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. 2024 Oct 1;16(19):3341.
doi: 10.3390/nu16193341.

Nutritional Counseling and Mediterranean Diet in Adrenoleukodystrophy: A Real-Life Experience

Maria Rita Spreghini  1 , Nicoletta Gianni  2 , Tommaso Todisco  1   3 , Cristiano Rizzo  4 , Marco Cappa  5 , Melania Manco  2
Affiliations

Nutritional Counseling and Mediterranean Diet in Adrenoleukodystrophy: A Real-Life Experience

Maria Rita Spreghini et al. Nutrients. .

Abstract

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.

Keywords: C26:0; Mediterranean diet; VLCFA-restricted diet; VLCFAs; X-ALD; adrenoleukodystrophy; hexacosanoic acid; nutritional therapy; very-long-chain fatty acids.

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Conflict of interest statement

The authors report that there are no competing interests to declare.

Figures

Figure 1
Figure 1
Distribution of C26:0 plasma levels (A), C26:0/C22:0 ratio (B) and triglycerides (C) at time T1 in adult patients compliant to nutritional treatment as compared to non-compliant patients. Figure 1B presents an outlier.
Figure 2
Figure 2
Distribution of C26:0 plasma levels (A), C26:0/C22:0 ratio (B) and cholesterol (C) in adult patients compliant to nutritional treatment, comparing T0 vs. T1. Figure 2B presents an outlier.
Figure 3
Figure 3
Distribution of C26:0/C22:0 ratio at time T1 in carriers compliant to nutritional treatment as compared to non-compliant carriers. The figure presents outliers.
Figure 4
Figure 4
Distribution of C26:0/C22:0 ratio (A) and C24:0/C22:0 ratio (B) in carriers compliant to nutritional treatment, comparing T0 vs. T1. Both the figures present outliers.
See this image and copyright information in PMC

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