Hypogonadism

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Hypogonadism
Classification and external resources
ICD-10	E28..3,E29..1,E23..0
ICD-9	257.2
MeSH	D007006

Hypogonadism (also called Low testosterone or Low T) is a medical term for a defect of the reproductive system that results in lack of function of the gonads^[1] (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.

Contents 1 Classification 1.1 Primary or Secondary 1.2 Congenital vs. acquired 1.3 Hormones vs. fertility 1.4 Affected system 2 Steroid use 3 Symptoms 3.1 In Men 3.2 In Women 4 Diagnosis 4.1 In Men 4.2 In Women 5 Treatment 6 Coping 7 References 8 External links

[edit] Classification

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

[edit] 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 and Polycystic ovary syndrome, also called hypogonadotropic hypogonadism.^[2]

[edit] Congenital vs. acquired

• An example of congenital hypogonadism (present at birth) in females is Turner syndrome, and in males is Klinefelter syndrome.
• An example of acquired hypogonadism is the Anabolic Steroids Induced Hypogonadism (ASIH).

[edit] 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.

[edit] 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.

[edit] Steroid use

This section requires expansion.

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.

[edit] Symptoms

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[edit] In Men

• Effects of low testosterone in men may include: (not all are present in any single individual)^[3] and^[2]
  • Fatigue
  • Muscle loss/atrophy
  • Poor libido
  • Erectile Dysfunction
  • Slight increase size of breasts
  • Hot flashes
  • Decrease in growth of, or loss of, beard and body hair
  • Loss of bone mass (osteoporosis)
  • Irritability
  • Infertility
  • Impaired growth of the penis and testicles
  • Decrease in firmness of testicles
  • Frequent urination (polyuria) without infection/waking at night to urinate
  • Difficulty concentrating
  • Dementia-like memory loss
  • Depression
  • Anxiety
  • Psychological and relationship problems
  • Anemia
  • Increasing weight
  • Glucose intolerance (early diabetes)
  • Cholesterol/Lipid abnormalities
  • Poor sleep secondary to achy muscles
  • Night Sweats
  • Difficulty in choosing words in language
  • Dry Skin
  • Cracking nails

[edit] In Women

• Effects of low estrogen levels in women may include: (not all are present in any individual)^[4] and^[2]
  • Hot flashes
  • Irritability
  • Poor libido
  • Infertility
  • Loss of, or failure to develop, Menstruation
  • Loss of body hair
  • Loss of bone mass (osteoporosis)
  • Heart disease
  • Sleep disturbances
  • Symptoms of urinary bladder discomfort like frequency, urgency, frequent infections, lack of lubrication, discharge
  • Shrinking of breasts
  • loss of or non existent sense of smell

[edit] Diagnosis

[edit] 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.^{[citation needed]}

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.

[edit] In Women

Similar to men, the LH and FSH will be used, particularly in women who believe they are in menopause. These levels change 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.

[edit] 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).^[5]

For both men and women, Clomifene treatment can stimulate the body to naturally increase hormone levels while avoiding infertility and other side effects as a consequence of direct hormone replacement therapy.^[6]

For women, estradiol and progesterone are replaced. Some types of fertility defects can be treated, others cannot.

[edit] Coping

Hypogonadism can have many psychological effects, especially in younger patients due to infertility and appearance. A supportive family that understands the condition is paramount, as well as psychological treatment. Possible treatments include the use of regular injections or the application of gels or ointments.

[edit] References

[edit] External links

• Anabolic Steroid Induced Hypogonadism Homepage
• NIH
• eMedicine
• Anabolic Steroid Induced Hypogonadism
• Low testosterone information
• Understanding Hypogonadism in Children and Hypogonadism in Mature Males
• 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

v • d • e Endocrine pathology: endocrine diseases (E00-35, 240-259) Pancreas/ glucose metabolism Hypofunction Diabetes mellitus types: (type 1, type 2, MODY 1 2 3 4 5) · complications (coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy, cardiomyopathy) insulin receptor (Rabson-Mendenhall syndrome) · Insulin resistance Hyperfunction Hypoglycemia · Hyperinsulinism · Zollinger-Ellison syndrome Hypothalamic/ pituitary axes Hypothalamus gonadotropin (Kallmann syndrome, Adiposogenital dystrophy) · CRH (Tertiary adrenal insufficiency) · vasopressin (Neurogenic diabetes insipidus) · general (Hypothalamic hamartoma) Pituitary Hyperpituitarism anterior (Acromegaly, Hyperprolactinaemia, Pituitary ACTH hypersecretion) · posterior (SIADH) · general (Nelson's syndrome) Hypopituitarism anterior (Kallmann syndrome, Growth hormone deficiency, ACTH deficiency/Secondary adrenal insufficiency) · posterior (Neurogenic diabetes insipidus) · general (Empty sella syndrome, Pituitary apoplexy, Sheehan's syndrome, Lymphocytic hypophysitis) Thyroid Hypothyroidism Iodine deficiency · Cretinism (Congenital hypothyroidism) · Myxedema · Euthyroid sick syndrome Hyperthyroidism Hyperthyroxinemia (Thyroid hormone resistance, Familial dysalbuminemic hyperthyroxinemia) · Hashitoxicosis · Thyrotoxicosis factitia · Graves' disease Thyroiditis Acute infectious · Subacute (De Quervain's, Subacute lymphocytic) · Autoimmune/chronic (Hashimoto's, Postpartum, Riedel's) Goitre Endemic goitre · Toxic nodular goitre · Toxic multinodular goitre Thyroid nodule Parathyroid Hypoparathyroidism Pseudohypoparathyroidism Hyperparathyroidism Primary · Secondary · Tertiary · Osteitis fibrosa cystica Adrenal Hyperfunction aldosterone: Hyperaldosteronism/Primary aldosteronism (Conn syndrome, Bartter syndrome, Glucocorticoid remediable aldosteronism) · AME · Liddle's syndrome · 17α CAH cortisol: Cushing's syndrome (Pseudo-Cushing's syndrome) sex hormones: 21α CAH · 11β CAH Hypofunction/ Adrenal insufficiency (Addison's, WF) aldosterone: Hypoaldosteronism (21α CAH, 11β CAH) cortisol: CAH (Lipoid, 3β, 11β, 17α, 21α) sex hormones: 17α CAH Gonads ovarian (Polycystic ovary syndrome, Premature ovarian failure) testicular (5-alpha-reductase deficiency, 17-beta-hydroxysteroid dehydrogenase deficiency) · Androgen receptor (Androgen insensitivity syndrome) general (Hypogonadism, Delayed puberty, Precocious puberty) Height Gigantism · Dwarfism/Short stature (Laron syndrome, Psychogenic dwarfism) Multiple Autoimmune polyendocrine syndrome (APS1, APS2) · Carcinoid syndrome · Multiple endocrine neoplasia (1, 2A, 2B) · Progeria · Woodhouse-Sakati syndrome see also congenital, neoplasia