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PubMed1980

["Hormone receptor diseases" in Japan: A nation-wide survey for testicular feminization syndrome, pseudohypoparathyroidism, nephrogenic diabetes insipidus, Bartter's syndrome and congenital adrenocortical unresponsiveness to ACTH (author's transl)].

A nation-wide survey for five "hormone receptor diseases" was carried out. For the first survey, an inquiry was sent to all hospitals in Japan having more than 200 beds, in order to determine how many patients there were between 1968-1977. A further detailed survey was carried out on patients who were reported in the first survey. The approximate numbers of patients in Japan estimated from these surveys are the following: testicular feminization syndrome (TFS), 390; pseudohypoparathyroidism (PHP), 220, nephrogenic diabetes insipidus (NDI), 280; Bartter's syndrome, 90; congenital adrenocortical unresponsiveness to ACTH (CAUA), 18. In 73 cases of TFS, partial virilization was observed in 23% (the incomplete form). Testes were found in all cases and the epididymis in 84%, whereas none of the patients had seminal vesicles. PHP consisted of 38 Type-I cases, 6 Type-II cases and 25 unclassified cases. There were 27 males and 42 females. Skeletal anomalies were found in two-thirds of the patients. Grades of hypocalcemia and soft tissue calcification were more prominent in Type I. After treatment, none of the Type-I patients showed normal urinary cyclic AMP response to parathormone, although urinary phosphate response was normalized in one and markedly improved in 4. In 78 patients with NDI, there were 67 males and 11 females. The age of the onset of NDI ranged from 0 to over 50, but 22 out of 29 cases of hereditary NDI had the onset at age 0. There seemed to be at least two subtypes; one beginning in the neonatal period or early childhood, and the other having the onset in late childhood or adult. The important initial symptoms were fever and anorexia in the early onset type. Growth retardation was remarkable in early childhood. Diuretics were effective in most of the cases. There were 22 male and 12 female patients with Bartter's syndrome. Indomethacin was effective in 9 out of 10 patients studied.

Adolescent↗
PubMed2001

Feminizing genitoplasty in patients with 46XX congenital adrenal hyperplasia.

Congenital adrenal hyperplasia (CAH) is the most common cause of ambiguous genitalia in newborns. This paper is based upon review of the literature and personal experience. We focus upon the surgical anatomy, pre-operative evaluation, including imaging, mainly by transabdominal ultrasound, and upon the goals and the history of surgical reconstruction. The various surgical techniques are mentioned with a detailed description of our technique used in 52 patients. The timing and staging of the operation and the implications of prenatal therapy are discussed. In conclusion, we believe that infants with 46XX CAH can undergo one-stage feminizing genitoplasty very early in life with satisfactory cosmetic and functional results.

Adrenal Hyperplasia, Congenital↗
PubMed1978

Absence of positive feedback effect of oestrogen on LH release in patients with testicular feminization syndrome.

The response of serum LH to exogenous oestrogen administration was studied in 5 patients with testicular feminization syndrome (TFS). The serum LH levels were elevated in all the patients, while serum testosterone levels were within the normal male range. Serum FSH levels were elevated in 4 patients and normal in one patient. Intravenous administration of 100 microgram of LH-RH provoked a further increase in both LH and FSH. Following intravenous injection of 20 mg of conjugated oestrogen (Premarin), the LH levels were serially determined until 120 h in TFS patients, 5 normal males, and 10 normal females during the mid-follocular phase (D7-9). Both TFS patients and normal males showed no LH release following oestrogen injection in contrast to normal females who displayed a significant increase in LH with a peak at 48 to 56 h after the injection. These results seem to suggest that the insensitivity of the hypothalamus to androgen in TFS patients do not affect the sex differentiation of the hypothalamus. The possible role of oestradiol conversion from testosterone in the hypothalamus is discussed.

Adolescent↗
PubMed1980

The role of oestrogens on gonadotrophin secretion in the testicular feminization syndrome.

The role of oestrogens on gonadotrophin secretion was assessed in three related patients with the complete form of testicular feminization syndrome. Serum LH and FSH levels were measured before and after LRH stimulation as well as before, during and after chronic clomiphene citrate administration. Moderately elevated LH basal levels with a significant LH rise following LRH were observed. Normal or even low FSH level with poor response to LRH were found in all subjects. Administration of clomiphene citrate resulted in a significant serum LH increase without any change of FSH. Following castration both LH and FSH rose and a normal response to LRH was observed. These results were interpreted as demonstrating that, while endogenous oestrogens modulate LH secretion in patients with androgen unresponsiveness, it plays no role in regulating FSH secretion and suggested that a factor of testicular origin without androgenic or oestrogenic activity is responsible for FSH regulation.

