Malnutrition in adults with cystic fibrosis

Objective: To determine the prevalence and clinical features of malnutrition and its relationship with the CFTR genotype in a cystic fibrosis (CF) adult population. Design: Cross-sectional study. Setting: Department of Pulmonology, Cochin Hospital, Paris, France. Subjects: 163 CF adults seen between 1997 and 1999. Results: Mean age was 28.8 y. Mean body mass index (BMI) was 19.1 kg/m(2). Malnutrition ( BMI <18.5 kg/m(2)) was seen in 81 patients (49.7%). Its severity was associated with diagnosis of CF before the age of 18 y (P<0.01), FEV1 values below 30% (P<0.01), the yearly decline of FEV1 ( P<0.01), pancreatic insufficiency ( P<0.01) and gastro-oesophageal reflux ( P<0.01). Malnutrition was observed in 58.7% of patients with a severe CFTR genotype but in 28.6% of patients with a mild genotype ( P<0.001). Conclusion: Malnutrition remains frequent in adults with CF except in patients presenting with a mild CFTR genotype ( leading to a mild phenotype and to later diagnosis). Sponsorship: None.