Nutritional Counseling and Mediterranean Diet in Adrenoleukodystrophy: A Real-Life Experience.
Study Goal
The researchers aimed to evaluate the effectiveness of a VLCFA-restricted Mediterranean diet in reducing plasma VLCFA levels in patients and carriers of adrenoleukodystrophy (X-ALD).
Results Summary
Compliant adult patients and carriers showed significant reductions in plasma VLCFA levels (C26:0, C26:0/C22:0 ratio) and triglycerides after one year on the diet. The diet was effective in lowering these biomarkers compared to non-compliant individuals and baseline measurements.
Population
36 patients and 20 carriers of adrenoleukodystrophy (X-ALD).
Effective Dosage
Not specified
Duration
1 year
Interactions
None mentioned
| Intervention | Direction | Endpoint | Population | Dosage | Impact | Claim # |
|---|---|---|---|---|---|---|
VLCFA-restricted Mediterranean diet | decrease | C26:0 levels | compliant adult patients | 1.7 (1.2) vs. 2.5 µmol/L (1.7) | significantly lower | #1 |
VLCFA-restricted Mediterranean diet | decrease | C26:0/C22:0 ratio | compliant adult patients | 0.04 (0.02) vs. 0.06 (0.03) | significantly lower | #2 |
VLCFA-restricted Mediterranean diet | decrease | triglycerides | compliant adult patients | 93 (56.5) vs. 128 mg/dL (109.5) | significantly lower | #3 |
VLCFA-restricted Mediterranean diet | decrease | C26:0 | compliant adult patients | 2.4 (1.7) vs. 1.7 (1.2) µmol/L | significantly reduced | #4 |
VLCFA-restricted Mediterranean diet | decrease | C26:0/C22:0 ratio | compliant adult patients | 0.06 (0.04) vs. 0.04 (0.02) | significantly reduced | #5 |
VLCFA-restricted Mediterranean diet | decrease | cholesterol | compliant adult patients | 173.5 (68.3) mg/dL vs. 157 (54) mg/dL | significantly reduced | #6 |
VLCFA-restricted Mediterranean diet | decrease | C26:0/C22:0 ratio | compliant carriers | 0.02 (0.01) vs. 0.04 (0.009) | lower | #7 |
VLCFA-restricted Mediterranean diet | decrease | C26:0/C22:0 ratio | compliant carriers | 0.03 (0.02) vs. 0.02 (0.01) | significantly decreased | #8 |
VLCFA-restricted Mediterranean diet | decrease | C24:0/C22:0 ratio | compliant carriers | 1.0 (0.2) vs. 0.9 (0.3) | significantly decreased | #9 |
VLCFA-restricted diet | decrease | plasma VLCFA levels and their ratios | patients and carriers | - | effective in reducing | #10 |
Background/Objectives: Adrenoleukodystrophy (X-ALD) is a metabolic disorder caused by dysfunctional peroxisomal beta-oxidation of very-long-chain fatty acids (VLCFAs). A VLCFA-restricted Mediterranean diet has been proposed for patients and carriers to reduce daily VLCFA intake. Methods: We retrospectively evaluated plasma VLCFAs in a cohort of 36 patients and 20 carriers at baseline and after 1 year of restricted diet. Results: At T1, compliant adult patients had significantly lower C26:0 levels [1.7 (1.2) vs. 2.5 µmol/L (1.7), p < 0.05], C26:0/C22:0 ratio [0.04 (0.02) vs. 0.06 (0.03), p < 0.05], and triglycerides [93 (56.5) vs. 128 mg/dL (109.5), p < 0.05] than non-compliant ones. C26:0 [2.4 (1.7) vs. 1.7 (1.2) µmol/L, p < 0.05], the C26:0/C22:0 ratio [0.06 (0.04) vs. 0.04 (0.02), p < 0.05], and cholesterol [173.5 (68.3) mg/dL vs. 157 (54) mg/dL, p < 0.05] were significantly reduced in compliant adult patients at T1 vs. baseline. As for carriers, the C26:0/C22:0 ratio was lower [0.02 (0.01) vs. 0.04 (0.009), p < 0.05] at T1 in compliant carriers, as compared to non-compliant ones. The C26:0/C22:0 [0.03 (0.02) vs. 0.02 (0.01) p < 0.05] and C24:0/C22:0 [1.0 (0.2) vs. 0.9 (0.3), p < 0.05] ratios were significantly decreased at T1 vs. T0. Conclusions: A VLCFA-restricted diet is effective in reducing plasma VLCFA levels and their ratios and must be strongly encouraged as support to therapy.