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. 2018 Aug 15;198(4):526-528.
doi: 10.1164/rccm.201802-0243LE.

Changes in Lung Clearance Index in Preschool-aged Patients with Cystic Fibrosis Treated with Ivacaftor (GOAL): A Clinical Trial

Felix Ratjen  1 , Michelle Klingel  1 , Philip Black  2 , Michael R Powers  3 , Hartmut Grasemann  1 , Melinda Solomon  1 , Scott D Sagel  4 , Scott H Donaldson  5 , Steven M Rowe  6 , Margaret Rosenfeld  7
Affiliations

Changes in Lung Clearance Index in Preschool-aged Patients with Cystic Fibrosis Treated with Ivacaftor (GOAL): A Clinical Trial

Felix Ratjen et al. Am J Respir Crit Care Med. .
No abstract available

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Figures

Figure 1.
Figure 1.
(A) Absolute and (B) relative median changes per subject in lung clearance index (LCI) in preschool children treated with ivacaftor. Absolute LCI significantly improved 1 month after the first dose of ivacaftor (median [interquartile range (IQR)] LCI at 1 mo, 7.1 [6.6 to 8.5]; P = 0.02). The improvement in LCI was maintained 6 months after the first dose (median [IQR] LCI, 7.5 [7.3 to 7.6]; P = 0.03). Similarly, relative LCI significantly improved from baseline at both 1 month (median [IQR] change in LCI from baseline, −23.6% [−34.2 to −20.2]; P < 0.001) and 6 months after treatment (median [IQR] change in LCI from baseline, −24.6 [−31.4 to −20.4]; P < 0.001). The dotted lines represent an upper limit of normal of 8 in A and a change of 0% in B.
See this image and copyright information in PMC

References

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