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Table 11 (abstract P30). Characteristics of the patients and main results

From: Abstracts from the 23rd Italian congress of Cystic Fibrosis and the 13th National congress of Cystic Fibrosis Italian Society

 

Patient 1

Patient 2

Current age (years)

32

47

Gender

Female

Female

Genotype

3272-26AG /E585X

3849+10kbCT /F508del

Sweat chloride (mmol/L) Before Ivacaftor

82

57

After Ivacaftor

83 (6 and 18 months)

19 (6 months)

FEV1 (% PREDICTED) Before Ivacaftor

29

38

After Ivacaftor

44 (6 months); 52 (18 months)

50 (6 months)

6MWT (meters) Before Ivacaftor

500

508

After Ivacaftor

630 (6 and 18 months)

572 (6 months)

Days of antibiotic therapy 12 months Before Ivacaftor

152 (67 IV and 85 oral)

88 (36 IV and 52 oral)

After Ivacaftor

49 (18 months) oral

0 (6 months)

CFQ-R Respiratory Domain

33 (Basal)

78 (18 months)

72 (Basal)

83 (6 months)