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At least 19 recordsLinked to original sources
PubMed1976

Gonadal calcification. Indication for gonadectomy in gonadal dysgenesis.

Germ cell tumors, including gonadoblastoma, are usually found in patients with gonadal dysgenesis who have Y chromatin in their genotype. Diffuse calcification is a common finding in gonadoblastomas. Bilateral calcifications in the gonadal sites were seen on the intravenous pyelogram of a patient with 46,XX pure gonadal dysgenesis. Although the calcification was present in the fimbria of the fallopian tube, we suggest that gonadectomy is indicated in the patient with gonadal dysgenesis and probably gonadal calcification.

Adolescent↗
PubMed1979

A patient with pure gonadal dysgenesis, gonadal tumour and virilisation.

A phenotypic female, karyotype XY presented with virilisation and amenorrhoea. The gonads showed evidence of oestrogen and testosterone production and, in view of the risk of malignancy, a laparotomy was performed. A streak gonad was present on one side with a streak gonad containing discrete tumours on the other. The uterus and Fallopian tubes were present. Removal of the gonads and uterus and subsequent oestrogen replacement therapy have produced a satisfactory clinical result.

Adult↗
PubMed1976

[Evaluation of gonadotropic and gonadal secretions in primary abnormalities of the gonads and male pseudohermaphrodism before and after the age of puberty].

Blood levels of gonadotropins (FSH and LH) and gonadal steroids (testosterone or estradiol) have been evaluated in 72 children and adolescents with primary gonadal defects (45 agonadal patients, 7 with asymetrical gonadal dysgenesis, 9 with Klinefelter syndrome, 4 with partial testicular dysgenesis and 7 with partial ovarian dysgenesis) and in 17 cases of male pseudohermaphrodism. A LH-RH test has been performed in most cases, and a testicular stimulation test with chorionic gonadotropin in patients with testicular tissue. Agonadal subjects had increased blood and pituitary releasable gonadotropins: very high in infants and young children, much less from 7 to 11 years, a high spurt being observed at 12 years. This diphasic pattern relates to the varying sensitivity of hypothalamic receptors and suggests that adrenal steroids may restrain gonadotropic secretion at 7-8 years. As a diagnostic tool, the increase of gonadotropins may be missing in the 7-11 years group. In the 1-12 years patients with partially defective gonads, blood and pituitary releasable gonadotropins and blood steroids are usually normal, but testosterone response to chorionic gonadotropin may be already blunted. From 13 years the gonadotropic secretion is usually increased, even when testosterone secretion and reserve are within normal range. Most male pseudohermaphrodites showed normal gonadotropic and testicular secretions. But four patients had highly increased responses to LH-RH, suggesting a defect of testicular secretion or of receptors, and demonstrating some heterogeneity in the male pseudohermaphrodism group.

Adolescent↗
PubMed1978

Hemagglutinins in female fish gonads: comparative investigations on perch (Perca fluviatilis) gonads of different stages of development.

Female gonads of the perch (Perca fluviatilis) in various stages of development are tested for hemagglutinin activity against human erythrocytes. Based on the different agglutination patterns obtained, and on inhibition tests with L-fucose and a mature gonad of a male perch, the appearance two different agglutinins in the course of the development of the female gonad is assumed.

Agglutinins↗
PubMed1979

[Biology of the Rajidae from Tunisian coasts. XX. Raja clavata Linne, 1758. Weight of the body, liver and gonads. Liver and gonadal ratio. Coefficients of condition].

The body and liver growth-weight rate is faster in the subadults ones that in the youngs and the adults whatever the sex, as gonads growth-weight rate in the males and nidamental glands in the females; ovaries growth-weight rate is, at the contrary more important in the adults ones. In all individuals during growth or along the year, the variation of cubic and relative of condition ane subjected to liver, gonads and ponderal changes. The part of ecological factors and diet are not to be important but seems more difficult to point out.

Animals↗
PubMed1979

Evidence for a gonad-specific receptor for H-Y antigen: binding of exogenous H-Y antigen to gonadal cells is independent of beta 2-microglobulin.

This report addresses the question whether two different types of binding exist for the reaction of H-Y antigen with the cell surface. Anti-H-Y antiserum in the presence of complement was cytotoxic only for gonadal cells expressing their own H-Y antigen, but not to ovarian cells loaded with H-Y antigen. H-Y antigen was co-redistributed with beta 2--microglobulin on newborn testicular cells, but some residual H-Y activity was found on similarly treated testis cells from 15 day old rats. After beta 2--microglobulin redistribution, testis cells maintained their binding capacity for exogenous H-Y antigen prepared from epididymal fluid or Daudi cell culture supernatants. This result suggests that exogenous H-Y antigen is bound via a gonad-specific receptor which is independent of beta 2--microglobulin and that this type of binding for H-Y antigen is different from the beta 2--m-associated expression of H-Y antigen on the cell surface.

