Skip to main content

Table 3 Characteristics of the subjects with stop codon mutations and variation of clinical and functional parameters after treatment with gentamicin.

From: In vitroprediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study

Genotype Sputum colonisation Age (year) Δscore FEV1var FVCvar FEF25–75var Sweat Cl- at D0 Sweat Cl- at D15 ΔCl-free-iso at D0 ΔCl-free-iso at D15 ICC
Y122X+/+ SA 11 -4 24 23 31 126 91 0 0 -
Y122X+/+ PA* 16 -2 -12 -6 -15 79 37 NP 0 -
Y122X+/+ PA*,SA 18 -4 2 -2 -8 109 115 0 NP +
Y122X+/+ PP* 15 -5 25 19 86 90 91 -0.5 0 +
Y122X+/+ PP* 13 -15 18 8 96 103 46 -1.6 -3.8 +
Y122X+/+ SA 22 -13 3 0 7 108 100 -3.7 -17.6 +
Y122X+/+ BC* 21 -22 18 24 150 136 135 0 -4 +
Y122X+/+ PA* 12 -12 3 -9 NP 119 86 0 -8.2 NP
Y122X+/F508del SA* 10.5 -3 21 21 45 114 65 -1 -3.3 +
R1162X +/+ SA 14 -2 0.4 0 4 116 131 0 0 -
F508del/W1282X PA 13 -2 15 14 27 103 100 0 -1.3 NP
G542X +/+ SA 11 -4 21 17 20 113 105 0 0 NP
R553X/CFTRdele17b PA* 10 0 NP NP NP 115 NP -4 NP NP
  1. PA: Pseudomonas aeruginosa; SA: Staphylococcus aureus; PP: Pseudomonas putida; BC: Burkholderia cepacia; * bacteria resistant to gentamicin. FEV1var refers to the ratio (FEV1D15~FEV1D0)/FEV1D0; FVCvar refers to the ratio (FVCD15~FVCD0)/FVCD0. FEF25–75var refers to the ratio (FEF25–75(D15)~FEF25–75(D0))/FEF25–75(D0). Δscore refers to value of CFCSD15-CFCSD0. ΔCl-free-iso refers to ΔCl-free-isoproterenol. ICC: immunocytochemistry. NP: not performed.