Frequency of the delta F508 and exon 11 mutations in Norwegian cystic fibrosis patients.

We have searched for the delta F508 mutation in 77 Norwegian cystic fibrosis patients. Of the 154 chromosomes tested, 93 (60%) carried the delta F508 mutation. Haplotypes at the D7S23 locus (KM19 and XV2C markers) were determined.

Eight-year follow-up of pulmonary function and oxygen uptake during exercise in 16-year-old males with cystic fibrosis.

Eight of nine Norwegian 16-year-old males with cystic fibrosis, and six age-matched, physically active controls were included in an eight-year follow-up study, involving pulmonary and bicycle exercise testing. The individual's level of regular physical exercise was registered, and we investigated whether or not this could be correlated to changes in clinical status, lung function and maximal oxygen uptake. Four males with cystic fibrosis trained regularly for 4-7 h weekly, while the other four patients did no regular exercise.

Dopamine and catecholamines in cystic fibrosis in response to a marathon race.

The excretions of 3,4-dihydroxyphenylalanine (DOPA) metabolites and 5-hydroxyindoleacetic acid (5-HIAA) were followed before, during, and after a marathon race in three patients with cystic fibrosis (CF) and three healthy controls. Moderately increased DOPA excretions in CFs compared with controls before, during, and after the race support the idea that there is an altered metabolism of DOPA in CF. Noradrenaline, adrenaline, homovanillic acid, and vanilmandelic acid were increased in CFs compared with controls mainly during and after the race, which might be a reflection of the heavier working intensity that the CF patients had in comparison with their healthy controls.

Fatty acids in cystic fibrosis in response to a marathon race.

Lipid metabolism was studied in three cystic fibrosis (CF) patients (18 years old) and three healthy controls (35-40 years old) who completed the New York Marathon 1984. Lipase was not detectable in plasma from two of the CF patients, but in one CF patient with a functioning pancreas and in the three controls it increased during and after the run. Cholesterol concentrations were lower in all patients than in the controls.

Biochemical changes and endocrine responses in cystic fibrosis in relation to a marathon race.

Biochemical changes and endocrine responses during the New York Marathon (42195 m) were investigated in three 18-year-old male adolescents with cystic fibrosis (CF) and three healthy men who accompanied the CFs during the race. The ambient temperature was 20 degrees-28 degrees C and the relative humidity 98%-75% during the run. The CF patients, who had Shwachman scores of 60, 85 and 95 completed the run without major problems in 6.