Hypogonadism Hypogonadism is when the sex glands produce little or no hormones.

Hypogonadism
Medical term which describes a diminished functional activity of the gonads that may result in diminished sex hormone biosynthesis and impaired gamete production and regulation. Spermatogenesis and ovulation in males and females may be impaired by hypogonadism. Depending on the degree of severity, may result in partial or complete infertility.

Female sex hormones control sexual development and the menstrual cycle. Under activity of the ovaries leads to low levels of these hormones in the body. A decline in hormone levels occurs naturally during the menopause, but at other times it may indicate an underlying disorder. Hypogonadism may cause distressing symptoms but is often treatable.

Male hypogonadism is defined as the failure of the testes to produce androgen, sperm, or both. Although the disorder is exceedingly common, its exact prevalence is uncertain.

Testosterone production declines with advancing age. This apparent physiologic decline in circulating androgen levels is compounded in frequency by permanent disorders of the hypothalamicpituitary-gonadal axis.

Transient deficiency states

acute stressful illnesses, such as surgery and myocardial infarction.

Chronic deficiency states

associated with wasting illnesses, such as cancer and acquired immunodeficiency syndrome.

Male factor infertility is probably responsible for one third of the 10% to 15% of couples who are unable to conceive within 1 year of unprotected intercourse

diminished, absent, or faulty spermatogenesis

obstructive ductal disease

epididymal hostility immunologic disorders

erectile or ejaculatory dysfunction

The cause of hypogonadism may be "primary" or "central. In primary hypogonadism, the ovaries or testes themselves do not function properly. Some causes of primary hypogonadism include:
Genetic and developmental disorders Surgery

autoimmune disorders

Infection

Radiation

Liver and kidney disease

The most common genetic disorders that cause primary hypogonadism

Ullrich-Turner syndrome (Gonadal

dysgenesis) Named after Henry H. Turner (American Endocrinologists) it is a chromosomal abnormality in which all or part of one of the sex chromosomes is absent or has abnormalities 45x karyotype It occurs only in 1:2000 1:5000 Females

Syndrome Manifestations

Short stature Broad chest Cubitus valgus Low hairline Webbed necks Lymph edema

Amenorrhea Increased weight, obesity Small fingernails Poor breast development Nonverbal Learning Disability (problems with math, social skills and spatial relations)

Medical Management
Growth hormone therapy Standard karyotyping
Estrogen Replacement therapy

Nursing Responsibilities
Careful monitoring throughout life for such possible complications as recurrent ear infections and possible hearing loss; kidney or thyroid disorders; heart problems; diabetes; and high blood pressure. They should have annual blood tests as part of routine physical checkups.

 They should watch their weight carefully because obesity increases their already high risk of diabetes. They can become pregnant with the help of assisted reproductive techniques.

Dr. Harry Klinefelter A condition in which a human has an extra X chromosome 47, XXY Exists in roughly between 1:500 to 1:1000 live male births

Syndrome Manifestations weak muscles less muscle control and coordination taller than average less facial and body hair broader hips

larger breasts ( gynecomastia) weaker bones lower energy level infertile microorchidism learning or reading impairment

Medical Management
Testosterone Breast tissue therapy removal
Speech therapy and Fertility educational support

treatment

Psychological Standard karyotyping counseling

Nursing Responsibilities
Provide supportive environment to both parent and client Educate the parents of the patient with Klinefelter syndrome Encourage patient to take part in activities to improve his physical motor skills, such as karate, soccer, basketball, baseball, or swimming.

