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

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

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.

MeSH terms

  • Adrenoleukodystrophy* / blood
  • Adrenoleukodystrophy* / diet therapy
  • Adult
  • Counseling
  • Diet, Mediterranean*
  • Fatty Acids / blood
  • Female
  • Humans
  • Male
  • Middle Aged
  • Patient Compliance
  • Retrospective Studies
  • Triglycerides / blood
  • Young Adult

Substances

  • Fatty Acids
  • Triglycerides

Grants and funding

The study was supported by the Italian Ministry of Health with “current research funds”. Dr. Manco also received Italian Ministry of Health FPRC 5xmille2023 funds. The funder had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript; or in the decision to publish the results.