Metabolism of3H-dehydroepiandrosterone sulphate by subjects with steroid sulphatase deficiency

E. A. Bergner, L. J. Shapiro

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    17 Scopus citations


    Patients with steroid sulphatase deficiency develop ichthyosis with accumulation of cholesterol sulphate in plasma and in the stratum corneum. The present study was undertaken to determine whether desulphation of the C19 steroid DHEAS is also impaired. The mean plasma concentrations of DHEA and androstenedione were significantly lower for patients than for controls (p<0.02 and 0.001) while the mean concentration of DHEAS was higher (p<0.002). Following intravenous administration of3H-DHEAS, one patient failed to desulphate3H-DHEAS as evidenced by an absence of urinary3H-glucuronides. A second produced normal amounts of urinary3H-glucuronides (indicative of desulphation capacity) in a baseline study but did not desulphate3H-DHEAS following ampicillin treatment to alter gut microflora. A third patient had consistent sulphatase activity with and without ampicillin.

    Original languageEnglish
    Pages (from-to)403-415
    Number of pages13
    JournalJournal of Inherited Metabolic Disease
    Issue number4
    StatePublished - Dec 1988


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