NUTRITIONAL STATUS IN CYSTIC FIBROSIS

Abstract

OBJECTIVE: We aimed to investigate the nutritional status of patients with cystic fibrosis (CF) in relation to their clinical manifestations. METHODS: In 68 patients with CF (aged 2–38 years), body weight, height, and composition (bioelectrical impedance analysis), respiratory function, Pseudomonas colonization, pancreatic function, CF-related diabetes mellitus (CF-DM), and genotype were measured. RESULTS: BMI was <5th percentile in 12 patients (18%), between the 5th and 10th percentiles in 6 (7%), between the 10th and 85th percentiles in 41 (60%), between the 85th and 95th percentiles (overweight) in 4 (6%), and >95th percentile (obese) in 5 (7%). Among 18 patients with a BMI at <10th percentile, 18 (100%) had pancreatic insufficiency, 16 (89%) had Pseudomonas, and 7 (38%) had CF-DM. Among 41 patients with a BMI in the 10th to 85th percentile, 37 (90%) had pancreatic insufficiency, 28 (82%) had Pseudomonas, and 9 (22%) had CF-DM. Among 9 patients with a BMI at >85th percentile, 3 (33%) had pancreatic insufficiency, 1 (11%) had Pseudomonas, and none had CF-DM. Forced expiratory volume in 1 second was significantly better among overweight patients than among patients with a low or normal BMI (P < .05). In addition, forced expiratory volume in 1 second correlated with BMI (P = .014), age (P = .029), and percent free fat mass (P = .039). Overweight/obese patients were homozygotes for mild mutations. CONCLUSIONS: Most patients with CF had an optimal nutritional status. A small percentage were overweight or obese, especially those with pancreatic sufficiency and carriers of mild mutations. These patients had mild-to-moderate lung disease and were less likely to be colonized with Pseudomonas or have liver disease.