Table 1.
Patient Characteristics by Cystic Fibrosis Transmembrane Regulator Mutation Status
| All Eligible Patients N = 66 | CFTR Mutation Class I-III N = 54 | CFTR Mutation Class IV-V N = 12 | |||||
|---|---|---|---|---|---|---|---|
| Variable | Observed | N | Observed | N | Observed | N | p Value |
| Age – mean (SD), years | 32.3 (11.4) | 66 | 30.4 (8.8) | 54 | 40.8 (17.2) | 12 | 0.0637 |
| Male gender | 32 (48.5) | 66 | 27 (50.0) | 54 | 5 (41.7) | 12 | 0.601 |
| Medicaid Insurance | 23 (35.4) | 65 | 17 (32.1) | 53 | 6 (50.0) | 12 | 0.241 |
| CFTR mutation status | 66 | ||||||
| Homozygous Class I-III | 54 (81.8) | ||||||
| Heterozygous Class I-III | 12 (18.2) | ||||||
| Homozygous Class IV-V | 0 (0) | ||||||
| FEV1 - % predicted (SD) | 59.3 (23.3) | 66 | 57.3 (22.7) | 54 | 68.3 (24.9) | 12 | 0.178 |
| Weight – mean (SD), kg | 66.1 (14.6) | 66 | 63.9 (11.9) | 54 | 76.0 (21.2) | 12 | 0.079 |
| BMI – mean (SD), kg/m2 | 23.4 (4.3) | 66 | 22.8 (3.5) | 54 | 26.2 (6.4) | 12 | 0.099 |
| BMI < 18.5 | 9 (13.6) | 66 | 7 (13.0) | 54 | 2 (16.7) | 12 | 0.663b |
| BMI 18.5–24.99 | 38 (57.6) | 66 | 34 (63.0) | 54 | 4 (33.3) | 12 | 0.104b |
| BMI 25–29.99 | 13 (19.7) | 66 | 11 (20.4) | 54 | 2 (16.7) | 12 | 1.000b |
| BMI ≥ 30 | 6 (9.1) | 66 | 2 (3.7) | 54 | 4 (33.3) | 12 | 0.008 b |
| BMI meeting CFF goala | 38 (57.6) | 66 | 29 (53.7) | 54 | 9 (75.0) | 12 | 0.177b |
| CF-related diabetes on insulin | 15 (23.1) | 65 | 14 (26.4) | 53 | 1 (8.3) | 12 | 0.267b |
| CF-related PI on enzymes | 58 (89.2) | 65 | 52 (98.1) | 53 | 6 (50.0) | 12 | < 0.001 b |
| Prednisone use (at time of survey) | 5 (7.7) | 65 | 5 (9.4) | 53 | 0 (0) | 12 | 0.575b |
| PEG tube in place | 2 (3.1) | 65 | 1 (1.9) | 53 | 1 (8.3) | 12 | 0.338b |
| Nutritional supplement use | 32 (49.2) | 65 | 26 (49.1) | 53 | 6 (50.0) | 12 | 0.953 |
Data are presented as No. (%) unless indicated otherwise. Certain background clinical data were not recorded and were thus unavailable for one individual
aCFF goal for weight is BMI ≥22 kg/m2 for females, and ≥ 23 kg/m2 for males
b= Fisher’s exact test
CF cystic fibrosis, SD standard deviation, CFTR cystic fibrosis transmembrane conductance regulator gene, FEV1 forced expiratory volume in one second, BMI body mass index, CFF Cystic Fibrosis Foundation, PI pancreatic insufficiency, PEG percutaneous endoscopic gastrostomy