Résumé
Introduction: Inhibition of pituitary gonadotropin secretion in men by testosterone (T) is principally mediated by aromatization to estrogen (E), which inhibits hypothalamic secretion of gonadotropin-releasing hormone (GnRH). Material and Methods: Longitudinal clinical investigation unit-based evaluation of the clinical and biochemical response to E-receptor blockade. Initial monotherapy with 50 mg of clomiphene citrate (CC) daily for a period of 9 months, with diurnal morning peak testosterone and luteinizing hormone (LH) levels evaluated at three-month intervals thereafter. Th e patient then resumed hormone replacement therapy (HRT) using T cream with adjuvant CC therapy. Main Outcome Measures were Baseline and stimulated T and LH levels; eff ect on sexual function. Result(S): CC therapy resulted in complete normalization of pulsatile gonadotropin secretion, serum T level, and sexual function. Conclusion(S): Isolated hypogonadotropic hypogonadism (IHH) may result from an acquired defect of enhanced hypothalamic sensitivity to E-mediated negative feedback. Whereas direct T replacement therapy can further suppress endogenous gonadotropin secretion, treating IHH men with gonadotropins can stimulate endogenous T secretion and enhance fertility potential. Reversal of gonadotropin defi ciency with CC was found to have a similar biological eff ect.
Sujets)
Clomifène/administration et posologie , Clomifène/analogues et dérivés , Clomifène/usage thérapeutique , Gonadotrophines hypophysaires/déficit , Hormonothérapie substitutive/méthodes , Hormonothérapie substitutive/statistiques et données numériques , Humains , Hypogonadisme/traitement médicamenteux , Hypogonadisme/épidémiologie , Hypothalamus/physiologie , MâleRésumé
A 21 year old female patient with primary amenorrhoea was diagnosed to have isolated gonadotropin deficiency with probable functional hypothalamic amenorrhoea. The evaluation included buccal smear for sex chromatin, trial of medroxy-progesterone acetate, trial of oestrogen-progesterone preparation and estimation of serum prolactin, gonadotrophin and oestrogen levels. When diagnosed as isolated gonadotropin deficiency, treatment with gonadotropin is rewarding.
Sujets)
Adulte , Aménorrhée/traitement médicamenteux , Association de médicaments , Oestrogènes/administration et posologie , Femelle , Gonadotrophines hypophysaires/déficit , Humains , Hypothalamus/effets des médicaments et des substances chimiques , Progestérone/administration et posologieRésumé
Estudiamos dos familias donde varios de sus hijos son deficientes de hormona del crecimiento. En una de estas, dos varones son deficientes aislados de hormona del crecimiento, mientra que en la otra, hay dos hembras y un varón afectados de defiencias de hormona del crecimiento, de gonodotropinas y de hormonas y de hormonas tiroideas. No hay consanguinidad entre los padres de las familias. Se sugiere un patrón hereditario autosómico recesivo o recesivo ligado al sexo