Animals↗
PubMed1979

Hypothalamic-pituitary responses to high concentrations of gonadal steroids in a three-month-old infant with a feminizing gonadal stromal tumor.

A 3-month-old female infant feminized by an ovarian stromal tumor is presented. Clinicopathologic aspects of such tumors occurring in infancy are discussed. In addition, the evaluation of hypothalamic-pituitary function preoperatively and postoperatively are presented. In the preoperative, high-steroid environment, both basal and stimulated secretion of follicle-stimulating hormone (FSH) was suppressed. Secretion of luteinizing hormone (LH) was normal. Following surgical castration, stimulated secretion of both gonadotropins clearly increased. These findings suggest an active but less sensitive negative feedback mechanism for FSH than for LH during infancy, i.e., high concentrations of gonadal steroids are necessary to maximally suppress FSH secretion while normal steroid concentrations appear to maximally suppress LH secretion. Developmental changes in hypothalamic-pituitary sensitivity to negative feedback controls are discussed in light of these findings.

Female↗
PubMed1976

Enzymatic sulfation of steroids: II. The control of the hepatic cortisol sulfotransferase activity and of the individual hepatic steroid sulfotransferases of rats by gonads and gonadal hormones.

Ovariectomy has relatively little effect on hepatic cortisol sulfotransferase activity (HCSA) in female rats, diminishing it only 30%. On the other hand, castration more than doubles HCSA in males. HCSA is due to 3 steroid sulfotransferases, STI, STII, and STIII. Its dimunition in ovariectomized rats is due to decreased STI and STII. Castration of males results in elevation of STII. Thus, ovaries appear to stimulate STI and STII production and testes appear to inhibit production of STII and perhaps STI. Studies with testosterone and estradiol-17beta support a role for sex hormones as mediators of gonadal effects on HCSA, by stimulating or inhibiting production of the individual enzymes. Estradiol-17beta administration reverses the effect of ovariectomy on HCSA. Testosterone administration to intact or castrated females decreases HCSA by 60-70%, due to disappearance of all STI and most STII activity. Thus, androgen administration appears to suppress both STI and STII production. In intact males testosterone administration elevates HCSA 70-80% due to increased STIII. Estradiol-17beta administration to intact or castrated males elevates HCSA 9-10-fold. In intact animals this is due to elevated STI and STII but not STIII. In castrates all three enzymes are elevated by the estrogen.

Animals↗
PubMed1976

Pituitary-gonadal relations in infancy: 2. Patterns of serum gonadal steroid concentrations in man from birth to two years of age.

Testosterone, estradiol, 170H-progesterone, and androstenedione (except in cord samples) concentrations were determined in cord sera (30 male and 14 female) and in peripheral sera from infants (121 male and 110 female), age 1 day to 2 years. Male and female cord serum levels of these steroids were not significantly different. In both sexes levels during the first week were lower than those in cord sera. In male infants serum testosterone and 170H-progesterone levels rose sharply in the second week of life, reached a peak at 1-2 months, and then declined to the range seen in later childhood by 6 months of age; male serum androstenedione and estradiol concentrations were higher during the first 2 months of life, but no distinct pattern of rise and fall was seen. In girls serum testosterone levels fell in the first week to the range seen throughout childhood; serum concentration of estradiol, androstenedione, and 17OH-progesterone in girls were markedly variable, with many values above the childhood range being seen, particularly in the first 6 months. These data provide further evidence of active Leydig cell function in male infants. They suggest that there is also ovarian secretion of sex steroids in some female infants in response to the elevated FSH and LH levels which are seen at this time.

Adolescent↗
PubMed1975

Mixed gonadal dysgenesis.

Mixed gonadal dysgenesis (MCG) is a rare intersexual disorder, characterized in most cases by the presence of a testis and a contralateral streak gonad; in some cases the contralateral gonad may be rudimentary not having differentiated into an ovary or into a testis and in other cases it may be absent. A personal case was recently studied by the authors in some detail: A 51-year-old individual reared as a female with primary amenorrhea, short stature, subnormal intelligence, male type habitus, hirsutism, moderate breast development, ambiguous external genitalia with a 5 x 2 cm phallus, labia majora with scrotal type skin, and a urogenital sinus. Internal genitalia consisted of a hypoplastic vagina and uterus, a streak gonad on the right and intraabdominal testis, fallopian tube, and epididymis on the left side. The dysgenetic testis contained a gonadoblastoma and the streak a gonadoblastoma and dysgerminoma. A buccal smear was chromatin negative and karyotype analysis revealed XO/XY mosaicism. Thyroid and adrenocortical function was normal. A plasma testosterone level of 0.15 mu-g% rose to 0.45 mu-g% with 3 days of HCG administration. With bilateral gonadectomy the plasma testosterone level fell to 0.06 mu-g% and there was no response following HCG administration. 109 cases of MGD and 2 cases of unilateral gonadal dysgenesis reported in the world literature between 1937 and 1973 were reviewed. On the basis of their clinical and pathological features, the latter two patients were excluded from the category of MGD. In 15 of the 109 cases of MGD, a gonadoblastoma obscuring the gonad of origin was opposed by a dysgenetic gonad with or without a gonadoblastoma or no gonad. In these cases the presence of a testis was not proven histologically and their clinical-pathological features were similar to those found in pure gonadal dysgenesis. Of the remaining 94 cases of MGD, 72 had testicular tissue on one side and a streak or embryonic gonad on the other while 22 had a testis and no contralateral gonad...

