TABLE 1

Study population

MelbournePerth
Subjects111149
Visits495537
Age at first BAL years0.3±0.10.3±0.1
Age at last BAL years6.0±0.16.2±0.3
Male66 (59.6)67 (45.2)
BMI at first BAL kg·m–215.7±1.715.3±2.3
BMI at last BAL kg·m–216.1±1.615.7±1.7
Mode of presentation
 Newborn screening70 (63.6)122 (85.9)
 Meconium ileus21 (19.1)8 (5.63)
 Family history8 (7.3)1 (0.7)
 Respiratory symptoms3 (2.7)2 (1.4)
 Failure to thrive1 (0.9)6 (4.2)
 Other7 (6.4)3 (2.1)
Genotype
 Homozygous Phe508del/Phe508del61 (55.0)68 (45.6)
 Heterozygous Phe508del39 (35.1)72 (48.3)
 Other11 (9.9)9 (6.0)
Hospitalisation and treatment
 Patients with hospitalisation data66 (59.4)66 (44.3)
 Patients with no respiratory admissions75 (67.4)96 (64.0)
 Antibiotics prescribed105 (94.1)131 (87.9)
  • Data are presented as n, mean±sd or n (%). BAL: bronchoalveolar lavage; BMI: body mass index.