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Table 1 Improvement of functional parameters during ivacaftor therapy

From: Ivacaftor in a young boy with the rare gating mutation S549R - use of lung clearance index to track progress: a case report

  Start 6 weeks 12 weeks 28 weeks 41 weeks
Sweat chloride level [mmol/l] 115 40 30 40 52
FEV1 [l ] (z-score) a 1.25 (−1.2) 1.53 (0.5) 1.48 (0.2) 1.63 (0.7) 1.65 (0.5)
LCI (z-score) b 14.5 (14.2) 8.3 (2.4) 7.6 (1.1) 8.1 (2.0) 7.8 (1.5)
Weight [kg] (z-score) 21.8 (−1.5) 23.2 (−1.1) 23.7 (−0.9) 23.9 (−0.9) 26 (−0.5)
BMI [kg/m2] (z-score) 14.6 (−0.8) 15.6 (−0.2) 15.7 (−0.1) 15.5 (−0.2) 16.4 (0.2)
  1. Improvement of different functional parameters during 41 weeks of ivacaftor therapy: FEV1 Forced expiratory volume in the first second, LCI lung clearance index. aReference population for z-score values of FEV1 are the global multi-ethnic reference equations of the global lung function initiative [16]. bReference population for z-score values of the LCI is our intracentric age-matched healthy control population