Evolución nutricional y función pulmonar en niños y adolescentes chilenos con fibrosis quística / Growth and pulmonary function in Chilean children and adolescents with cystic fibrosis

ABSTRACT

Background: Nutrition influences morbidity and mortality in patients with cystic fibrosis (CF), affecting their lung function. Aim: To characterize the nutritional status of a group of CF patients and to analyze its evolution and relationship to lung function. Patients and Methods: A retrospective cohort of CF children and adolescents attemded in our institution for 15 years, was analyzed. Age and form of presentation, mutation, weight and stature (measured annually at least), microbial colonization and forced expiratory volume in the first second (FEV1) were registered. Results: We gathered information about 33 patients, 64 percent males, diagnosed at 23.8 ± 45.6 months old (range 1-216), 85 percent had a genetic study (10 children had one or more Df508 alleles) and 94 percent had pancreatic insufficiency. In their last visit they were 13.0 ± 5.8 years old, their body mass index z-score (BMIz) was -0.25 ± 1.2 and their FEV1 was 80.4 ± 28.6 percent. According to BMI 73 percent were eutrophic, 18 percent undernourished and 9 percent were overweight. According to weight/ for height index (WH), the figures for eutrophy, undernutrition and overweight were 70, 6 and 24 percent, respectively. Only 12 percent had short stature. Those with P. aeruginosa infection had lower BMI. There was a positive correlation between FEV1 and BMIz (+0.46, p = 0.02), but not with WH. During follow-up, there was a gradual deterioration of weight, height, and BMIz after 10-12 years of age and an overall gradual FEV1 decrease. Conclusions: The prevalence of malnutrition in these patients with CF is high; undernutrition is higher if defined by BMIz and unlike WH, correlates to lung function. Nutritional deterioration starts before adolescence.