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J Inherit Metab Dis. 1988;11(4):403-15.

Metabolism of 3H-dehydroepiandrosterone sulphate by subjects with steroid sulphatase deficiency.

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Howard Hughes Medical Institute, Department of Pediatrics, University of California, Los Angeles School of Medicine, Torrance 90502.


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 less than 0.02 and 0.001) while the mean concentration of DHEAS was higher (p less than 0.002). Following intravenous administration of 3H-DHEAS, one patient failed to desulphate 3H-DHEAS as evidenced by an absence of urinary 3H-glucuronides. A second produced normal amounts of urinary 3H-glucuronides (indicative of desulphation capacity) in a baseline study but did not desulphate 3H-DHEAS following ampicillin treatment to alter gut microflora. A third patient had consistent sulphatase activity with and without ampicillin.

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