Hypogonadism

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Hypogonadism

ICD-10	E28.3,E29.1,E23.0
ICD-9	257.2
OMIM	{{{OMIM}}}
DiseasesDB	{{{DiseasesDB}}}
MedlinePlus	{{{MedlinePlus}}}
eMedicine	{{{eMedicineSubj}}}/{{{eMedicineTopic}}}
MeSH	{{{MeshNumber}}}

Hypogonadism is a medical term for a defect of the reproductive system that results in lack of function of the gonads (ovaries or testes). The gonads have two functions: to produce hormones (testosterone, estradiol, antimullerian hormone, progesterone, inhibin B), activin and to produce gametes (eggs or sperm). Deficiency of sex hormones can result in defective primary or secondary sexual development, or withdrawal effects (e.g., premature menopause) in adults. Defective egg or sperm development results in infertility.

The term hypogonadism is usually applied to permanent rather than transient or reversible defects, and usually implies deficiency of reproductive hormones, with or without fertility defects. The term is less commonly used for infertility without hormone deficiency.

Classification

There are many possible types of hypogonadism and several ways to categorize them.

by Primary or Secondary

• Primary - defect is inherent within the gonad: eg. Noonan syndrome, Turner syndrome (45X,0), Klinefelter syndrome (47XXY), XX males with SRY gene
• Secondary - defect lies outside of the gonad: eg. Kallmann syndrome, Polycystic ovary syndrome and Adiposogenital dystrophy.

by Congenital vs. acquired

• An example of congenital hypogonadism (present at birth) is Turner syndrome.

by Hormones vs. fertility

Hypogonadism can involve just hormone production or just fertility, but most commonly involves both.

• Examples of hypogonadism that affect hormone production more than fertility are hypopituitarism and Kallmann syndrome; in both cases, fertility is reduced until hormones are replaced but can be achieved solely with hormone replacement.
• Examples of hypogonadism that affect fertility more than hormone production are Klinefelter syndrome and Kartagener syndrome.

by Affected system

Hypogonadism is also categorized by endocrinologists by the level of the reproductive system that is defective.

• Hypogonadism resulting from defects of the gonads is traditionally referred to as primary hypogonadism. Examples include Klinefelter syndrome and Turner syndrome. Mumps is known to cause testicular failure, and in recent years has been immunized against in the US. A varicocele can reduce hormonal production as well.

• Hypogonadism resulting from hypothalamic or pituitary defects are termed secondary hypogonadism or central hypogonadism (referring to the central nervous system).
  • Examples of Hypothalamic defects include Kallmann syndrome.
  • Examples of Pituitary defects include hypopituitarism.

• An example of a hypogonadism resulting from the lack of hormone response is androgen insensitivity syndrome, where there are inadequate receptors to bind the testosterone, resulting in a female appearance despite XY chromosomes.

Steroid use

Hypogonadism may be induced by chronic use of anabolic/androgenic steroids (AAS). The negative-feedback system of the hypothalamic-pituitary-gonadal axis (HPTA) shuts down pituitary production of gonadotropins after extended exposure to AAS. This has been documented both in patients receiving AAS for legitimate medical reasons such as AIDS or cancer and in athletes using AAS illicitly.

Hypogonadism may persist for some time after steroid use is discontinued, or indefinitely until treated.

Symptoms

In Men

• Effects of low testosterone in men may include: (not all are present in any single individual)
  • Word Finding difficulty (decrerase in verbal fluency)
  • Dry Skin
  • Cracking nails
  • Fatigue
  • Muscle loss/atrophy
  • Frequent urination without infection
  • Getting up at night to urinate
  • Dementia-like memory loss
  • Poor sleep secondary to achy muscles
  • Poor libido
  • Erectile Dysfunction
  • Increasing weight
  • Glucose intolerance (early diabetes)
  • Cholesterol/Lipid abnormalities
  • Slight increase size of breasts
  • Depression
  • Anxiety
  • Hot flashes
  • Decrease in beard and body hair growth
  • Loss of bone mass (osteoporosis )
  • Difficulty concentrating
  • Irritability
  • Psychological and relationship problems
  • Anaemia
  • Infertility
  • Decrease in firmness of testicles
  • Impaired growth of the penis and testicles

In Women

• Effects of low estrogen levels in women may include: (not all are present in any individual)
  • Hot flashes
  • Sleep disturbances
  • Symptoms of urinary bladder discomfort like frequency, urgency, frequent infections, lack of lubrication, discharge
  • Shrinking of breasts

Diagnosis

In Men

Low Testosterone can be identified through a simple blood test performed by a laboratory, ordered by a physician. Normal testosterone levels are said to range from 298 - 1098 ng/dl, but most men with levels below 350 will benefit from treatment.^{[How to reference and link to summary or text]}

Physicians measure gonadotropins (LH and FSH) to distinguish primary from secondary hypogonadism. In primary hypogonadism the LH and/or FSH are usually elevated, meaning the problem is in the testicles, whereas in secondary hypogonadism, both are normal or low, suggesting the problem is in the brain.

In Women

Similar to men, the LH and FSH will be used, particularly in women who believe they are in menopause. These levels changes during a woman's normal menstrual cycle, so the history of having ceased menstruation coupled with high levels aids the diagnosis of being menopausal. Commonly, the post-menopausal woman is not called hypogonadal

Hypogonadism is often discovered during evaluation of delayed puberty, but ordinary delay, which eventually results in normal pubertal development, wherein reproductive function is termed constitutional delay. It may be discovered during an infertility evaluation in either men or women.

Hypogonadism in older men is often overlooked by physicians, and may be a significant issue in the aging process.

Treatment

Hypogonadism is most often treated by replacement of the appropriate hormones. For men, this is testosterone. Commonly-used testosterone formulations include transdermal testosterone, injectable testosterone, and buccal testosterone. Oral testosterone is no longer used in the U.S. because it is broken down in the liver and rendered inactive. Another feasible alternative is human chorionic gonadotropin (hCG). For women estradiol and progesterone are replaced. Some types of fertility defects can be treated; some cannot.

Coping

Hypogonadism can have many psychological affects, especially in younger patients due to infertility and appearance. A supportive family that understands the condition is paramount, as well as psychological treatment. And excepting regular injections or Gel application and blood tests.

See also

• Gynecological disorders
• Male genital disorders

External links

• Anabolic Steroid Induced Hypogonadism Homepage
• NIH
• eMedicine
• Anabolic Steroid Induced Hypogonadism
• Low testosterone information
• Long-Term Pharmacokinetics and Clinical Efficacy of Andromen® Forte 5% Cream for Androgen Replacement Therapy in Hypogonadal Men
• Downloadable information booklet on Causes, Effects and Treatment of Hypogonadism