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At least 19 recordsLinked to original sources
PubMedDate not supplied

[Congenital adrenogenital syndrome and virilizing adrenocortical tumor in prepuberal females. Problems of diagnosis and therapy in 2 cases of virilization].

After reviewing the causes of virilization in the newborn and grown female child, the Authors describe a case of congenital adrenal hyperplasia (S.A.G.C.) due to 21-hydroxylase deficiency in a newborn and a case of virilizing adrenal tumor in a four and half years old female child. The diagnostic and therapeutical approach of these unhealthy forms is argued and, on the ground of the personal observation and literature data, the frequent incidence of virilizing adrenocortical tumors within the same family is remarked and underlined.

Adenoma↗
PubMedDate not supplied

[Physiopathogenesis of idiopathic hairy virilism. II. Sources and mechanisms of the increase of production of androgens in idiopathic hairy virilism].

The rise in the secretion of androgens appears in many cases (50 per cent of cases) of primary idiopathic hairy virilism to be mixed or exclusively ovarian which in turn excludes the adrenal (less than 10 per cent of cases) in the production of androstenedione and testosterone. The part that the adrenals play in hyperandrogenization seems to be the result of enzymic partial blocks which are more or less compensated for. Thus about half of cases of idiopathic hairy virilism are of mixed origin, although the role of the ovary is by and large predominant for testosterone, whether the ovaries are or are not macroscopically polycystic. Both overproduction of androgens and/or overproduction of oestrogens by the ovaries can be responsible for overfunctioning of the adrenals in an androgenic manner because of enzymic dysfunction. In some cases the reverse can be demonstrated but then the functional pathology is more obscure.

Androgens↗
PubMed1977

[Physiopathogenesis of idiopathic hairy virilism. I. Peculiarities of the metabolism of androgens in idiopathic hairy virilism and general physiopathogenesis].

In most cases the persistent rise in the level of production of testosterone associated with a lowering of transport protein leads to the presence of free testosterone at a threshold level, which in turn brings into play irreversibly 5 alpha reductase that is potentially present in every woman in the areas where male characteristics are to be found. As a result the production is associated with a primitive drop in transport protein or with an excess of change of androstenedione into testosterone in the pilosebaceous follicle itself. This can be primary or secondary to a leveling out of the production of testosterone. This rise in production of testosterone comes about because of the summation of a rise of its glandular secretion and rise in both hepatic and peripheral conversion of androstenedione into testosterone, an absolute value (a rise in its production) and not a relative value (a diminution of the coefficient of transformation).

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗
PubMed1977

Virilizing activities of various steroids in female rat fetuses.

Virilizing activities of nineteen steroids were examined by a method measuring the abridgment of urovaginal septum length of female rat fetuses directly under microscope, following oral administration of the steroids to the mothers for 4 days during the late period of gestation. The characteristics of the virilizing activities of the steroids seemed to depend on their chemical structures. No virilizing activities were observed in progesterone, retroprogesterone, chlormadinone acetate and nandrolone phenpropionate. The introduction of 6-methyl and 17-acetoxy groups into progesterone exhibited a marked virilizing activity. With testosterone and its derivatives, the introduction of 17alpha-ethynyl group into testosterone slightly decreased the virilizing activity compared with the parent compound. On the contrary, the introduction of [3, 2-C] pyrazole ring in addition to the saturation of A-ring strikingly increased the virilizing activity. The most active virilizing steroid in this group was stanozolol followed by oxymetholone, methyl-testosterone and dimethysterone. Testosterone propionate was assumed to be less active than methyltestosterone by this route. With 19-nortestosterone derivatives, the virilizing activities of compounds with 17alpha-ethynyl group were moderate, but the presence of 17alpha-ethyl group caused a marked increase of virilizing activity. While, nandrolone phenpropionate with long side chain at C-17 exhibited no significant virilizing activity. The present results show the possibility of the evaluation on a potential virilizing activity of compounds in the female fetus, and also suggest that a dossociation between the virilizing activity and the androgenic activity may exist among some compounds.

Animals↗
PubMed1994

Ovarian hyperandrogynism as a result of congenital adrenal virilizing disorders: evidence for perinatal masculinization of neuroendocrine function in women.

