Effect of Dehydroepiandrosterone (DHEA) Supplementation on Fatty Acid and Hormone Levels in Patients with X-linked Adrenoleukodystrophy

  • Johanna Assies
  • Elisabeth B. Haverkort
  • Ritsaert Lieverse
  • Peter Vreken
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 544)


In X-linked adrenoleukodystrophy (X-ALD) the peroxisomal ß-oxidation of saturated very long chain fatty acids (VLCFA-carbon length >22 atoms) is impaired. These fatty acids accumulate in blood and tissues, in particular in the nervous system, adrenal cortex and testes. 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 still normal (Assies et al 1998). In animal studies DHEA-administration had a peroxisome proliferator effect and induced the expression of peroxisomal enzymes involved in the ß-oxidation of fatty acids (Waxman,1996, Depreter et al.). To study the effect of DHEA on fatty acids in X-ALD patients we conducted a randomised double blind study in which fourteen men, age range 21-63 years and one boy (12 year), received 50 mg of DHEA or placebo for 3 months, followed by a one month wash-out period, then 3 month of placebo or vice versa.


Placebo Cortisol Testosterone DHEA Androstenedione 


  1. Assies, J., van Geel, B.M., Barth, P.G., 1998, Low dehydroepiandrosterone sulphate (DHEAS) levels in X-linked adrenoleukodystrophy. Clin Endocrinol 49:691–693.CrossRefGoogle Scholar
  2. Depreter, M., Vandesompele, J., Espeel, M, Speleman, F., Roels, F., 2003, Real time RT- PCR analysis of the peroxisomal gene expression pattern after dehydroepiandrosterone and vitamin D treatment: therapeutic implications. J. Endocrinol 175: 779–792.CrossRefGoogle Scholar
  3. Waxman, D.J., 1996, Role of metabolism in the activation of dehydroepiandrosterone as a peroxisome proliferator. J Endocrinol 150: S129–S147.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2003

Authors and Affiliations

  • Johanna Assies
    • 1
  • Elisabeth B. Haverkort
    • 2
  • Ritsaert Lieverse
    • 3
  • Peter Vreken
    • 4
  1. 1.Departments of Psychiatry and Internal Medicine, Academic Medical CentreUniversity of AmsterdamAmsterdam
  2. 2.Department of DieteticsAcademic Medical CentreAmsterdam
  3. 3.Department of PsychiatryGGZ BuitenamstelAmsterdam
  4. 4.Laboratory of Genetic and Metabolic DiseasesAcademic Medical CentreAmsterdam

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