Table 1.
Median and IQR [median (IQR)] of plasma VLCFA levels (C26:0, C26:0/C22:0 ratio, C24:0/C22:0 ratio), total cholesterol and triglycerides, at T0 and T1, divided by compliant and non-compliant to nutritional treatment, for each sample group.
| Patients | Carriers | |||||
|---|---|---|---|---|---|---|
| Children | Adults | Female Carriers | ||||
| T0 | T1 | T0 | T1 | T0 | T1 | |
| Age, Years Old | 8.9 (8.1) | 39.1 (22.3) | 51.7 (14.3) | |||
| Weight, kg | 32.6 (21) | 33.3 (18.4) | 72.2 (13) | 72 (7.1) | 61.7 (20.4) | 61.6 (17) |
| Height, cm | 139.6 (32.4) | 146.3 (33.3) | 173 (10.6) | 173 (11.1) | 158 (5.5) | 158 (4.9) |
| BMI, kg/m2 | 17.6 (2.9) | 17.3 (2.3) | 23.5 (4.3) | 23.8 (4.8) | 24.7 (4.9) | 22.3 (5.5) |
| C26:0, µmol/L | 1.4 (0.9) | 1.6 (0.7) vs. 1.5 (0.7) | 2.4 (1.7) | 1.7 (1.2) vs. 2.5 (1.7) * b ** b |
1.4 (0.7) | 1.2 (0.7) vs. 1.8 (0.2) |
| C26:0/C22:0 ratio | 0.04 (0.03) | 0.03 (0.03) (A) 0.04 (0.008) (B) |
0.06 (0.04) | 0.04 (0.02) (A) 0.06 (0.03) (B) * b ** e |
0.03 (0.02) | 0.02 (0.01) (A) 0.04 (0.009) (B) * d ** a |
| C24:0/C22:0 ratio | 1.2 (0.2) | 1.1 (0.08) (A) 1.2 (0.2) (B) |
1.4 (0.3) | 1.2 (0.5) (A) 1.4 (0.4) (B) |
1.0 (0.2) | 0.9 (0.3) (A) 1.2 (0.3) (B) ** b |
| Cholesterol, mg/dL | 141.5 (32.8) | 123 (29) (A) 149 (35) (B) |
173.5 (68.3) | 157 (54) (A) 193 (39) (B) ** b |
191 (50.8) | 183 (40.5) (A) 186.5 (12.8) (B) |
| Triglycerides, mg/dL | 63 (19.8) | 62 (81) (A) 70.5 (19.5) (B) |
103 (63.8) | 93 (56.5) (A) 128 (109.5) (B) * c |
77.5 (37) | 74 (48.3) (A) 84.5 (79.8) (B) |
| Adherence to Nutritional Treatment, % | 50% | 53.9% | 80% | |||
(A): compliant to nutritional treatment; (B): non-compliant to nutritional treatment; * significant comparison between compliant and non-compliant at T1; ** significant comparison between compliant at T1 and T0. a: p = 0.001; C 26:0: hexacosanoic acid; b: p = 0.01; C26:0/C22:0 ratio: hexacosanoic acid/docosanoic acid ratio; c: p = 0.02; C24:0/C22:0 ratio: tetracosanoic acid/docosanoic acid ratio; d: p = 0.03; e: p = 0.05.