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[Hypogonadotrophic hypogonadism and peripheral adrenal insufficiency in 2 brothers]
1Service de médecine infantile, CHRU de Grenoble, France.
Abstract:
Two siblings with chronic adrenal insufficiency, diagnosed at 12 years of age, failed to show a spontaneous onset of puberty in spite of adequate adrenocortical therapy. Testosterone plasma levels were low, without concomitant increase in gonadotropin levels. This association strongly suggest a sex-linked cytomegalic adrenocortical hypoplasia.