Table 1. G551D/F508del subjects.
| Gland numbers | Gland volumes/rates | Ratios | |||||||||||
| ID | G | TestDate | MCh glands (n) | Cktl glands (n) | C/M n glands (%) | MCh Avg. Final Vol. (nl/gland) | MCh Rate Estimate nl/gl/min | Cktl Avg Final Vol (pl/gl) | Cktl Rate (pl/gl/min) | C mean/M mean | % WT | % WT (Highest 12 M-sweat) | % WT (Hi-12, Loss-Corrected) |
| S1 G551D/F508del (−) ivacaftor | |||||||||||||
| S1 | M | 10/20/2011 | 71 | 0 | 0% | 75.83 | 5.06 | 0 | 0 | 0.00% | 0.00% | - | — |
| S1 | M | 1/13/2012 | 85 | 0 | 0% | 64.58 | 4.31 | 0 | 0 | 0.00% | 0.00% | - | — |
| S1 | M | 2/4/2012 | 75 | 0 | 0% | 71.34 | 4.76 | 0 | 0 | 0.00% | 0.00% | - | — |
| S1 (−) Averages | 77 | 0 | 0% | 70.58 | 4.71 | 0 | 0 | 0.00% | 0.00% | 0.00% | 0.00% | ||
| S1 G551D/F508del (+) ivacaftor | |||||||||||||
| S1 | M | 3/23/2012 | 82 | 13 | 16% | 70.99 | 4.73 | 152 | 5.06 | 0.11% | 0.42% | - | — |
| S1 | M | 4/10/2012 | 71 | 15 | 21% | 91.91 | 6.13 | 105 | 3.52 | 0.06% | 0.22% | - | — |
| S1 | M | 4/17/2012 | 66 | 11 | 17% | 97.02 | 6.47 | 110 | 3.66 | 0.06% | 0.22% | - | — |
| S1 (+) A xverages | 73 | 13 | 18% | 86.64 | 5.78 | 122 | 4.08 | 0.07% | 0.29% | 0.64% | 1.59% | ||
| S2 G551D/F508del (−) ivacaftor | |||||||||||||
| S2 | F | 2/2/2012 | 99 | 0 | 0% | 28.61 | 1.91 | 0 | 0 | 0.00% | 0.00% | - | — |
| S2 | F | 3/20/2012 | 100 | 0 | 0% | 29.16 | 1.94 | 0 | 0 | 0.00% | 0.00% | - | — |
| S2 (−) Averages | 99.5 | 0 | 0% | 28.88 | 1.93 | 0 | 0 | 0.00% | 0.00% | 0.00% | 0.00% | ||
| S2 G551D/F508del (+) ivacaftor | |||||||||||||
| S2 | F | 5/8/2012 | 82 | 4 | 5% | 35.55 | 2.37 | 11 | 0.37 | 0.02% | 0.06% | - | — |
| S2 | F | 5/15/2012 | 90 | 1 | 1% | 29.16 | 1.94 | 2 | 0.07 | 0.00% | 0.01% | - | — |
| S2 (+) Averages | 86 | 2.5 | 3% | 32.36 | 2.16 | 7 | 0.22 | 0.01% | 0.04% | 0.13% | 2.75% | ||
| S3 G551D/F508del (+) ivacaftor | |||||||||||||
| S3 | F | 7/24/2012 | 90 | 21 | 23% | 45.03 | 3.00 | 95 | 3.18 | 0.11% | 0.41% | 0.58% | 2.27% |
| S5 G551D/F508del (+) ivacaftor data | |||||||||||||
| S5 | M | 11/2/2012 | 58 | 29 | 50% | 78.94 | 5.26 | 703 | 23.42 | 0.45% | 1.74% | 3.07% | 4.11% |
| S6 G551D/F508del (−) ivacaftor data | |||||||||||||
| S6 | F | 2/2/2012 | 143 | 0 | 0% | 14.37 | 0.96 | 0 | 0 | 0.00% | 0.00% | 0.00% | 0.00% |
| S7 G551D/F508del (−) ivacaftor data | |||||||||||||
| S7 | F | 2/13/2012 | 123 | 0 | 0% | 32.16 | 2.14 | 0 | 0 | 0.00% | 0.00% | 0.00% | 0.00% |
| Summary (−) ivacaftor: 4 G551D subjects | |||||||||||||
| Sums or Averages | 852 | 0 | 0% | 36.50 | 2.43 | 0 | 0 | 0.00% | 0.00% | 0.00% | 0.00% | ||
| Summary (+) ivacaftor 4 G551D subjects | |||||||||||||
| Sums or Averages | 906 | 317 | 29% | 42.93 | 2.84 | 267 | 6.80 | 0.29% | 1.15% | 1.84% | 4.07% | ||
Summary of results for CF subjects with G551D mutations. Six adult G551D CF subjects were tested. Table is arranged by subject and condition, each row shows the results for a single test or means/sums when multiple tests were performed. Subjects had been taking ivacaftor for at least 3 weeks prior to (+) ivacaftor testing. Columns labeled ‘gland numbers’ show the number of glands that secreted to an intradermal injection of MCh, then to the β-adrenergic cocktail at the same site (C-sweat), and the ratio of the two expressed as a percentage. Columns labeled ‘gland volumes/rates’ show the average final M-sweat or C-sweat volumes per gland expressed as nanoliters for a 15 min period (M-sweat) or 30 min period (C-sweat) followed by average rates (per min, per gland). The 4 columns labeled ‘ratios’ show: 1) the mean C-sweat rate as a percent of the mean M-sweat rate; 2) the C/M ratios as a percentage of the average C/M ratio of 0.2651 obtained for 6 control subjects [10]; 3) that value for the 12 glands having the highest M-sweat rates; and 4) the value for the 12 highest glands after correcting for sweat volume loss.