Table 1—

Patient characteristics

No chronic P. aeruginosa infection
Chronic P. aeruginosa infection
Male subjects
Female subjects
Male subjects
Female subjects
CFRDNGTCFRDNGTCFRDNGTCFRDNGT
n44 (24)110 (62)52 (34)93 (62)106 (62)166 (96)121 (77)120 (73)
Median age (years)25.5 (27.5)18.0* (17.0)24.5 (26.0)18.0* (17.0)24.5 (26.0)19.5* (20.0)25.0 (25.0)18.0* (18.0)
Median age at diagnosis (months)4.0 (4.0)10.0 (5.5)12.0 (15.0)6.0 (6.0)6.0 (7.5)6.0 (6.0)6.0 (6.0)5.0 (5.0)
Median % predicted FEV171.1 (69.7)71.4 (73.3)53.6 (57.5)73.6* (75.4)49.0 (49.0)59.0* (59.8)42.0 (42.5)61.0* (59.7)
Median % predicted FVC179.8 (79.3)81.0 (81.1)67.8 (84.5)85.2* (71.4)62.1 (59.9)70.2* (69.3)60.8 (62.9)73.8* (72.1)
Median height Z score−0.91 (−0.83)−0.42* (−0.33)−0.78 (−0.63)−0.46 (−0.65)−0.98 (−0.83)−0.44* (−0.36)−0.77 (−0.79)−0.44 (−0.42)
Median weight Z score−0.78 (−0.62)−0.53 (−0.30)−0.64 (−0.66)−0.16* (0.06)−0.82 (−0.94)−0.89 (−0.89)−0.86 (−0.83)−0.52* (−0.42)
Median BMI (kg/m2)21.8* (21.9)20.0 (20.2)20.1 (20.2)20.5 (20.5)21.5 (20.9)19.4* (19.1)20.0 (20.2)19.6 (19.6)
Median lipase dose (units/kg/day)7002 (4495)6342 (4987)4939 (3933)5725 (5111)7112 (5298)8034 (6592)6530 (4325)6885 (5777)
Percent receiving CFRD medication91 (96)86 (88)93 (98)92 (91)
  • Data shown are for all genotypes (homozygous ΔF508).

  • *

    * P < 0.05 between CFRD male and female subjects (all genotypes) vs. their sex-matched NGT control subjects (all genotypes);

  • P < 0.05 between homozygous ΔF508 CFRD male and female subjects vs. their genotype- and sex-matched NGT control subjects.