Women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency often have a polycystic ovary-like syndrome, consisting of hyperandrogynism, infertility, menstrual irregularities, and elevated LH levels. This is generally considered secondary to poor control of the congenital adrenal hyperplasia. However, our experience led us to suspect that ovarian hyperandrogenism occurs even when congenital adrenal hyperplasia is well controlled on glucocorticoid therapy. Therefore, we tested the hypothesis that congenital adrenal virilizing disorders result in ovarian hyperandrogenism. We studied eight women with congenital adrenal virilizing disorders, seven with well controlled classic 21-hydroxylase deficiency and one with congenital virilizing adrenal carcinoma removed at 1.7 yr of age. We also studied six women with late-onset 21-hydroxylase deficiency, without signs of congenital virilization. An ovarian source of androgens was assessed after suppressing adrenal function with dexamethasone and then testing pituitary-ovarian function by a GnRH agonist (nafarelin) test. Five women with congenital adrenal virilizing disorders (four with classic 21-hydroxylase deficiency and one with congenital virilizing adrenal carcinoma) and one women with late-onset 21-hydroxylase deficiency had ovarian hyperandrogenism as determined by subnormal suppression of free testosterone after dexamethasone and/or by increased 17-hydroxyprogesterone response to nafarelin while on dexamethasone. All women with congenital adrenal virilization and ovarian hyperandrogenism had elevated LH levels after dexamethasone or elevated early LH response to nafarelin, which suggests that LH excess is the cause of their ovarian hyperandrogenism. This was not the case for the late-onset 21-hydroxylase-deficient woman. Our data are compatible with the hypothesis that congenital adrenal virilization programs the hypothalamic-pituitary axis for hypersecretion of LH at puberty. This is postulated to frequently cause ovarian hyperandrogenism even when adrenal androgen excess is subsequently controlled by glucocorticoid therapy.

Adolescent↗
PubMed1999

[A clinical study on virilization of external genitalia in congenital adrenal hyperplasia].

PURPOSE: In the newborn female with congenital adrenal hyperplasia (CAH), there are variable degrees of clitoral enlargement and virilization of the urethra. In order to investigate the factors that cause these variations, the degree of musculinization of the external genitalia in 44 patients with CAH was studied retrospectively. PATIENTS AND METHODS: This study includes a total of 44 patients with CAH aged 2 months to 12 years and 11 months old who had consulted Nagoya City University Hospital from 1977 to 1994. The length of clitoris on the first consultation was compared with the age and its change was followed. Also, the relationship among the length of clitoris on the first consultation, the degree of urethral virilization, the clinical forms i.e. salt-losing or simple-virilization, and the glucocorticoid hormonal condition were investigated with each other. In 5 pairs of sisters, the length of clitoris on the first consultation was compared within each pairs, and also the degree of urethral virilization was compared. RESULTS: The clitoral length on the first consultation was significantly correlated with the age (y = 1.8 x + 1.8, r = 0.7, p < 0.0001). The mean clitoral length at birth calculated from these data was 18 mm, which is as large as that of adult female one, and the clitoral enlargement rate was 1.8 mm/yr. The correlation was not clear between the clitoral length and the degree of urethral virilization, the clinical forms, and the glucocorticoid hormonal condition, either. On the other hand, the degree of urethral virilization was comparable within clitoral length was not comparable within them. CONCLUSIONS: The urethral virilization was suggested to be determined by the internal hormonal environment during embryonal stage which was shared by the each pair of sisters. On the Other hand, the clitoral length was depend on the patient's age though the factor which determined the length was not apparent. These facts indicated that, not only the severely musculinizing cases, but also the new born female with mildly virilizing urethra should be followed strictly not to undergo the clitoral enlargement.

Adrenal Hyperplasia, Congenital↗
PubMed2004

Prenatal diagnosis of P450 oxidoreductase deficiency (ORD): a disorder causing low pregnancy estriol, maternal and fetal virilization, and the Antley-Bixler syndrome phenotype.

We report studies on the second pregnancy of a woman who had previously given birth to a virilized female infant. The cause of the virilization had not been established, but common forms of congenital adrenal hyperplasia (CAH) were excluded. Longitudinal monitoring of the second pregnancy revealed that estriol excretion failed to increase normally, reaching a maximum 0.7 mg/24 hr at the end of pregnancy (normal mean 30 mg/24 hr). The mother showed signs of virilization by the 23rd week of gestation and aromatase deficiency was suspected. However, predicted urinary metabolites for diagnosis of aromatase deficiency (for example, 16alpha-hydroxyandrosterone) were not increased significantly during the pregnancy. Interestingly, excretion of the androgen metabolite androsterone increased rapidly at the beginning of pregnancy and peaked around the 20th week, suggesting increased production of testosterone and 5alphaDHT, probably the cause of maternal virilization. Urine steroid analysis by GC/MS showed gradually increasing excretion (9 mg/24 hr) of the normally minor metabolite 5alpha-pregnane-3beta,20alpha-diol (epiallopregnanediol), an epimer of the dominant progesterone metabolite pregnanediol (5beta-pregnane-3alpha,20alpha-diol). We believe epiallopregnanediol is largely the maternal urinary excretion product of fetal 5-pregnene-3beta,20alpha-diol, the principal metabolite of pregnenolone, implying a build-up of the latter steroid in the fetal adrenal. These findings suggested that the 'block' in the estriol biosynthetic pathway occurs at an early stage with 17-hydroxylation of pregnenolone being affected. The male baby born of this pregnancy had normal genitalia but showed a urinary steroid profile indicating partial deficiencies of P450c17 and P450c21. However, no mutations in the corresponding CYP17 and CYP21 genes were identified. Urinary steroid analysis carried out on his virilized older sibling showed the same pattern of metabolites. Recently, we determined that this disorder is caused by mutations in P450 oxidoreductase (OR), the essential redox partner for CYP17 and CYP21 hydroxylases. The novel metabolic profile has now been seen in many patients, most diagnosed with the skeletal dysplasia Antley-Bixler syndrome. We propose that excessive excretion of epiallopregnanediol together with low estriol may be prenatally diagnostic for OR deficiency (ORD).

