Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042755 (masculinization)
 2,562 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Virilization may occur during pregnancy as the result of an ovarian Krukenberg tumor. mechanism of the androgen overproduction in this exceptional condition is still poorly understood. A new case is reported in which only in the postpartum clinical, endocrine, and endoscopic studies led to the diagnosis of an ovarian Krukenberg tumor secondary to a gastric carcinoma. In the mother, basal hormonal studies were done 1 and 4 weeks after delivery, then after gastric and ovarian surgery. Three months after delivery, ovarian steroid response to hCG (priming dose, 5000 IU; then 1500 IU every other day for 12 days) and a study of progesterone (P) metabolism at a steady state after a constant infusion of [3H]P and cold P (92 micrograms/min leads to blood production rate (BPR) of 152 mg/day designed to reproduce the BPR of P usually seen in pregnancy) were successively performed. Hormones were measured by specific RIAs after chromatographical purification. Basal hormonal levels were normal in the child. In the mother, on the 5th day postpartum, mean hormone levels (in nanograms per dl) were: testosterone (T), 4181; androstenedione (delta 4), 8876; 17 alpha-hydroxyprogesterone (17-OHP), 9746; P 1075; estrone (E1), 195; and estradiol (E2), 151. One month later, levels were normal for the follicular phase; T, 40; delta 4, 146; P, 52; E2, 9; and E2, 4.5. At both times, dehydroepiandrosterone was normal (703-750). Hormone levels increased progressively during hCG stimulation but their time course was different between hormones. At the end of the test, T. 144; delta 4, 746;' 17-OHP, 789; P, 723; E1, 37; and E2, 20. The MCR of P was decreased, 1450 liters/day (normal, 2020). Conversion ratios between products and precursor during constant infusion were normal. From these data, obtained in four different conditions (postpartum period, hCG stimulation, progesterone infusion, and after oophorectomy), the following can be concluded: adrenal production of dehydroepiandrosterone was normal; the ovarian overproduction of androgens likely resulted from the excessive reductive metabolism of both placental and ovarian P along the delta 4 steroid biosynthetic pathway by an hypertrophic stromal compartment; and HCG stimulation seems to be the necessary stimulus for this condition. The enhancement by T on its own peripheral production is also discussed.
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PMID:Approach to the mechanism of androgen overproduction in a case of Krukenbery tumor responsible for virilization during pregnancy. 26 6

Five patients were found to have hyperthecosis ovarii as evidenced by the presence of large lipid containing thecal cells in the ovarian stroma. The clinical picture was similar in all of them, featuring mild virilization, obesity and oligomenorrhea, with refractoriness to clomid therapy. Plasma FSH levels were low normal, while LH levels were slightly elevated. Urinary 17-ketosteroids levels were elevated, and plasma testosterone concentrations were upper normal. The response to dexamethasone suppression and HCG stimulation is discussed. The effect of wedge resection on the clinical and endocrinologic pictures is evaluated.
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PMID:Hyperthecosis syndrome. Clinical, endocrinologic and histologic findings. 41 61

Comparative results of the suppression-stimulation test by dexamethasone and chorionic gonadotropin, chromatographic separation of 17-ketosteroids, and plasma testosterone levels in the ovarian and adrenal veins, in cases of virilism in women. Thirteen patients with hirsutism and virilization were investigated as follows: 1. measurement of plasma testosterone (T) levels by radioimmunoassay (RIA) during suppression-stimulation tests by the administration of Dexamethasone (DXM) and chorionic Gonadotropin (HCG). 2. chromatographic determination of urinary 17-ketosteroids, pregnanediol (P2), and pregnanetriol (P3). An attempt was made to classify virilism as "ovarian" or "adrenal" based on the results of 1. and 2. 3. bilateral ovarian and adrenal venous catheterization through the femoral vein to measure T (RIA) levels. 4. laparotomy with bilateral wedge resections of the ovaries for therapeutic and biopsy purposes. Surgical catheterization of the ovarian veins was carried out during the operation. The results of these tests show that: a) the dynamic DXM-HCG test can be used to separate those cases in which the ovary is not involved in T formation from those in which, apparently, it is involved. b) chromatographic determination of urinary steroids has no aetiological value, as the variations in the different fractions are not significant. c) in all patients, the principal source of T is the adrenals and not the ovaries, even when there is an increase in T in the ovarian efferent blood vessels.
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PMID:[Biological parameters of virilism in women]. 74 80

