Table 1.
Anthropometric and biochemical parameters in 105 CF patients heterozygous for the F508del and another CFTR mutation at baseline and after 1 year of ETI therapy.
| Baseline | 1 year of ETI | p | |
|---|---|---|---|
| Body mass index (kg/m2) | 21.0 (19.9–22.6) | 23.0 (21.0–24.6) | < 0.001 |
| FEV 1 (%) | 58.5 (37.0–80.0) | 74.0 (50.3–93.0) | < 0.001 |
| Liver parameters | |||
| ALT (U/L) | 20 (12–28) | 24 (18–35) | < 0.001 |
| Total bilirubin (mg/dL) | 0.50 (0.31–0.67) | 0.74 (0.59–1.10) | < 0.001 |
| Conjugated bilirubin (mg/dL) | 0.20 (0.15–0.30) | 0.30 (0.2–0.4) | < 0.001 |
| GGT (U/L) | 15 (10–13) | 16 (13–23) | 0.028 |
| Alkaline phosphatase (U/L) | 95 (74–125) | 101 (81–126) | 0.15 |
| Lipid metabolism | |||
| Total cholesterol (mg/dL) | 140 (119–158) | 156 (138–178) | < 0.001 |
| LDL cholesterol (mg/dL) | 74 (53–90) | 88 (74–103) | < 0.001 |
| HDL cholesterol (mg/dL) | 53 (41–67) | 51 (44–65) | 0.087 |
| Triglycerides (mg/dL) | 76 (62–92) | 75 (61–103) | 0.63 |
| Cholestanol (mg/dL) | 0.33 (0.24–0.41) | 0.36 (0.28–0.42) | 0.09 |
| Phytosterols (mg/dL) | 0.40 (0.27–0.64) | 0.45 (0.32–0.59) | 0.36 |
| Lathosterol (mg/dL) | 0.37 (0.27–0.48) | 0.30 (0.22–0.41) | 0.01 |
Data are reported as median (interquartile range).
FEV forced expiratory volume, ALT alanine aminotransferase, GGT gamma-glutamyltransferase, LDL low-density lipoprotein, HDL high-density lipoprotein.