Adolescent↗
PubMed1980

A role of guanosine 3',5'-monophosphate in human-prolactin-induced estrogen production by feminizing adrenal neoplastic cells.

Using feminizing adrenal neoplastic gland (FANG) cells we showed that hPRL stimulated cGMP biosynthesis. Also we could clearly demonstrate a stimulatory effect of db-cGMP plus theophylline on estrogen production without concomitant stimulation of cell growth. These findings indicate that a stimulatory effect of hPRL on estrogen biosynthesis by Fang-8 cells may act via increased cGMP concomitant with depressed cAMP.

Adrenal Gland Neoplasms↗
PubMed1979

Induction of androgen-dependent protease and serous-like granules by tri-iodothyronine in the submandibular gland of mice with testicular feminization.

Esteroprotease, an androgen-dependent enzyme of the mouse submandibular gland, was increased by injection of tri-iodothyronine (T3) in mice with testicular feminization (Tfm) which are genetically deficient in androgen receptors. Histochemical and electron microscopic studies also demonstrated increases of RNA and serous-like granules in cells of the convoluted tubules of the gland. These findings suggest that the esteroprotease gene in Tfm mice is normal and that T3 can induced both esteroprotease and serous-like granules independently of androgen.

Androgen-Insensitivity Syndrome↗
PubMed1989

A dual role of growth hormone as a feminizing and masculinizing factor in the control of sex-specific cytochrome P-450 isozymes in rat liver.

The effects of GH on the major constitutive sex-specific forms of cytochrome P-450 (P-45015 beta and P-45016 alpha) were studied in hypophysectomized rats at the mRNA level. Time-course experiments were performed with or without simultaneous treatment with thyroxine and cortisol. Intermittent administration of GH, mimicking the male secretory pattern, caused complete masculinization of the male specific P-45016 alpha at a pretranslational level in the absence and presence of thyroxine and cortisol. When GH was administered continuously, mimicking the female secretory pattern, the female specific P-45015 beta was induced, an effect that was dramatically potentiated by simultaneous treatment with thyroxine and cortisol. A synergistic effect of thyroxine and cortisol at a pretranslational level was demonstrated, although the major potentiating effect could be attributed to thyroxine. Thus it was concluded that GH, depending on its secretory pattern is the sole masculinizing factor for cytochrome P-450, and that it is also a feminizing factor, although this activity requires the synergistic action of thyroid hormones and glucocorticoids to reach its full effect.

Animals↗
PubMed1991

Testicular steroidogenesis in the testicular feminized (Tfm) mouse: loss of 17 alpha-hydroxylase activity.

Testicular feminized (Tfm) mice are totally insensitive to androgen and may be used to study the role of the androgen receptor in normal development and function. We have examined testicular and Leydig cell steroidogenesis in Tfm mice. Serum bioactive LH was high in Tfm mice but serum testosterone was low and this was associated with a severe reduction in testicular testosterone production in vitro. Examination of [3H]pregnenolone metabolism by testes of Tfm mice indicated that progesterone, rather than testosterone, was the major steroid produced. Leydig cells were isolated from normal and Tfm mice and from normal mice in which testicular descent was surgically prevented before puberty. As in whole testes, androgen production in response to human chorionic gonadotrophin was severely reduced in Leydig cells from testes of Tfm mice compared with normal or cryptorchid groups. In contrast, progesterone production by Leydig cells from testes of Tfm mice was markedly increased in comparison with other groups. Total steroid production (progesterone plus androstenedione plus testosterone), however, was only 24% of normal in Leydig cells from Tfm mice. The pattern of steroid production by Leydig cells from cryptorchid testes was similar to control, although total steroid production was reduced to about 50% (this was significantly higher than the Tfm group, P less than 0.05). The high progesterone/androgen ratio in testes from Tfm mice suggested that 17 alpha-hydroxylase was depleted in these animals. To confirm this, activity of the four major steroidogenic enzymes associated with the smooth endoplasmic reticulum was measured.(ABSTRACT TRUNCATED AT 250 WORDS)

17-Hydroxysteroid Dehydrogenases↗
PubMed1993

Suppressed expression of the cytochrome P45017 alpha protein in the testicular feminized (Tfm) mouse testes.