In male patients with hypogonadotropic hypogonadism development of the testicles may be brought about by means of pulsatile administration of LHRH. The results in 29 treated patients are described. The treatment lasted 53 weeks on average and in all patients led to a rise of the plasma levels of LH, FSH and testosterone, and to an increased testicular volume. Spermatogenesis started in 22 patients. The LHRH treatment induces the secretion of gonadotrophins and consequently, testicular steroidogenesis. This causes virilization to begin. Furthermore, under the influence of the FSH in particular, the tubules and spermatogenesis develop. Fertility may then be achieved. It is concluded that pulsatile LHRH treatment is a suitable method to induce development of the testicles, including spermatogenesis. Once spermatogenesis is initiated, HCG treatment can maintain and even improve this development.
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PMID:[Induction of spermatogenesis in hypogonadotropic hypogonadism using pulsatile administration of LHRH]. 180 90

A case of male hermaphroditism is presented. It complied with all traditional criteria of male hermaphroditism due to Leydig's cells hypoplasia (anomaly of the internal genitalia, karyotype 46, XY, presence of the testicular tissue, no response of the hypoplastic Leydig's cells to HCG stimulation with simultaneous dramatic decrease in their number in the interstitial tissue. Emphasis is on the marked difference in incomplete virilization of the external genitalia related to the occurrence of Leydig's cells insufficiency.
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PMID:[Hypoplasia of Leydig's cells as a cause of male pseudohermaphroditism]. 281 79

Ten cases of boys without testis are presented. Clinical and anatomical patterns of the disease are analysed. Total absence of testicular tissue is proven by the lack of plasma testosterone response to HCG stimulation and by cautious surgical investigation. Artificial virilization by prolonged androgen administration associated with silastic prothesis implantation is the only possibility of treatment. Anorchism does not represent a form of gonadal dysgenesis or sexual ambiguity since external genitalia are always non ambiguous, chromosomal constitution is XY without any structural anomaly of Y and H-Y histocompatibility antigen is present. Absence or destruction of the testis are caused by different mechanisms, according to the data obtained by individual case studies. The most likely is that foetal testes developped normally are destroyed in utero or in the perinatal period or even soon after birth. The most frequent mechanism with a genetic predisposition, seems to be bilateral pedicle twisting.
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PMID:[Total testicular regression syndrome or anorchidism]. 614 46

A case of ovarian androblastoma (arrhenoblastoma) of the intermediate differentiation type in a 5-year old girl is reported. The clinical features were precocious isosexual puberty with secondary masculinization. The tumour was removed but relapsed 26 months later, requiring ovariectomy and salpingectomy. The patient, who has now been followed up for 7 years and 6 months, is definitely cured. Biochemically, the androblastoma was accompanied by high plasma levels of oestrogens, androgens and, quite exceptionally, human chorionic ganodotropin, alpha HCG and beta HCG. Light and electron microscope examination failed to differentiale Sertoli cells from granulosa cells and Leyding cells from luteinized ovarian stroma cells. Areas resembling syncytiotrophoblastic cells were very limited and could not be seen under the electron microscope. Some cells looked like undifferentiated germ cells. The HCG secreted was biologically active on rabbit ovaries (Reiprich's reaction) but inactive on the girl's contralateral ovary, which was found on two biopsies to be unstimulated and prepubertal.
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PMID:[Precocious pseudo-puberty due to HCG-secreting androblastoma of the ovary. One case (author's transl)]. 625 78

