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/m2. Malnutrition (BMI<18.5 kg/m2) 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).