Effect of dehydroepiandrosterone supplementation on fatty acid and hormone levels in patients with X-linked adrenoleucodystrophy

source extracted confidence: high 2026-04-09
#adjuvants-immunologic #administration-oral #adrenoleukodystrophy #adult #androstenedione

Effect of dehydroepiandrosterone supplementation on fatty acid and hormone levels in patients with X-linked adrenoleucodystrophy

Assies et al., 2003 | Clin Endocrinol (Oxf) | Rct

Citation

Assies J, Haverkort E B, ... Vreken P. Effect of dehydroepiandrosterone supplementation on fatty acid and hormone levels in patients with X-linked adrenoleucodystrophy. Clin Endocrinol (Oxf). 2003-Oct;59(4):459-66

Abstract

OBJECTIVE: In X-linked adrenoleucodystrophy (X-ALD) the peroxisomal beta-oxidation of saturated very long-chain fatty acids (VLCFAs; carbon length > 22 atoms) is impaired. These fatty acids accumulate in blood and tissues, in particular in the nervous system, adrenal cortex and testis. Most patients have a primary adrenocortical insufficiency with low levels of cortisol and dehydroepiandrosterone (DHEA) and its sulphate ester (DHEA-S), collectively called DHEA(S). Surprisingly, very low plasma levels of DHEA(S) may be found when plasma cortisol and ACTH levels are normal. In animal studies DHEA administration had a peroxisome proliferating effect and induced the expression of peroxisomal enzymes involved in the beta-oxidation of fatty acids. PATIENTS AND DESIGN: To study the effect of DHEA on fatty acids in X-ALD patients, we conducted a randomized double-blind study in which 14 men (age range 21-63 years) and one boy (12 years) received 50 mg of DHEA or placebo for 3 months, followed by a 1-month wash-out period, then 3 months of placebo or vice versa. RESULTS: A significant rise was seen in the plasma levels of DHEA-S, Delta4-androstenedione and IGF-I. The elevated saturated VLCFAs in plasma and erythrocytes did not change. However, in erythrocytes significant decreases were found in the total amount of fatty acids, in C16:0, C18:0 and in C20:4omega-6, C22:5omega-6, C18:1omega-9, C20:1omega-9 and C20:3omega-9. In plasma, decreases were found for C18:1omega-9 and increases for C20:1omega-9. CONCLUSIONS: Dehydroepiandrosterone supplementation for 3 months did not lower the elevated plasma levels of saturated very long-chain fatty acids in patients with X-linked adrenoleucodystrophy. Instead, a decrease in saturated and mono- and polyunsaturated fatty acids in erythrocytes and plasma was found. An increase of C20:1omega-9 was found in plasma only.

Key Findings

A significant rise was seen in the plasma levels of DHEA-S, Delta4-androstenedione and IGF-I. The elevated saturated VLCFAs in plasma and erythrocytes did not change. However, in erythrocytes significant decreases were found in the total amount of fatty acids, in C16:0, C18:0 and in C20:4omega-6, C22:5omega-6, C18:1omega-9, C20:1omega-9 and C20:3omega-9. In plasma, decreases were found for C18:1omega-9 and increases for C20:1omega-9.

Outcomes Measured

  • cortisol levels

Population

Field Value
Population x
Sample Size See abstract
Age Range 21-63 years
Condition See abstract

MeSH Terms

  • Adjuvants, Immunologic
  • Administration, Oral
  • Adrenoleukodystrophy
  • Adult
  • Androstenedione
  • Child
  • Dehydroepiandrosterone
  • Double-Blind Method
  • Erythrocytes
  • Fatty Acids
  • Fatty Acids, Unsaturated
  • Humans
  • Insulin-Like Growth Factor I
  • Male
  • Middle Aged

Evidence Classification

  • Level: Rct
  • Publication Types: Clinical Trial, Journal Article, Randomized Controlled Trial
  • Vertical: dhea

Provenance

  • PMID: 14510908
  • DOI: (not available)
  • PMCID: Not in PMC
  • Verified: 2026-04-10 via PubMed E-utilities API

Source extracted via PubMed E-utilities API on 2026-04-10