Kistik Fibroziste Mikrobesin Ögelerinin Önemi

Yıl 2021, Cilt: 19 Sayı: 1, 127 - 134, 09.04.2021

Sevinç Eşer Durmaz Nurcan Yabancı

Öz

Kistik fibrozis, kistik fibrozis transmembran kondüktans regülatör (KFTR) geninde meydana gelen mutasyonların neden olduğu, solunum sistemi başta olmak
üzere epitel dokuya sahip hücrelerin tümünü etkileyen klinik olarak ilerleyici akciğer hastalığı ile karakterize otozomal ressesif geçişli kalıtsal bir hastalıktır.
Bireylerin yaklaşık olarak %90’ında pankreatik yetersizlik ve bununla birlikte malabsorbsiyon durumu görülmektedir. Hastalığın patogenezinde yer alan kronik
enfeksiyon ve artmış enerji gereksinimi, malabsorbsiyonlar ile birlikte yetersiz beslenme için risk oluşturmaktadır. Malabsorbsiyonlar nedeniyle mikrobesin
ögelerinin emiliminde azalma, gereksinimdeki artış, yetersiz alım ve bu durumun sonucu olarak hastalığın kötü prognozu, yaşam kalitesinin ve süresinin
azalmasına neden olmaktadır. Kistik fibroziste beslenme durumu değişikliklerinin belirlenmesi, buna yönelik erken ve uygun beslenme müdahalelerinin yapılması
gerekmektedir. Kistik fibrozisli bireylerde yaşam boyunca büyüme ve beslenme durumunun takip edilmesi önerilir. Pankreatik enzim replasman tedavisi (PERT),
hidrasyon ve elektrolit dengesi ile birlikte yaşamın erken dönemlerinde diyetisyen kontrolünde optimal beslenme durumu sağlanmalıdır. Kistik fibroziste optimal
beslenme, doğru pişirme yöntemleri ile birlikte diyet ile mikrobesin ögeleri alımında etkili olmasına rağmen, bu hastalarda mikrobesin ögeleri yetersizliği
önlenemeyebilmektedir. Bu durumda optimal beslenme ile birlikte rehberlerin önerileri göz önünde bulundurularak, bireylerin gereksinimine ve biyokimyasal
değerlerine uygun vitamin-mineral desteğine gereksinim duyulmaktadır.

Anahtar Kelimeler

mikrobesin ögeleri,, malabsorbsiyon,, Kistik fibrozis,

The Importance of Micronutrients in Cystic Fibrosis

Öz

Cystic fibrosis is an inherited disease with an autosomal recessive transition,
characterized by a clinically progressive lung disease, which is caused by mutations
occurring in the transmembrane conductance regulator (KFTR) gene, affecting all
cells with epithelial tissue, especially the respiratory system. Approximately 90%
of individuals experience pancreatic insufficiency and malabsorption. Chronic
infection and increased energy requirement in the pathogenesis of the disease,
together with malabsorption, pose a risk for malnutrition. Decreased absorption
of micronutrients due to malabsorptions, increase in the requirement, inadequate
intake, and as a result, poor prognosis of the disease leads to decreased quality
of life and duration. Nutritional status changes should be determined in cystic
fibrosis and early and appropriate nutritional interventions should be made. It
is recommended to follow the growth and nutritional status throughout life in
cystic fibrosis. Optimal nutritional status should be provided in the early stages
of life, under the control of a dietitian, with pancreatic enzyme replacement
therapy (PERT), hydration and electrolyte balance. Although optimal nutrition in
cystic fibrosis is effective in diet and micronutrient intake with proper cooking
methods, deficiency of micronutrients in these patients can not be prevented. In
this case, taking into account the recommendations of the guidelines along with
optimal nutrition, vitamin-mineral supplements in accordance with the needs and
biochemical values of individuals are needed.