Amenorrhea↗
PubMed1975

Laparoscopy in endocrine and genetic disorders of the gonads.

The guideline of this work is to examine the diagnostic value of laparoscopy in some endocrine and genetic syndromes connected with gonadal pathology or abnormal sexual development in relation to the various hormonal and cytogenetic techniques usually utilized by the endocrinologist. After a brief introduction, Chapter 1 stresses our purpose to examine the advantages and limits of laparoscopy by investigating the most significant cases that came to our observation. Attention is drawn in Chapter 2 to the application of laparoscopy in a particular form of amenorrhea (uterus absence), admittedly of great endocrinologic interest. In particular, the results obtained by the application of this technique are reported in cases of uterus agenesis and the testicular feminization syndrome. The clinical, cytogenetic and hormonologic study of one case of uterus agenesis is reported together with that of four cases of the testicular feminization syndrome with the view to discussing the differential diagnosis between these two syndromes and the particular diagnostic problems connected with the latter. Our conclusion is that laparoscopy, although usually having only a confirmatory value in these syndromes, may assume in certain cases major diagnostic importance (the post-puberal testicular feminization syndrome without palpable gonads, the pre-puberal testicular feminization syndrome, rare cases of the testicular feminization syndrome associated with chromosome aberrations). Chapter 3 discusses the application of laparoscopy in female gonadal dysgenesis on the basis of three cases of Turner's syndrome with different diagnostic problems and one of pure gonadal dysgenesis. It is stressed that, although in the majority of cases of Turner's syndrome the laparoscopic findings are quite characteristic (streak gonads), in some cases the gonads may also present the aspect of hypoplastic ovaries. This finding is observed in two cases of Turner's syndrome with XO/XX mosaicism, of which one case with spontaneous menarche. The diagnostic value of laparoscopy is discussed in the cases of pure gonadal dysgenesis and in variants of Turner's syndrome chromatin-positive with X chromosome partial monosomy and chromatin-negative with a Y chromosome. Our conclusion is that laparoscopy assumes particular diagnostic value in cases of pure gonadal dysgenesis without chromosome aberrations, because chromosome aberrations may be absent in this syndrome, as well as in chromatin-negative variants with a Y chromosome because the gonads and gonaducts differentiation may be variable.

Adolescent↗
PubMed1977

Morphological and biochemical findings in the tissues of maldeveloped gonads in 45,XX females with early primary ovarian insufficiency.

The gonads or excisions there of obtained from 13 cases of early primary ovarian failure in 46,XX females subjected to explorative laparotomy have been examined microscopically, histochemically and biochemically. Clinically, the cases were characterized by either primary amenorrhoea or by one or several spontaneous menstruations followed by amenorrhoea until operation. Roentgenologically and macroscopically, the gonads were characterized as either "steak" gonads or hypoplastic ovaries. There were no associated somatic anomalies except for slight dysplastic bone changes and/or osteoporosis. Based on morphological findings, the gonads were grouped as follows. Type A ("dysgenetic" or "aplastic") gonads characterized by a complete absence of follicular apparatus or its remnants were composed of ovarian cortical-like stroma. Some of them contained hilus cells and rete structures. Type B gonads were severely hypoplastic ovaries differing from the previous ones by the presence of various active, but predominantly inactive derivatives of the follicular apparatus, such as scarce primordial or growing follicles, corpora albicantia or atretica. Type C gonads were designated as hypoplastic sclerocystic ovaries showing a microscopical pattern similar to that of the "Stein-Leventhal ovaries", with follicles, follicular cysts and predominantly perifollicular hyperthecosis. Biochemical investigations were based on incubation studies with labelled precursors. They permitted to conclude that steroid biosynthesis was severely affected in all the gonads examined, differing most markedly from the ordinary ovarian steroid biosynthesis in the A-type gonads, and being rather similar to that observed in the "Stein-Leventhal ovaries" in the C-type ones.

Adolescent↗