The testes of testicular feminized (Tfm) mice synthesize and secrete abnormally low amounts of testosterone, as a consequence of selectively decreased cytochrome P450(17 alpha) activity. To investigate the mechanism of this deficiency, three steroidogenic enzymes were immunolabeled in the testes of normal and Tfm adult (2.5-6 month old) mice. Cholesterol side-chain cleavage cytochrome P450 (P450scc) and delta 5-3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) were detected in the Leydig cells of both normal and Tfm mice whereas, in contrast to normal mice, only a small proportion of Leydig cells were immunostained for cytochrome P450-17 alpha-hydroxylase, C17-->20 lyase (P450(17 alpha)) in the testes of Tfm mice. The numbers of cells differed from male to male and interestingly were markedly higher in the right testis. Explants of testes from Tfm mice were kept in organ culture at 32 degrees C for 45 h, with or without dibutyryl cyclic AMP (100 or 500 mumol/l). All Leydig cells remained positive for P450scc and 3 beta-HSD, and P450(17 alpha) became detectable in the majority of Leydig cells in both left and right testes, showing that the lack of expression of P450(17 alpha) protein in Tfm mouse testes in vivo is not structural but is a regulatory phenomenon.

Androgen-Insensitivity Syndrome↗
PubMed1989

The feminization of poverty: myth or reality?

The feminization of poverty is a widely discussed concept in the mass media, and in academic circles, which captures the following facts: there are more poor women than poor men, and women are more likely to fall into poverty because of gender-related factors. In this article, I examine the concept's empirical basis and theoretical significance. The data show that poverty among men increased faster than poverty among women during the 1980-81 recession. While the sex composition of the poverty population has remained relatively unchanged since 1966, its age composition has changed: poverty has increased substantially among working men and women aged 18 to 44. Also, the higher proportion of women in the poverty population cannot be considered simply an effect of male privilege; on the contrary, it may be partially accounted for by higher mortality rates among working-class men. Theoretically, I argue that the meaning of these trends cannot be established using only age and sex categories of analysis. The trends document the progressive immiseration of the working class. Younger workers of both sexes today are worse off than older workers. A discussion of poverty purely in terms of the age, sex, or racial/ethnic composition of the poverty population hides the roots of poverty in the mode of production and succeeds in obfuscating the issues, fueling conflict between men and women, young and old, and white and nonwhite. Only by taking into account the class relations that structure people's life chances is it possible to understand the significance of present trends.

Adolescent↗
PubMed1977

Dysgerminomas (seminomas) in genetic males with female phenotype. One case of gonadal dysgenesis and gonadoblastoma and one of testicular feminization.

Gonadal tumors in patients with somatosexual ambiguity are rare but of considerable clinical and pathological interest. Two patients with dysgerminomas and abnormal sexual differentiation were recently treated at the Department of Gynecologic Oncology. One (Case 1) was an 18-year old girl and the other (Case 2) an unmarried woman of 58 years. In spite of their female phenotype both showed a male genotype, 46, XY. In Case 1 the dysgerminoma (or rather seminoma) originated in a gonadoblastoma in one of two dysgenetic testes but in Case 2 in one of two "feminizing testes". The clinico-pathological features of both cases will be reported and commented and the relation between the germ cell tumors, gonadal dysgenesis and the Y-chromosome will be discussed.

Adolescent↗
PubMed1992

Testicular feminization syndrome and associated gonadal tumors in Denmark.

We studied the incidence of testicular feminization syndrome in Denmark over a 7-year period and found it to be about 1:20,400. Twenty-one patients are described in greater detail. Four patients had gonadal tumors, none of these being malignant. Ten patients (47.6%) had inguinal hernias in early childhood. All patients but one were gonadectomized. Eleven patients (52.4%) disclosed signs of partial androgen function. Only 5 of them had their gonads removed immediately.

Adolescent↗
PubMed1996

Cytochrome c oxidase in rat adrenal and liver: effects of anti-androgen treatment and studies in testicular feminized rats.

The role of androgen receptors in androgen-induced changes in rat adrenocortical and liver cytochrome c oxidase (COX) has been investigated. The anti-androgen flutamide, blunted the increase in COX activity and COX subunits II/III and IV, that is seen with androgen treatment. Testicular feminized (Tfm) rats had levels of COX activity and COX subunits II/II and IV in adrenal cortex and liver that were intermediate between the high levels found in normal male rats and the lower levels of normal female rats. These data suggest that androgen effects on adrenal and liver COX are mediated through interactions with androgen receptors known to be present in these issues. However, the observed changes in COX activity and COX subunits were not accompanied by altered levels of mRNAs encoding for COX II or COX IV.

Adrenal Cortex↗
PubMed1994

Complete testicular feminization with normal gonadotropin and high androgen secretion: a case report.

