Cystic fibrosis (CF) is classified as metabolic and multisystem disease with autosomal recessive inheritance caused by mutations in the gene located on chromosome 7 encoding cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR is a transmembrane chloride channel of epithelial cells and affects the activity of the mucous membrane of the sweat glands, airway epithelium, pancreatic ducts, vas deferens, bile ducts and intestines. In CF, increased concentration of chlorides in the sweat, pancreatic insufficiency and impaired absorption are observed as well as changes in the respiratory system related to, among others, impaired airway patency, weakening of the mucociliary clearance mechanism and the development of bacterial infections. CF is a chronic condition requiring comprehensive therapy. Nutritional treatment is an essential element of CF therapy. Malnutrition is a common complication in patient with CF and eating disorders. The majority of patients with CF have higher energy, protein and fat needs. In addition, supplementation with enzyme preparations, vitamins, sodium chloride, as well as the use of high-energy nutrients is recommended. The aim of the study was to evaluate current nutritional recommendations of patients with CF.

cystic fibrosis; nutritional treatment; supplementation

Cutting GR. Cystic fibrosis genetics: from molecular understanding to clinical application. Nat Rev Genet. 2015; 16(1): 45–56.

Kelly J. Environmental scan of cystic fibrosis research worldwide. J Cyst Fibros. 2017; 16(3):367-370.

ECFSPR Annual Report 2016, Orenti A, Zolin A, Naehrlich L, van Rens J et al, 2018.

Harris A. Cystic fibrosis gene. Br Med Bull. 1992; 48(4): 738-53.

Montgomery GS, Howenstine M. Cystic Fibrosis. Pediatrics in Review. 2009; 30(8): 302-310.

Kowalska M, Mandecka A, Regulska-Ilow B. Zaburzenia stanu odżywienia w mukowiscydozie – zalecenia żywieniowe i suplementacja diety. [Disturbances in nutrition in cystic fibrosis - nutritional recommendations and supplementation]. Medycyna Ogólna i Nauki o Zdrowiu. 2017; 23(2): 115–121 (in Polish).

King SJ, Nyulasi IB, Strauss BJ, 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(7–8): 753– 759.

Engelen M, Com G, Deutz NE. Protein is an important but undervalued macronutrient in the nutritional care of patients with Cystic Fibrosis. Curr Opin Clin Nutr Metab Care. 2014; 17(6): 515–520.

Hauschild D, Rosa AF, Ventura JC, Barbosa E, Moreira EAM, Neto NL, et al. Association of nutritional status with lung function and morbidity in children and adolescents with cystic fibrosis: a 36-month cohort study. Rev Paul Pediatr. 2018;36(1):31-1138.

Barni GC, Forte GC, Forgiarini LF, Abrahao CLO, Dalcin PTR. Factors associated with malnutrition in adolescent and adult patients with cystic fibrosis. J Bras Pneumol. 2017; 43(5): 337-343.

Neri LCL, Bergamaschi DP, Silva Filho LVRFD: Evaluation of nutritional status in patients with cystic fibrosis according to age group. Rev Paul Pediatr. 2019; 37 (1): 58-64.

Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clinical Nutrition. 2019; 35: 557- 577.

Walkowiak J, Pogorzelski A, Sands D, Skorupa W, Milanowski A, Nowakowska A. Zasady rozpoznawania i leczenia mukowiscydozy. [Rules for the diagnosis and treatment of cystic fibrosis]. Zalecenia Polskiego Towarzystwa Mukowiscydozy 2009 Poznań – Warszawa – Rzeszów. Standardy medyczna/pediatria. 2009; 6: 352-378 (in Polish).

Birch L, Lithander FE, Hewer SL, Harriman K, Hamilton-Shield J, Perry R. Dietary interventions for managing glucose abnormalities in cystic fibrosis: a systematic review protocol. Systematic Reviews. 2018; 7: 98.

Otto-Buczkowska E, Marciniak-Brzezińska M. Cukrzyca u chorych z mukowiscydozą. Forum Medycyny Rodzinnej. 2016; 10(6): 295–298.

Sands D, Mielus M, Umławska W, Lipowicz A, Oralewska B, Walkowiak J. Dietary pattern and its relationship between bone mineral density in girls and boys with cystic fibrosis – preliminary report. Dev Period Med. 2015; 19(1): 105-113.

Wani WA, Nazir M, Bhat JI, Ahmad QI, Charoo BA, Ali SW. 2018. Vitamin D status correlates with the markers of cystic fibrosis-related pulmonary disease. Pediatrics and Neonatology 2018: 1-6. doi.org/10.1016/j.pedneo.2018.07.00

Kowalska M, Mandecka A, Regulska-Ilow B. Zaburzenia stanu odżywienia w mukowiscydozie – zalecenia żywieniowe i suplementacja diety [Disturbances in nutrition in cystic fibrosis - dietary recommendations and dietary supplementation]. Medycyna Ogólna i Nauki o Zdrowiu. 2017; 23 (2): 115–121 (in Polish).

Stephenson AL, Mannik LA, Walsh S, Brotherwood M, Robert R, Darling PB. 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.

Bhatt J. Treatment of pulmonary exacerbations in cystic fibrosis. Eur Respir Rev 2013; 22: 205–216.

Van Biervliet S, Declercq D, Somerset S. Clinical effects of probiotics in cystic fibrosis patients: A systematic review. Clin Nutr ESPEN. 2017; 18: 37- 43.