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ABNORMAL ANDROGEN AND OESTROGEN METABOLISM IN MEN WITH STEROID SULPHATASE DEFICIENCY AND RECESSIVE X‐LINKED ICHTHYOSIS
Author(s) -
LYKKESFELDT G.,
BENNETT P.,
LYKKESFELDT A. E.,
MICIC S.,
MØLLER S.,
SVENSTRUP B.
Publication year - 1985
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/j.1365-2265.1985.tb01096.x
Subject(s) - endocrinology , medicine , androstenedione , steroid sulfatase , dihydrotestosterone , dehydroepiandrosterone , testosterone (patch) , androgen , sex hormone binding globulin , androstenediol , androgen deficiency , ichthyosis , chemistry , steroid , hormone , biology , genetics
SUMMARY Serum levels of sex hormone binding globulin (SHBG), testosterone, free testosterone, dihydrotestosterone, androstenedione, dehydroepiandrosterone sulphate, oestradiol, oestrone, oestrone sulphate, FSH, and LH were measured in 20 steroid sulphatase‐deficient men with recessive X‐linked ichthyosis and in normal men. The serum oestrone sulphate level was significantly higher than normal in the patients ( P < 0·0001). In affected men, there was a tendency towards higher dehydroepiandrosterone sulphate levels and no decline with age was seen in the patients as opposed to normal men (interaction: P < 0·025). Serum androstenedione, and oestradiol levels were lower than normal in the patients ( P < 0·0005 and P = 0·055, respectively), while their LH level was higher than normal ( P < 0·0005). The serum levels of SHBG, total and free testoster‐one, dihydrotestosterone, oestrone, and FSH were not significantly different from normal in the icthyotic patients. We suggest that the observed abnormalities in these patients are a consequence of the enzyme deficiency which severely impairs the ability of tissues to hydrolyse steroid sulphates.

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