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Gonadotropin dynamics in XX males.

G Pérez-Palacios, M Medina, A Ullao-Aguirre

The Journal of Clinical Endocrinology and Metabolism

|August 1, 1981

Summary

XX males exhibit hypergonadotropic hypogonadism due to testicular failure. Their hypothalamic-pituitary axis shows preserved androgen responsiveness despite elevated LH and FSH levels and low testosterone.

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Area of Science:

Endocrinology
Reproductive Medicine
Genetics

Background:

XX males, a genetic condition characterized by the absence of the Y chromosome, often present with disorders of sexual development.
Assessing hypothalamic-pituitary gonadotropin function is crucial for understanding reproductive capabilities in individuals with XX male syndrome.

Purpose of the Study:

To evaluate the hypothalamic-pituitary gonadotropin function in postpubertal XX males.
To investigate the responsiveness of the hypothalamic-pituitary unit and testicular Leydig cells to hormonal stimulation and castration.

Main Methods:

Serum levels of luteinizing hormone (LH), follicle-stimulating hormone (FSH), and testosterone were measured.
Responses to human chorionic gonadotropin (hCG) and gonadotropin-releasing hormone (GnRH) were assessed.

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Hormonal dynamics were monitored before and after castration and during testosterone enanthate administration.

Main Results:

XX males presented with elevated LH and FSH, low testosterone, and subnormal Leydig cell response to hCG.
Normal LH and FSH responses to GnRH were observed, with a significant LH increase post-castration.
Testosterone administration suppressed gonadotropins and abolished the GnRH pituitary response, similar to other hypergonadotropic hypogonadism cases.

Conclusions:

XX males exhibit hypergonadotropic hypogonadism secondary to testicular failure.
The hypothalamic-pituitary unit in XX males demonstrates preserved androgen responsiveness.
These findings contribute to understanding the endocrine profiles in XX male syndrome.