Kaynakça

• 1. Rogers CL. Nutritional Management of the Adult with Cystic Fibrosis. Nutrition Issues in Gastroenterology. 2013;113:10-24.
• 2. Türk Toraks Derneği. Kistik Fibrozis. 2017. Erişim adresi: https://www.toraks.org.tr/halk/News.aspx?detail=4356.
• 3. El-Koofy N, El-Mahdy M, Fathy M, El Falaki M, El Dine Hamed DH. Nutritional rehabilitation for children with cystic fibrosis: Single center study. Clinical nutrition ESPEN. 2020;35:201-6.
• 4. Solomon M, Bozic M, Mascarenhas MR. Nutritional Issues in Cystic Fibrosis. Clinics in chest medicine. 2016;37:97-107.
• 5. Schonenberger KA, Reber E, Bally L, Geiser T, Lin D, Stanga Z. Nutritional assessment in adults with cystic fibrosis. Nutrition.2019;68:510-8.
• 6. Tham A, Katz TE, Sutherland RE, Garg M, Liu V, Tong CW, et al. Micronutrient intake in children with cystic fibrosis in Sydney, Australia. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2020;19(1):146-52.
• 7. Sutherland R, Katz T, Liu V, Quintano J, Brunner R, Tong CW, et al. Dietary intake of energy-dense, nutrient-poor and nutrientdense food sources in children with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2018;17(6):804-10.
• 8. Collins S. Nutritional management of cystic fibrosis - an update for the 21st century. Paediatric respiratory reviews. 2018;26:4-6.
• 9. Karhan AN, Emiralioğlu N, Tuğcu GD, Yalçın E, Ersöz D, Özçelik U ve ark. Kistik Fibrozis’li Çocuk ve Erişkin Hastalarda, Yağda Eriyen Vitamin Eksiklikleri ile alnutrisyon Sıklığının Değerlendirilmesi ve Pulmoner Fonksiyonlarla İlişkisinin Araştırılması. Türkiye Çocuk Hastalıkları Dergisi. 2019;13(6): 463-7
• 10. Irish Nutrition & Dietetic Institute (INDI). Vitamins and Minerals in Cystic Fibrosis 2016. Erişim Adresi: https://www.indi.ie/ images/Vitamins_and _Minerals _CF_ INDI_ 2016.pdf.
• 11. Greer RM, Buntain HM, Lewindon PJ, Wainwright CE, Potter JM, Wong JC, et al. Vitamin A levels in patients with CF are influenced by the inflammatory response. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2004;3(3):143-9.
• 12. Zhang Z, Chin LH, Lai HJ. Nutrition and Cystic Fibrosis. 2017:911-34.
• 13. Singh VK, Watson RR. Fat-Soluble Vitamin Deficiency in Cystic Fibrosis. 2015:109-15.
• 14. Reifen R. Vitamin A as an anti-inflammatory agent. Proc Nutr Soc. 2002;61(3):397-400.
• 15. Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. Journal of pediatric gastroenterology and nutrition. 2002;35(3):246-59.
• 16. Graham-Maar RC, Schall JI, Stettler N, Zemel BS, Stallings VA. Elevated vitamin A intake and serum retinol in preadolescent children with cystic fibrosis. Am J Clin Nutr. 2006;84(1):174-82.
• 17. Woestenenk JW, van der Ent CK, Houwen RH. Pancreatic Enzyme Replacement Therapy and Coefficient of Fat Absorption in Children and Adolescents With Cystic Fibrosis. Journal of pediatric gastroenterology and nutrition. 2015;61(3):355-60.
• 18. Patchell C. Cystic Fibrosis. In: Shaw V, editor. Clinical Paediatric Dietetics. Fourth Edition ed: Wiley Blackwell; 2019. p. 222-41.
• 19. Hall WB, Sparks AA, Aris RM. Vitamin D deficiency in cystic fibrosis. International Journal of Endocrinology. 2009; 1-9.
• 20. Le TN. Updates in vitamin D therapy in cystic fibrosis. Current opinion in endocrinology, diabetes, and obesity. 2018;25(6):361-5.
• 21. Herscovitch K, Dauletbaev N, Lands LC. Vitamin D as an antimicrobial and anti-inflammatory therapy for Cystic Fibrosis. Paediatric respiratory reviews. 2014;15(2):154-62.
• 22. Regalado Lam Chew Tun R, Porhownik N, Taback S, Oleschuk C. Effect of high dose vitamin D3 therapy on serum vitamin D3 levels in vitamin D insufficient adults with cystic fibrosis. Clinical nutrition ESPEN. 2018;23:84-8.
• 23. Bertolaso C, Groleau V, Schall JI, Maqbool A, Mascarenhas M, Latham NE, et al. Fat-soluble vitamins in cystic fibrosis and pancreatic insufficiency: efficacy of a nutrition intervention. Journal of pediatric gastroenterology and nutrition.2014;58(4):443-8.
• 24. Akbıyık H, Gökmen Özel H, Emiralioğlu N, Köksal G, Yalçın E, Ersöz D, Özçelik H, Kiper N. Kistik Fibrozisli Hastalarda Malnütrisyon ve Beslenme Durumunun Saptanması. Beslenme ve Diyet Dergisi 2015;43:43-50.
• 25. Okebukola PO, Kansra S, Barrett J. Vitamin E supplementation in people with cystic fibrosis. The Cochrane database of systematic reviews. 2020;9:1-3.
• 26. Maqbool A, Stallings VA. Update on fat-soluble vitamins in cystic fibrosis. Curr Opin Pulm Med. 2008;14(6):574-81.
• 27. Jagannath VA, Thaker V, Chang AB, Price AI. Vitamin K supplementation for cystic fibrosis (Review). Cochrane Database of Systematic Reviews. 2020;(6): 1-3.
• 28. Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clinical nutrition. 2016;35(3):557-77.
• 29. Sagel SD, Khan U, Jain R, Graff G, Daines CL, Dunitz JM, et al. Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial. American journal of respiratory and critical care medicine. 2018;198(5):639-47.