THE TESTIS IN PATIENTS WITH ABNORMALITIES OF SEX DIFFERENTIATION. HISTOLOGY AND ENDOCRINE FUNCTION

1973 ◽  
Vol 73 (1) ◽  
pp. 179-188 ◽  
Author(s):  
R. Sandrini Neto ◽  
M. A. Rivarola ◽  
R. Coco ◽  
C. Bergadá
Keyword(s):  
Sexual Differentiation ◽  
Normal Values ◽  
Sex Differentiation ◽  
External Genitalia ◽  
Poor Response ◽  
Testicular Tissue ◽  
Endocrine Function ◽  
Plasma Testosterone ◽  
Before And After ◽  
Female External Genitalia

ABSTRACT Testicular function was studied in 18 patients with abnormalities of sexual differentiation. Fourteen patients were prepubertal and four postpubertal. Plasma testosterone was determined before and after 5000 IU HCG administered daily during 5' days. Of the fourteen prepubertal patients, the two true hermaphrodites and the patient with asymmetrical gonadal differentiation showed a poor response to gonadotrophin stimulation. This could be explained by the small amount of active testicular tissue or its coexistence with ovarian and rudimentary gonadal tissue which might exert some influence on the endocrine function of the testis. In the group of eight patients with male pseudo-hermaphroditism and ambiguous external genitalia, the response to HCG showed a large variation. The three patients with small testes had lowest values i. e. 210, 198 and 192 ng/100 ml. The remaining five patients showed normal values ranging between 360 and 720 ng/100 ml. The ambiguous external genitalia of these patients could be explained as being due to a diminished androgen target organ sensitivity limited to the external genitalia. The three prepubertal patients with male pseudo-hermaphroditism and female external genitalia showed a marked increase in plasma testosterone with HCG ranging between 598 and 1100 ng/100 ml. The four pubertal cases also had high values in basal conditions, between 920 and 1870, which increased even more after gonadotrophin stimulation, in one case from 1382 to 2264 ng/100 ml. This hypersensitivity to exogenous HCG, even in infancy, correlates with the elevated basal values observed in adult patients with otherwise female external genitalia and androgen insensitivity. Finally, the HCG stimulation test could be useful for the demonstration of testicular tissue in prepubertal patient with abnormalities of sexual differentiation, although it cannot be used as a prognosis of masculinization or feminization at puberty.

True hermaphroditism: clinical aspects and molecular studies in 16 cases

1997 ◽  
Vol 136 (2) ◽  
pp. 201-204 ◽  
Author(s):  
Durval Damiani ◽  
Marc Fellous ◽  
Ken McElreavey ◽  
Sandrine Barbaux ◽  
Elenilde S A Barreto ◽  
...  
Keyword(s):  
Sexual Differentiation ◽  
Laboratory Data ◽  
External Genitalia ◽  
Testicular Tissue ◽  
Clinical Aspects ◽  
Diagnostic Challenge ◽  
True Hermaphroditism ◽  
Main Complaint ◽  
Testicular Differentiation ◽  
Made In

Abstract Although true hermaphroditism (TH) accounts for less than 10% of intersex patients, it stands as a diagnostic challenge and has allowed a better understanding of the mechanisms involved in sexual differentiation. In this paper we review the clinical and laboratory data as well as molecular biology findings on 16 TH patients followed up at the Pediatric Endocrine Unit, Instituto da Criança, Hospital das Clínicas, São Paulo University Medical School. They were of a mean age of 3 years 8 months and nine of them were black. All the patients had ambiguous external genitalia as the main complaint. The 46,XX karyotype accounted for 50% of the cases and the ovotestis was the most frequent gonad found (59%). In the eight TH patients with a 46,XX karyotype, the sex-determining region of the Y chromosome (SRY) was negative, posing an intriguing question about the testicular differentiation mechanisms involved in these cases. In 7/19 ovotestes, the ovarian portion of the gonad has been preserved, keeping open the possibility of fertility. The female sex option was made in 10/16 cases (62·5%) and three patients exhibited spontaneous puberty. The mechanism through which testicular tissue develops without SRY has not yet been completely clarified, suggesting the involvement of the X chromosome as well as autosomal genes in the process. European Journal of Endocrinology 136 201–204

ENDOCRINE TESTICULAR FUNCTION IN VIVO AND IN VITRO IN INFERTILE MEN

1979 ◽  
Vol 90 (3) ◽  
pp. 544-551 ◽  
Author(s):  
E. Nieschlag ◽  
E. J. Wickings ◽  
J. Mauss
Keyword(s):  
Leydig Cell ◽  
Cell Function ◽  
Testicular Tissue ◽  
Plasma Testosterone ◽  
Cell Hyperplasia ◽  
Testosterone Levels ◽  
Infertile Men ◽  
Before And After

