Akademik Veri Yönetim Sistemi
GUNCEL PEDIATRI, cilt.19, ss.127-134, 2021 (ESCI)
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.