Table 1.
Cohort characteristics for subjects in the study. Body mass index (BMI), BMI percentile, and percent predicted FEV1 (FEV1) from the most recent in person clinic visit. Oral glucose tolerance test (OGTT) results from within 5 years. OGTT results indicate cystic fibrosis related diabetes (CFRD) if the fasting glucose is ≥126 mg/dL or the two-hour glucose is ≥200 mg/dL. OGTT results indicate Impaired glucose tolerance (IGT) if the two-hour glucose exceeds 140 mg/dL. OGTT indicates normal glucose tolerance (NGT) if the fasting glucose is < 100 mg/dL and two-hour glucose is < 140 mg/dL. Hemoglobin A1C (A1C) result is reported from within one year of CGM 1. Abbreviations: y (years), m (months), M (male), F (female), ETI (elexacaftor/tezacaftor/ivacaftor), Iva (Ivacaftor).
| No. | Age (y) | Sex | BMI (kg/m2) |
FEV1 | Mutation 1 (class) | Mutation 2 (class) | Exocrine Status | A1C | OGTT | Time since OGTT | CFTR Mod and duration |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 25.2 | M | 26.2 | 109 | F508del (2) | F508del (2) | PI | 4.9 | CFRD | 8 m | ETI 1y1m |
| 2 | 22.2 | M | 19.9 | 101 | F508del (2) | F508del (2) | PI | 5.2 | CFRD | 1 m | ETI 1y10m |
| 3 | 41.3 | M | 21.6 | 84 | F508del (2) | 1898 1G > A (1) | PI | 5.6 | CFRD | 2y9m | ETI 2y9m |
| 4 | 34.7 | M | 25.5 | 38 | F508del (2) | R1066C (2) | PI | 6.5 | n/a | n/a | ETI 1y2m |
| 5 | 29.4 | M | 25.7 | 94 | F508del (2) | F508del (2) | PI | 5.4 | IGT | 5 m | ETI 1y1m |
| 6 | 32.1 | F | 22.4 | 39 | F508del (2) | F508del (2) | PI | 5.2 | IGT | 3y | ETI 1y5m |
| 7 | 36.3 | M | 27.3 | 92 | F508del (2) | 621 + 1 -->G > T (1) | PI | 5.4 | IGT | 1y | ETI 1y5m |
| 8 | 16.5 | M | 23 74 %le |
97 | F508del (2) | F508del (2) | PI | 5.1 | IGT | 5 m | ETI 1y3m |
| 9 | 22.5 | F | 23.4 | 66 | F508del (2) | F508del (2) | PI | 5.3 | IGT | 3 m | ETI 1y11m |
| 10 | 25.7 | M | 27.7 | 89 | F508del (2) | N1303K (2) | PI | 5.8 | IGT | 1 m | ETI 1y10m |
| 11 | 21.1 | F | 19.4 | 80 | F508del (2) | 621 + 1G > T (1) | PI | 5.6 | IGT | 1y6m | ETI 2y1m |
| 12 | 14.9 | M | 21.4 66 %le |
97 | F508del (2) | 1717- 1G > A (1) | PI | 5.4 | NGT |
1 m | ETI 1y4m |
| 13 | 15.9 | M | 28.2 96 %le |
97 | R117H (4) | G542X (1) | PS | 5.2 | NGT | 2y | Iva 5y |
| 14 | 48.7 | F | 25.9 | 97 | F508del (2) | G551D (4) | PI | 5.3 | NGT | 5 m | ETI 1y5m |
| 15 | 31.2 | M | 32.5 | 98 | F508del (2) | E822X (1) | PI | 4.4 | NGT | 0 m | ETI 1y1m |
| 16 | 15.2 | M | 20.8 57 %le |
96 | F508del (2) | 1717––1 G > A (1) | PI | 5.2 | NGT | 7 m | ETI 1y5m |
| 17 | 31.3 | M | 19.6 | 75 | F508del (2) | G542X (1) | PI | 5.2 | NGT | 3y2m | None |
| 18 | 17 | F | 19 31 %le |
109 | F508del (2) | R347P (4) | PI | 5.5 | NGT | 3 m | ETI 1y8m |
| 19 | 51.2 | F | 37.6 | 44 | 3849 + 10kbC-> T (5) | 2184insA (1) | PI | 5.5 | NGT | 6 m | Iva 3y8m |
| 20 | 22.2 | M | 23.3 | 123 | F508del (2) | 1717 G1G > A (1) | PI | 5.3 | NGT | 4 m | ETI 1y6m |