[The autonomous secreting thyroid nodule at the non-inhibitory isofixing stage. Initial stage of Plummer's toxic adenoma. 3 anatomo-clinical cases].
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Biomedical subjects
Publications and source records attributed to J Vague.
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Aromatization of androgens into estrogens is an early phenomenon during phylogenesis and ontogenesis which occurs in most tissues. The mechanism which is responsible for this activity and for it's competition with 5 alpha reduction includes hypothalamo-pituitary receptors, genetic induction of sexual differentiation, binding proteins, and sulfo and glyco-conjugation. Aromatization is determinant before, during, and after ovulation, as well as in developing pregnancy. In the testis, in spite of it's importance, it's role is unknown. It's action is essential in the sexual differentiation of the brain. Aromatization in the muscle and the adipose tissue is not negligible under normal and pathological conditions.
Male pseudo-hermaphroditism by partial testosterone sensitivity, without steroido-genesis abnormality, without apparent 5 alpha reductase deficit - Empty complete scrotum and hypospadias surgically normalized. At 39, scrotal testis 32 X 15 mm right, 23 X 10 mm left. Infantile penis and prostata. Gynoid scapulo-pelvic proportions, adipo-muscular ratio and fat distribution. Bilateral gynecomastia. Caryotype 46 XY - 17 ks 26 mg. After intravenous 100 ng LH RH: plasma (pl) LH increased 105 to 285 ng/ml, pl testosterone 5 ng/ml unchanged, pl oestradiol 115 to 135 pg/ml. 48 h after intra muscular 300 mg testosterone propionate: pl testosterone increased, 13 to 39 ng/ml, androstenedione 1,5 to 3,04 ng/ml, dihydrotestosterone 0,85 to 1,45 ng/ml, OEstradiol 52 to 110 pg/ml. No effect of 29 g testosterone oenanthate in 2 years.
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85 cases of this syndrom have been published since the original case in 1964. The main clinical symptoms are those of Klinefelter's disease from which it differs by the caryotype only. Azoospermie is the rule. 3 mechanisms can be implicated: Mosaic 46 XX/47 XY with secondary loss of Y chromosome; Translocation of a part of Y chromosome upon an autosome or Y chromosome; Autosomal mutant gene acting upon the masculinizing site of X chromosome and inducing the primitive development of the testis as the short arm and proximal part of the long arm of Y chromosome normally do. These mechanisms perhaps are distinct origins of a syndrom which clinic alone cannot dissociate.