Adult↗
PubMed1995

Virilization associated with choriocarcinoma.

We demonstrate here two autopsy-proven cases of virilization associated with choriocarcinoma. The first patient was a 27-year-old woman with a 7-year history of metastatic choriocarcinoma who presented with left hemiparesis and virilization. Serum testosterone concentration was 18 nmol/l, free testosterone 471 pmol/l, dehydroepiandrosterone sulphate (DHEA-sulphate) 1.7 mumol/l, sex hormone binding globulin 12.3 nmol/l, estradiol (E2) 1,843 pmol/l, and hCG 1.6 x 10(5) IU/l. The second patient was a 21-year-old virilized woman with metastatic choriocarcinoma who presented in semicomatose state. Limited endocrine investigation revealed serum testosterone 27 nmol/l and hCG 2.7 x 10(5) IU/l. Both patients died despite chemotherapy and radiation therapy. Autopsy findings revealed choriocarcinoma with brain and multiple organ metastasis in both. Pathology of the ovaries of both patients demonstrated hyperplasia of luteinized theca cells and lutein cells. We postulate that an association of virilization and choriocarcinoma resulted from long-standing stimulation of ovary by hCG causing theca cell hyperplasia with subsequent hypertestosteronemia and virilization.

Adult↗
PubMed2003

[Virilism during pregnancy].

We discuss the causes of virilism in pregnancy and the impact of hyperandrogeny on the female foetus. We report a case of virilism in a 28-year-old, gravida 1, para 1 patient with normal pregnancy and review the literature. After conception, the patient had been well until the 18th week of gestation, when she developed signs of virilization; her serum testosterone was markedly elevated. She delivered of a normal male infant at term. After delivery, signs of virilization regressed with normalization of testosterone level but a wide mass of the left ovary that persisted for 8 months. The histological study of the tumor showed luteinized thecoma. Luteomas and hyper-reactio luteinalis were the principal causes of virilism in pregnancy, thecomas are rare.

Adult↗
PubMed2003

Mucinous cystadenoma of the ovary with functioning stroma and virilization in pregnancy: a case report and review of the literature.

Virilization caused by ovarian tumors with functioning stroma during pregnancy is extremely rare and has been reported in many ovarian tumors. In mucinous cystadenomas with maternal virilization during pregnancy the stromal cells responsible for the hormone secretion resemble lutein or Leydig cells and have been referred to as luteinized stromal cells. We present a case of a 30-year-old, gravida 2, para 1, woman who presented at approximately the 38th week of pregnancy with features of virilization. At the same time, a cesarean section was performed because of fetal distress and a male weighing 3,030 g without any gross abnormalities was delivered. A large tumor of the right ovary was detected and a right salpingo-oophorectomy was performed. Histopathologically, the tumor proved to be a benign mucinous cystadenoma. Masses typically resembling lutein stromal cells or Leydig cells of the testes or ovarian hilus were found in the wall of the cyst below the mucinous epithelium. No crystalloids of Reinke were identified. The stromal component of the tumor was characterized as functioning stroma with luteinized stromal cells. The glandular mucinous epithelium showed focal positivity for human chorionic gonadotrophin. The cytoplasm of the luteinized stromal cells reacted strongly and diffusely with antiserum for vimentin. Also, the cytoplasm of the luteinized stromal cells showed focal intense positivity for synaptophysin, and focal mild positivity for human chorionic gonadotrophin. Staining results for oestrogen and progesterone receptors were negative. In conclusion, we present an unusual case of clinical virilization during pregnancy associated with an ovarian mucinous cystadenoma with functioning stroma. The virilizing manifestations disappeared after removal of the ovarian neoplasm, supporting the perception that the functioning ovarian stroma was responsible for the androgen production.

Adult↗