[Study of a new case of male pseudohermaphroditism due to 17-ketosteroid reductase deficiency (author's transl)].
Ann Endocrinol (Paris), 1979;40(6):549-50.
Berthezène F, Forest MG, de Peretti E, Chevallier M, Claustrat B, Dubernard JM
PMID: 121223
Impact factor: 3.117
Abstract
We studied a 17 year old patient with primary amenorrhea, hirsutixm, clitoral enlargment, poor breast development and 46, XY karyotype. The results shown in table clearly indicate a 17-ketosteroid reductase deficiency. In the view of previously described patients we can conclude that: 1) intensity of virilisation depends on both plasma testosterone and androstenedione levels; 2) importance of gynecomastia depends on plasma E2 but not E1 levels; 3) FSH levels are not correlated with circulating androgens or estrogens but presumably depends on importance of seminiferous tubules' lesions.
MeSH terms
17-Hydroxysteroid Dehydrogenases; Adolescent; Androstenedione; Disorders of Sex Development; Estradiol; Estrone; Follicle Stimulating Hormone; Gynecomastia; Humans; Luteinizing Hormone; Male; Testis; Testosterone
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