Morphological and endocrinological studies were performed on a 19-year-old case of an arrhenoblastoma with marked virilization. The tumor was an intermediate type of Meyer's classification. Histochemically, 3 beta-HSD and G-6-PDH activities were demonstrated in Leydig cells. These cells also had ultrastructures typical of steroid-producing cells. Basal blood cells of pregnenolone, dehydroepiandrosterone, testosterone (T), androstenedione (A), progesterone, estradiol, estrone, LH, and FSH were determined pre- and postoperatively. T and A showed a very high level preoperatively, and were markedly decreased immediately after removal of the tumor. Stimulation of tumor cells by HMG-HCG did not show any significant changes in their main products of T and A. These findings suggest that Leydig cells of the present tumor produced mainly A and T independently on gonadotropins, and these hormones had virilized the patient.
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PMID:[Morphological and endocrinological study of ovarian arrhenoblastoma (author's transl)]. 627 28

Studies within the Arab population in Israel revealed 25 pseudohermaphrodites due to 17 beta-hydroxysteroid dehydrogenase (17 beta-HSD) deficiency. Twenty-three individuals, presently living in the Gaza strip, belong to a very large inbred kinship which extends over 8 generations. All affected subjects (46, XY) were born with mild to moderate degrees of ambiguity of an apparently normal-looking female genitalia and therefore were reared as girls. In childhood, genital abnormalities consisted of a clitoral-like phallus surrounded by a chordee, non-fused labial-scrotal folds and a urogenital sinus. The testes were in the inguinal canals, or rarely, in the labial-scrotal folds. Wolffian structures were normally differentiated while Mullerian structures were absent. At puberty, subjects developed a male body habitus with abundant body hair and beard. Gynecomastia was absent. The phallus and testes enlarged to adult proportions while the prostate remained small. Together with the physical change from girls to boys they developed a male identity having erections and ejaculations, which in 7 cases led to the spontaneous adoption of a male gender role. In adults the hormonal abnormalities consisted of greatly elevated delta 4-androstenedione (delta 4) (350-1267 ng/dl) associated with subnormal testosterone (T) levels (0.9-3.1 ng/ml). Dihydrotestosterone (DHT) levels, with the exception of 1 patient, were relatively low in all cases (27-35 ng/dl). Children had low levels of delta 4, T and DHT, which were normal for age. Although from puberty on there was a significant rise of the 3 androgens, delta 4 always remained extremely elevated and T and DHT relatively low when compared to normal controls. Dexamethasone failed to suppress the androgen pattern while HCG augmented the defect, making the diagnosis possible in 2 prepubertal children. Dehydroepiandrosterone (DHEA) and 17-hydroxyprogesterone (17-OHP) levels were normal or moderately elevated. Estradiol (E2) levels were normal in children and all but 2 adults, who had high levels. LH and FSH levels were very high after puberty, but normal before. However, there was an overresponse to LHRH in all age groups. The contrast between the lack of intrauterine virilization of the external genitalia in fetuses with 17 beta-HSD deficiency versus the marked masculinization that occurs after puberty still remains a puzzling phenomenon. It is conceivable that the postpubertal development of a male phenotype with change of gender identity and role occurs due to the joint effect of delta 4, T and DHT, even though secreted in inadequate proportions. Thus masculinization in these individuals is a slow process requiring a longer period of time than that of normal puberty to be completed.
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PMID:Male pseudohermaphroditism due to 17 beta-hydroxysteroid dehydrogenase deficiency: studies on the natural history of the defect and effect of androgens on gender role. 631 Feb 48

Virilism in females is often associated with menstrual disorders. There is an evident correlation between the severity of virilism, the level of androgens and the degree of menstrual disorder. A study was carried out between January 1978 and December 1980 on 75 women with various degrees of virilism. The majority of women with severe hirsutism and male-type alopecia were found in the group with amenorrhoea. Furthermore, the four cases with the full-blown picture of severe virilism were found exclusively in this group. Clarification of the virilizing manifestations on the basis of a determination of the basic hormonal status (including the steroid fractions), the dexamethasone suppression test (including the HCG stimulation test) and the oestrogen-gestagen suppression test, diagnostic laparoscopy and phlebography of the adrenal gland is discussed and the methods evaluated.
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PMID:[Androgenetic amenorrhea]. 682 May 86


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