A patient with a complete form of testicular feminization with normal gonadotropin and high testosterone levels is described here. These findings are in contrast to previous reports that have shown high circulating luteinizing hormone (LH) levels in adults affected by this syndrome. Testicular histology revealed tubular atrophy and hyperplastic Leydig cells which are in accordance with the high testosterone levels and the difficulty in visualization of the testes. Because of the increased risk of gonadal malignancy, a laparoscopic gonadectomy was performed.

Adult↗
PubMed1992

Incorporating feminism into the graduate curriculum.

Feminist theory and methods are vital catalysts to teaching/learning in graduate education. This article describes how feminism was integrated into a graduate course, specifically regarding nursing activism and health policy. Major themes include a learning model emphasizing the student's individual strands of life experience, reconstruction of nursing history, and small-group process. Class assignments, student responses, and the implications of feminist perspectives for advanced professional practice are discussed.

Curriculum↗
PubMedDate not supplied

[Testicular feminization syndrome in children].

The paper is devoted to the clinical picture and the diagnosis of the syndrome of testicular feminization in children. Problems of the pathogenesis, treatment of this pathology and the choice of sex for their upbringing are given.

Adolescent↗
PubMed1999

[Immunohistochemical study of Sertoli-stromal cell tumor; comparison between the tumor arising from the gonad of a testicular feminization syndrome bearing patient and from ovaries of non-bearing patients].

The association of Sertoli-stromal cell tumor with testicular feminization syndrome (TFS) has been elaborated in the past studies. Here, we described immunohistochemical studies on Sertoli cell tumor of the gonad in a TFS patient and compare with 2 other cases of spontaneous ovarian Sertoli-stromal tumor. [Case 1] The case was a 73 year-old Japanese patient (46XY karyotype), who had had primary amenorrhea. High level of testosterone was noted in laboratory investigation (1900 ng/ml). No ambiguous morphology of external genitalia was present, but atrophy of vagina was noted. The patient was diagnosed as TFS. A left gonadal tumor was identified histologically showing well differentiated Sertoli cell tumor. The tumor cells were positive for anti-vimentin antibody but negative for anti-keratin, EMA and p53 antibodies by immunohistochemistry. The right gonad was an immature testis. [Case 2] The case was a 33 year-old female with ovarian Sertoli-Leydig cell tumor. Immunohistochemically, positive reaction for anti-keratin and p53 antibodies were observed. [Case 3] The case was a 17 year-old female with moderately differentiated Sertoli cell tumor of the ovary. The tumor cells were positive for anti-keratin, EMA and p53 antibodies by immunohistochemistry. Difference in immunohistochemical reactions between Sertoli cell tumor in TFS and Sertoli-stromal cell tumors of the ovaries was probably due to variation in the degree of gonadal development.

Adolescent↗
PubMed1999

[Feminizing genital plasty in patients with intersexuality].

Feminizing genitoplasty (FGP) involves modification of a hypertrophic clitoris, vaginoplasty, and modelling of the labia. FGP is performed during childhood and usually in one stage. In 1996-1999 30 patients with intersex were operated on. 25 girls with female pseudohermaphroditism caused by congenital adrenal hyperplasia (CAH), 3 patients with mixed gonadal dysgenesis (MGD), 1 patient with male pseudohermaphroditism and one female patient with true hermaphroditism (HFV). Seventeen patients were between the ages of 3 and 6 years, 13 patients (all female with CAH) were 13 to 21 years old. One-stage FGP, i.e. partial resection of the clitoris and vaginoplasty (VP) was performed in 11 patients. A two-stage operation, i.e. partial resection of the clitoris and subsequent vaginoplasty, was done in 3 patients (all with VHN with a high-orifice vagina). Vaginoplasty after excision of the clitoris was made in 13 patients (all with CAH, 5 of them with a high-orifice vagina). Three female patients (2 VHN, 1 HFV) had partial resection of the clitoris and wait to have VP. Fourteen patients with partial resection of the clitoris healed p.p. Healing p.s. was recorded in 5/27 patients with vaginoplasty (all CAH). Three of them were older, menstruating and obese. In 4/14 patients after partial resection of the clitoris both modelling and fixation of the glans clitoridis was necessary. Urinary incontinence was not recorded. Stricture of the vaginal orifice was found in one 6-year-old patient with CAH after reconstruction of a high-orifice vagina. Thirteen older patients have a normal vaginal orifice, two of them have sexual intercourse. FGP can be performed already at toddler age, the cosmetic effect is favourable, the glans clitoridis is preserved and the psychosexual development of patients is not impaired. FGP can be used also in adults, severely virilized patients.

Child↗