What is Hypergonadism?
Hypergonadism (also known as “hypergamy”) refers to a condition where there are too many male hormones in the body. This means that the testicles produce too much testosterone, which causes problems with sexual development and other physical characteristics.
A normal male’s testosterone level ranges from 200 – 800 ng/dL. A man with high levels of testosterone may experience symptoms such as erectile dysfunction, decreased libido, acne, hair loss or baldness. These symptoms usually improve after treatment with antiandrogens.
However, some men have no effect upon their testosterone levels when treated appropriately. If untreated these symptoms can lead to infertility and increased risk of prostate cancer in older men.
In women, low levels of testosterone can cause irregular periods, obesity, osteoporosis and depression. Low levels of estrogen can increase the risk of developing certain cancers including ovarian and uterine cancer. Women with hypergonadism may develop gynecomastia (increased growth of male secondary sexual characteristics) due to elevated levels of male hormones in their bodies.
What are gonads?
Gonads are the organs that produce gametes in animals. Sperm in males and ova (eggs) in females are known as gametes. The word “gonad” comes from the Greek word “gonas” which means “seed”. Unlike other body organs, gonads are not essential for survival. In fact, humans can live perfectly fine without gonads. There are two types of gonads:
Gonads in males and females – These include the testes and ovaries respectively. While these gonads produce gametes, they also produce hormones such as testosterone and estrogen.
Gonads in hermaphroditic animals- This includes some plants such as mosses, liverworts and ferns as well as some flatworms. In these animals, gametes are produced by the body throughout its life cycle.
What are the causes of hypergonadism?
The main cause of hypergonadism is an over-production of male hormones such as testosterone. The following conditions can lead to elevated testosterone levels:
Androgen insensitivity syndrome (AIS) – In this condition, the body is not able to respond to androgens such as testosterone. As a result of this, the body produces excessive amounts of testosterone and other androgens.
5-alpha-reductase deficiency – Enzymes known as 5-alpha-reductases are responsible for converting testosterone to a more potent form of androgen called dihydrotestosterone (DHT). Because 5-alpha-reductase is defective, more testosterone circulates in the blood instead of DHT.
Klinefelter’s syndrome – In this condition, a male has an extra “X” chromosome. As a result of this, the male develops small testes that do not produce enough testosterone for normal sexual development.
Women with polycystic ovarian syndrome (PCOS) also experience higher than normal levels of testosterone in the blood. This occurs when special cells in the ovaries known as theca cells produce increased amounts of androgens. The exact cause of this is not known but it may be related to lack of ovulation in women suffering from PCOS.
Pituitary tumors – These tumors are rare and can cause over-production of hormones. If a tumor is pressing on the hypothalamus at the base of the brain, it can interfere with the body’s production and regulation of its own hormones. This is known as secondary hypersecretion of hormones.
What are the symptoms of hypergonadism?
The symptoms of hypergonadism are related to the condition that is causing an over-production of male hormones. These can be:
Development of male sexual characteristics such as facial hair growth, deepening of the voice and a large muscular build.
Enlarged genitalia or clitoris in women.
Infertility
Reduced body fat and an increase in muscle mass
How is hypergonadism diagnosed?
The following tests can help the doctor arrive at a diagnosis:
Hormone tests – These can be performed to measure the levels of important hormones such as testosterone, luteinizing hormone (LH), and follicle-stimulating hormone (FSH).
Ultrasound test – This can reveal whether or not there is an ovarian cyst or tumor present.
What is the treatment for hypergonadism?
Treatment will depend on the underlying cause. For instance:
Some babies are born with ambiguous genitalia and are diagnosed with AIS. After a period of observation, the baby’s genitalia is surgically altered to resemble that of a girl. This child will be brought up as a girl and given hormonal therapy to promote female characteristics such as the growth of vaginal tissue.
In adolescents and adults, the excessive male hormones can be reduced using hormonal therapy and surgery. The goal of treatment in these cases is to give the patient a more “feminine appearance” without reducing their libido or sexual function. This may be achieved by reducing the size of the clitoris, deepening the voice, or removal of excessive body hair.
In some cases, if fertility is desired, the ovaries and uterus can be preserved.
How is hypergonadism treated in children?
Children with hypergonadism are usually diagnosed very soon after birth because of ambiguous genitalia. If the condition is due to AIS, no immediate treatment may be necessary until the patient reaches puberty. At this time, hormonal therapy and surgery can be used to make the genitals resemble those of a female. The patient may also opt for a mastectomy (breast removal) and take estrogen to promote female characteristics such as the growth of vaginal tissue.
What is the outlook for people with hypergonadism?
The outlook is good for patients with AIS. If diagnosed before puberty, normal reproductive function can be achieved. If diagnosed after puberty, fertility may be impossible but normal female sexual characteristics can still be achieved.
For patients with POI, the outlook also appears to be good. With early diagnosis and treatment, normal female sexual function can still be achieved.
Last full review/revision December 2013.
Source: PubMed Plus
Sources & references used in this article:
Characterization of the hypothalamic-pituitary-gonadal axis in estrogen receptor (ER) null mice reveals hypergonadism and endocrine sex reversal in females lacking … by JF Couse, MM Yates, VR Walker… – Molecular …, 2003 – academic.oup.com
Advances in the Molecular Genetics of Hypogonadotropic Hypergonadism by JC Achermann, JL Jameson – Journal of Pediatric Endocrinology …, 2001 – degruyter.com
Hypergonadotrophic hypogonadism due to testicular adrenal rest tumours presenting with hypogonadotrophic hypergonadism by HL Ashby, RM Gama, H Sur, J Inglis… – Annals of clinical …, 2012 – journals.sagepub.com
Coexistence of primary endocrine deficiencies: a unique case of male hypergonadism associated with hypoparathyroidism, hypoadrenocorticism, and hypothyroidism. by U Weinberg, FB Kraemer… – The American journal of …, 1976 – europepmc.org
Osteoporosis with hypergonadism-2nd time lucky by MR Jenner, RP Kelch – J. clin. Endocrinol, 1982
Raised alpha-subunit does not always predict gonadotrophinoma in secondary hypergonadism by L Krishnan, H Hassan – Society for Endocrinology BES …, 2019 – endocrine-abstracts.org
Selective testicular 11beta-HSD inhibitors for the treatment of hypergonadism associated disorders and modulation of fertility by СN Hoffmann – Derm. Ztschr, 1921