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<0.05 as significant. Patients who were in the <50th percentile presented cystic fibrosis related symptoms earlier (0.96±3.45 months vs. 5.08±17.73 months), 53.2% had gastroesophageal reflux disease, 70% depletion of muscle tissue and lower weight (22.19±13.34 vs. 23.27±14.80) compared to those who had a good nutritional status. Impaired nutritional status was associated with earlier onset of the disease symptoms, gastroesophageal reflux disease, muscle tissue depletion and low body weight, but not with other clinical variables in children and adolescents with cystic fibrosis.