ABSTRACT In order to detect any possible Leydig cell dysfunction associated with male infertility, the endocrine capacity of the testes was investigated in vivo and in vitro in 21 infertile men. Plasma testosterone was determined before and after 3 days of hCG stimulation. Testicular tissue obtained by bilateral biopsies was subjected to (1) histological examination, (2) determination of basal testosterone concentration and (3) incubation with hCG. Patients were grouped according to histology. In vitro basal and stimulated testicular testosterone was similar in patients with normal histology, Sertoli-cell-only syndrome and spermatogenic arrest. Tissue from patients with Leydig cell hyperplasia showed 3-fold higher basal testosterone levels and a greater response to hCG. All patients had plasma testosterone levels and responses to hCG in the normal range. There was no significant correlation between the data obtained in vivo and in vitro, indicating that testosterone determinations in peripheral blood do not necessarily reflect the intratesticular situation. There was no evidence for gross abnormality in Leydig cell function accompanying disturbed spermatogenesis.

scholarly journals Nuclear androgen receptor regulates testes organization and oocyte maturation in zebrafish

2017 ◽  
Author(s):  
Camerron M. Crowder ◽  
Christopher S. Lassiter ◽  
Daniel A. Gorelick
Keyword(s):  
Androgen Receptor ◽  
Oocyte Maturation ◽  
Sexual Differentiation ◽  
Gonad Development ◽  
External Genitalia ◽  
Endocrine Function ◽  
Secondary Sexual Characteristics ◽  
Gonad Differentiation ◽  
Sexual Characteristics

ABSTRACTAndrogens act through the nuclear androgen receptor (AR) to regulate gonad differentiation and development. In mice, AR is required for spermatogenesis, testis development and formation of external genitalia in males and oocyte maturation in females. However, the extent to which these phenotypes are conserved in nonmammalian vertebrates is not well understood, because mutations in AR have not been generated in any other species. Here, we generate zebrafish with a mutation in the ar gene and examine the role of AR on sexual differentiation and gonad development. We find that zebrafish AR is not required for male sexual differentiation, since a portion of ar mutants develop a testis. However, we show that in zebrafish, as in mice, AR is required for the development of secondary sexual characteristics and for proper organization of the testis in males and for oocyte maturation in females. Additionally, we find that zebrafish ar mutant males have functional, mature sperm present in their testis, but are infertile due to an inability to release sperm. These findings suggest that AR is required for male sexual development and fertility, but not essential for sexual differentiation in zebrafish. The ar mutant we developed will be useful for modeling human endocrine function in zebrafish.

Inherited male pseudohermaphroditism due to gonadotrophin unresponsiveness

1981 ◽  
Vol 98 (1) ◽  
pp. 148-155 ◽  
Author(s):  
Gregorio Pérez-Palacios ◽  
Hugo E. Scaglia ◽  
Susana Kofman-Alfaro ◽  
Dolores Saavedra O. ◽  
Sergio Ochoa ◽  
...  
Keyword(s):  
Elevated Serum ◽  
External Genitalia ◽  
Serum Levels ◽  
Testicular Tissue ◽  
Male Pseudohermaphroditism ◽  
Breast Development ◽  
Seminiferous Tubules ◽  
Differentiated Cells ◽  
Before And After ◽  
Poorly Differentiated

Abstract. An inherited form of incomplete male pseudohermaphroditism was studied in two post-pubertal and one pre-pubertal sibling. All patients presented a 46XY karyotype, infantile female external genitalia, lack of breast development and sexual hair. Persistently elevated serum levels of gonadotrophins with normal pituitary responsiveness to LRH were found. Serum 17-OH progesterone, androstenedione, and testosterone levels were extremely low before and after gonadal stimulation with hCG. Laparotomy revealed absence of Wolffian and Mullerian derivatives. Testes were small and cryptorchidic. Microscopic and ultrastructural examination revealed seminiferous tubules with absence of spermatogenesis and normal Sertoli cells. The interstitial spaces were mainly occupied by poorly differentiated cells although in the post-pubertal patients there were small and randomly distributed nodules of Leydig cells without crystaloids. Incubation of testicular tissue from one post-pubertal patient with [14C]acetate showed lack of 14C-incorporation into appropriate steroid carriers. These data were interpreted as demonstrating that gonadotrophin resistance was the underlying abnormality of this syndrome, representing the human counterpart of the 'vet' pseudohermaphroditic rat.

scholarly journals Sex Differentiation in Amphibians: Effect of Temperature and Its Influence on Sex Reversal

