查看文章

Cystic fibrosis (CF) is the most common life threatening autosomal recessive disorder in the white population. Wasting has long been recognised as a poor prognostic marker in CF. Whether it predicts survival independently of lung function and arterial blood gas tensions has not previously been reported.

584 patients with CF (261 women) of mean (SD) age 21 (7) years were studied between 1985 and 1996, all of whom were being followed up in a tertiary referral centre. Lung function tests, body weight, arterial blood oxygen (Pao ₂) and carbon dioxide (Paco ₂) tensions were measured. The weight was calculated as a percentage of the ideal body weight for age, height, and sex.

Forced expiratory volume in one second (FEV₁) recorded at the start of the study was 1.8 (1.0) l (52 (26)% predicted FEV₁), Pao ₂ 9.8 (1.9) kPa, Paco ₂ 5.0 (0.9) kPa, and % ideal weight 92 (18 …