Table 1.
Patient | Agea | Sex | Mutation 1 | Mutation 2 | Pancreatic Status | Current Smoker | Baseline FEV1 (L)b (% predicted) | Baseline FVC (L)c (% predicted) | TISd | AZMe | LABAf | ICSg | CFh
Co-morbidities |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 19 | M | F508del | F508del | I | N | 2.62 (74) | 4.09(100) | Y | N | N | N | DIOS, B |
2 | 19 | M | F508del | 3849+1G->A | I | N | 3.07 (71) | - | N | N | N | N | Liver, S |
3 | 34 | M | F508del | E56K | S | N | 3.87 (106) | 4.98(115) | N | N | N | N | None |
4 | 18 | F | M1101K | M1101K | I | N | 2.22 (71) | 3.06(93) | Y | N | N | N | |
5 | 24 | F | F508del | F508del | I | Unknown | 1.19 (35) | 1.84(47) | N | N | N | N | DIOS |
6 | 28 | F | F508del | F508del | I | N | 2.39 (89) | 3.72(122) | N | N | N | N | IGT,DIOS |
7 | 38 | F | F508del | R347H | S | Unknown | 2.15 (77) | 3.14(95) | N | N | N | N | S |
8 | 48 | M | F508del | F508del | I | N | 2.30 (54) | 4.79(90) | Y | Y | Y | N | IGT |
9 | 19 | M | 1717-1G->A | Unknown | S | N | 3.73 (82) | 4.55(85) | N | N | N | N | S |
10 | 21 | M | F508del | F508del | I | Y | 2.63 (40) | 3.61(84) | N | N | N | N | DIOS |
11 | 33 | F | F508del | R334W | I | N | 1.11 (71) | 2.34(74) | N | N | N | N | DIOS |
12 | 26 | M | G542x | Unknown | I | Y | 2.96 (69) | 4.99(100) | N | Y | Y | N | CFRD |
13 | 26 | F | Unknown | Unknown | I | N | 2.80 (88) | 4.10(108) | Y | N | N | N | CFRD,B, S, DIOS, |
14 | 24 | M | F508del | F508del | I | N | 3.84 (84) | 5.80(115) | Y | N | N | Y | Liver |
15 | 28 | F | M1101K | M1101K | I | N | 2.67 (91) | 3.37 (101) | N | N | N | N | B,S |
I insufficient, S sufficient, Y yes, N no
a Age at time of first S. pyogenes isolation.
b Baseline FEV 1 forced expiratory volume in one second recorded 2 years prior to the index GAS isolate
c Baseline FVC forced volume capacity recorded 2 years prior to the index GAS isolate
d TIS Chronic inhaled tobramycin therapy
e AZM Chronic azithromycin
f LABA Long-acting beta agonist
g ICS Inhaled corticosteroid
h CF Comorbidities, DIOS Distal Ileal Obstruction Syndrome, B osteoporosis, Liver liver disease, S sinus disease, IGT Impaired Glucose Tolerance, CFRD CF related diabetes