Current dietary recommendations for patients with cystic fibrosis

Katarzyna Stefańska, Monika Jakimiec-Komisarczyk, Paulina Terlecka, Marcelina Makuch, Joanna Jakimiec, Magdalena Igras-Kołdyj, Michał Szczyrek, Robert Kieszko, Agnieszka Zwolak, Janusz Milanowski, Robert Jan Łuczyk

Abstract


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.

Keywords


cystic fibrosis; nutritional treatment; supplementation

Full Text:

PDF

References


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.




DOI: http://dx.doi.org/10.5281/zenodo.2635585

Refbacks

  • There are currently no refbacks.




Copyright (c) 2019 © The Author(s)

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Journal of Education, Health and Sport formerly Journal of Health Sciences

Declaration on the original version.

Editors indicates that the main version of the magazine is to issue a "electronic".

The journal has had 5 points in Ministry of Science and Higher Education parametric evaluation. § 8. 2) and § 12. 1. 2) 22.02.2019.

1223 Journal of Education, Health and Sport eISSN 2391-8306 7

ISSN 2391-8306 formerly ISSN: 1429-9623 / 2300-665X

Archives 2011 - 2014

PBN 2011 - 2014

https://pbn.nauka.gov.pl/sedno-webapp/search?search&searchCategory=WORK&filter.inJournal=36616

POL-index 2011 - 2014

https://pbn.nauka.gov.pl/polindex-webapp/browse/journal/journal-32a319c5-a850-4f44-ba70-1418a6087655

BASE 2011 - 2014

https://www.base-search.net/Search/Results?lookfor=Journal+of+Health+Sciences+%28Radom%29&type=all&oaboost=1&ling=1&name=&thes=&refid=dcrespl&newsearch=1

http://elibrary.ru/contents.asp?titleid=37467

http://journal.rsw.edu.pl/index.php/JHS/issue/archive

Indexed in Bases, Bazy indeksacyjne: ERIH Plus, Worldcat, PBN/POL-Index, ICI Journals Master List, Directory of Open Access Journals (DOAJ), ZBD, Ulrich's periodicals, Google Scholar, Polska Bibliografia Lekarska

US NLM = 101679844

101679844 - NLM Catalog Result - NCBI

https://www.ncbi.nlm.nih.gov/nlmcatalog/101679844

Find a library that holds this journal: http://worldcat.org/issn/23918306

Journal Language(s): English 

PBN Poland

https://pbn.nauka.gov.pl/sedno-webapp/journals/49068

POL-index

https://pbn.nauka.gov.pl/polindex-webapp/browse/journal/journal-c39c8169-88d2-45db-9e7f-d948ce9981c4

BASE

https://www.base-search.net/Search/Results?join=AND&bool0[]=AND&lookfor0[]=2391-8306&type0[]=all&page=11&l=pl&oaboost=1&refid=dcpagepl

Redaction, Publisher and Editorial Office

Instytut Kultury Fizycznej Uniwersytet Kazimierza Wielkiego w Bydgoszczy, Institute of Physical Education Kazimierz Wielki University in Bydgoszcz, Poland 85-091 Bydgoszcz ul. Sportowa 2  www.ukw.edu.pl Copyright by Instytut Kultury Fizycznej UKW w Bydgoszczy http://ojs.ukw.edu.pl/index.php/johs  Open Access ISSN 2391-8306 formerly ISSN: 1429-9623 / 2300-665X

The journal has been approved for inclusion in ERIH PLUS.

The ERIH PLUS listing of the journal is available at https://dbh.nsd.uib.no/publiseringskanaler/erihplus/periodical/info?id=485984

SIC Science citation index (calculated on the basis of TCI and Page Rank) 0

Russian Impact factor 0.16

Indexed in Index Copernicus Journals Master List.

http://journals.indexcopernicus.com/Journal+of+Education+Health+and+Sport,p24782242,3.html

ICV 2018 = 95.95 ICV 2017 = 91.30 ICV 2016 = 84.69 ICV 2015 = 93.34 ICV 2014 = 89.51 Standardized Value: 8.27 ICV 2013: 7.32 ICV 2012: 6.41 ICV 20115.48

The InfoBase Index IBI Factor for the year 2015 is 3.56 in InfoBase Index.com.

Website: www.infobaseindex.com

Universal Impact Factor 1.78 for year 2012. (http://www.uifactor.org/AppliedJournals.aspx)

Indexed in Polish Scholarly Bibliography (PBN) (PBN Polska Bibliografia Naukowa) (https://pbn.nauka.gov.pl/journals/36616)

is a portal of the Polish Ministry of Science and Higher Education, collecting information on publications of Polish scientists and on Polish and foreign scholarly journals. Polish Scholarly Bibliograhpy is a part of POL-on - System of Information on Higher Education. It is operated by the Interdisciplinary Centre for Mathematical and Computational Modelling, University of Warsaw.

Indexed in Russian Sciences Index Российский индекс научного цитирования (РИНЦ) http://elibrary.ru/contents.asp?titleid=37467

Indexed in Arianta Polish scientific and professional electronic journals Aneta Drabek i Arkadiusz Pulikowski

(http://www1.bg.us.edu.pl/bazy/czasopisma/czasop_full.asp?id=3595)