJ. (2017). Chapter 42 - Nutrition and Cystic Fibrosis, In: Nutrition in the Prevention and Treatment of Disease. 4th ed. Academic Press: 911-934.