Undernutrition and Clinical Deterioration in Children with Cystic Fibrosis: Findings from a Prospective Study

Cystic Fibrosis is a genetic disease that causes dysfunctions in the transmembrane chloride conductance regulatory protein in epithelial cells. The main manifestations are persistent pulmonary symptoms and low weight gain. Therefore, nutrition plays an essential role in the survival and quality of life of these patients. This study aimed to evaluate the influence of nutritional status on the clinical condition and life expectancy in Cystic Fibrosis pediatric patients. Prospective cohort study evaluating sociodemographic, birth, clinical, anthropometric data, food intake, Shwachman-Kulczycki score and lung function. A total of 102 patients were evaluated in the first stage and 88 patients at the end of the study. Relative risk (RR) and confidence interval (95% CI) were calculated, considering p