Table 1—

Clinical data of patients with atypical cystic fibrosis disease

AgeSexGenotypeSweat chloride mmol·L−1PS/PIFEV1% pred
22M3849+10kbC→T/405+1G→A112PS60
21M3849+10kbC→T/405+1G→A70PS43
19MG85E/5T57PS72
15M5T/?110PI100
16M3849+10kbC→T/ΔF50867PS69
69MD1152H/D1152H15PS100
52M?/?112PI35
38F3849+10kbC→T/W1282X100PS44
23FΔF508/?80PS83
28F3849+10kbC→T/ΔF50880PS88
16M3849+10kbC→T/W1282X67PS95
  • FEV1: forced expiratory volume in one second

  • M: male

  • F: female

  • PS: pancreatic sufficiency

  • PI: pancreatic insufficiency

  • ?: unknown