2021 ◽  
pp. 1-11
Author(s):  
Adrián Ruiz-García ◽  
Álvaro S. Roco ◽  
Mónica Bullejos
Keyword(s):  
Sexual Differentiation ◽  
Sex Differentiation ◽  
Environmental Contaminants ◽  
Natural Populations ◽  
Sex Reversal ◽  
The Other ◽  
Effect Of Temperature ◽  
Amphibian Species ◽  
Expected Increase

The role of environmental factors in sexual differentiation in amphibians is not new. The effect of hormones or hormone-like compounds is widely demonstrated. However, the effect of temperature has traditionally been regarded as something anecdotal that occurs in extreme situations and not as a factor to be considered. The data currently available reveal a different situation. Sexual differentiation in some amphibian species can be altered even by small changes in temperature. On the other hand, although not proven, it is possible that temperature is related to the appearance of sex-reversed individuals in natural populations under conditions unrelated to environmental contaminants. According to this, temperature, through sex reversal (phenotypic sex opposed to genetic sex), could play an important role in the turnover of sex-determining genes and in the maintenance of homomorphic sex chromosomes in this group. Accordingly, and given the expected increase in global temperatures, growth and sexual differentiation in amphibians could easily be affected, altering the sex ratio in natural populations and posing major conservation challenges for a group in worldwide decline. It is therefore particularly urgent to understand the mechanism by which temperature affects sexual differentiation in amphibians.

THE METABOLISM OF TESTOSTERONE, ANDROSTENEDIONE AND OESTRONE BY TESTES FROM A CASE OF TESTICULAR FEMINIZATION

1968 ◽  
Vol 42 (3) ◽  
pp. 391-403 ◽  
Author(s):  
A. P. WADE ◽  
G. S. WILKINSON ◽  
J. C. DAVIS ◽  
T. N. A. JEFFCOATE
Keyword(s):  
Testicular Tissue ◽  
Major Metabolite ◽  
Testicular Feminization ◽  
Phenolic Fraction ◽  
Enzyme Systems ◽  
Before And After ◽  
Complete Form ◽  
Urinary Steroid ◽  
Chromatographic Evidence

SUMMARY [4-14C]Testosterone, [4-14C]androstenedione and [4-14C]oestrone were incubated with testicular tissue obtained from an 18-yr.-old patient with the complete form of the testicular feminization syndrome. Considerable biosynthesis of testosterone from androstenedione occurred, but metabolism of testosterone by the tissue was minimal. The small phenolic fraction from these incubations did not contain any recognizable oestrogens. Metabolism of oestrone was almost complete, less than 4% being recovered as unchanged oestrone. Of eight areas of radioactivity found during chromatography, five were shown to be associated with oestrone, 2-methoxyoestrone, oestradiol-17β, 2-hydroxyoestradiol-17β, and 2-methoxy-oestradiol-17β. Chromatographic evidence suggested that an oxo-oestrone and a compound more polar than oestriol were present. No oestriol was found. The results confirm those of other workers to the effect that testosterone is the major metabolite of androstenedione in feminizing testes. Incubation of the testes with oestrone showed them also to possess the enzyme systems necessary for hydroxylation at position 2 and the subsequent methylation of this group. Urinary steroid measurements before and after removal of the testes showed these organs to be actively secreting. Attempts to demonstrate oestrogenic activity in the urine additional to that accounted for by chemical estimation were unsuccessful.

scholarly journals The Role of Endothelial Dysfunction in the Development of Cardiotoxic Action of Cytostatics in Patients with Lymphoproliferative Diseases

Kardiologiia ◽  
2019 ◽  
Vol 59 (4) ◽  
pp. 64-66 ◽  
Author(s):  
D. A. Budanova ◽  
Yu. N. Belenkov ◽  
I. Ya. Sokolova ◽  
O. N. Antyufeeva ◽  
V. I. Ershov ◽  
...  
Keyword(s):  
Endothelial Dysfunction ◽  
Normal Values ◽  
Clear Understanding ◽  
Age Group ◽  
Lymphoproliferative Diseases ◽  
Endothelin 1 ◽  
Vasoconstrictor Effect ◽  
Before And After ◽  
Chemotherapy Cardiotoxicity

Understanding mechanisms of chemotherapy cardiotoxicity is an important problem due to the lack of clear understanding of its occurrence. Development of endothelial dysfunction is considered to be one of possible ways in implementation of these side effects. The analysis of endothelin-1 and e-selectin levels in 26  patients with lymphoproliferative diseases before and after the completion of the treatment program was been performed. The results of the study showed normal values of E-selectin level and increased level of endothelin-1 in the whole group of patients before treatment. After completion of chemotherapy program, in the whole group, there was a decrease of these two markers. However, values of level of endothelin-1 with vasoconstrictor effect remained high even after the end of therapy. It is imp ortant that at detailed analysis the dynamics of investigated markers in patients of older age group (median age 64 years) was associated with worsening of endothelial dysfunction.

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