In central hypogonadism, the centers in the brain that control the gonads (hypothalamus and pituitary) do not function properly. Some causes of central hypogonadism include:

Nutritional deficiencies

Bleeding

Certain medications, including steroids and opiates

Infections

Genetic problems

Iron excess (hemochromatosis)

Radiation, Surgery

Rapid, significant weight loss

Trauma

Tumors

A genetic cause of central hypogonadism that also takes away the sense of smell:

The most common tumors affecting the pituitary

Girls who have hypogonadism during childhood will not begin menstruating. The condition can affect girls' breast development and height. If hypogonadism occurs after puberty, symptoms include:

In boys, hypogonadism in childhood affects muscle and beard development and leads to growth problems. In men the usual symptoms are:

If a brain tumor is present (central hypogonadism), there may be:

Headaches or vision loss Milky breast discharge (from a Prolactinoma) Symptoms of other hormonal deficiencies (such as hypothyroidism) People with anorexia nervosa (who diet to the point of starvation) and those who lose a lot of weight very quickly (such as after gastric bypass surgery) also may have central hypogonadism.

 Pathophysiology The gonad (ovary or testis) functions as part of the hypothalamicpituitary-gonadal axis. A hypothalamic pulse generator resides in the arcuate nucleus, which releases luteinizing hormone (LH)releasing hormone (LHRH), which is also termed gonadotropinreleasing hormone (GnRH), into the hypothalamic-pituitary portal system. Data suggest that a gene named KISS is important in the development of the LHRH-secreting cells.[2, 3] In response to these pulses of LHRH, the anterior pituitary secretes follicle-stimulating hormone (FSH) and LH, which, in turn, stimulate gonadal activity. The increase in gonadal hormones results in lowered FSH and LH secretion at the pituitary level, completing the feedback loop. In the testes, LH stimulates Leydig cells to secrete testosterone, whereas FSH is necessary for tubular growth. In the ovaries, LH acts on theca and interstitial cells to produce progestins and androgens, and FSH acts on granulosa cells to stimulate aromatization of these precursor steroids to estrogen.

 Hypogonadism may occur if the hypothalamic-pituitarygonadal axis is interrupted at any level. Hypergonadotropic hypogonadism (primary hypogonadism) results if the gonad does not produce the amount of sex steroid sufficient to suppress secretion of LH and FSH at normal levels. Hypogonadotropic hypogonadism may result from failure of the hypothalamic LHRH pulse generator or from inability of the pituitary to respond with secretion of LH and FSH. Hypogonadotropic hypogonadism is most commonly observed as one aspect of multiple pituitary hormone deficiencies resulting from malformations (eg, septooptic dysplasia, other midline defects) or lesions of the pituitary that are acquired postnatally. In 1944, Kallmann and colleagues first described familial isolated gonadotropin deficiency. Recently, many other genetic causes for hypogonadotropic hypogonadism have been identified.

Normosmic hypogonadotropic hypogonadism, in which the sense of smell is not disrupted, has been associated with mutations in GNRH1, KISS1R, and GNRHRgenes. Although their exact functions are unclear, the genes TAC3 and TACR3have also been associated with normosmic hypogonadotropic hypogonadism.Kallmann syndrome (anosmic hypogonadotropic hypogonadism) has been associated with mutations in KAL1, FGFR1, FGF8, PROK2, and PROKR2 genes. The relationship with Kallmann syndrome is thought to be because these genes are all related to the development and migration of GnRH neurons. Mutations of an additional gene, CHD7, which has been associated with CHARGE syndrome, has also been found in patients with both normosmic or anosmic hypogonadotropic hypogonadism.

Pathophysiology The physiologic regulation of the hypothalamic-pituitary-gonadal axis is shown in Figure 1. Circulating testosterone is largely protein-boundthe major protein is sex hormonebinding globulin (SHBG)with only 2% present as the biologically active or free fraction. Some clinicians believe that the bioavailable fraction, the fraction present in the supernatant after ammonium sulfate precipitation, representing testosterone loosely bound predominantly to serum albumin, is more meaningful. Hepatic SHBG production rises with aging and thyroid hormone excess and declines in hyperinsulinemic states (obesity and type 2 diabetes), so that free testosterone values may not always be concordant with total testosterone values. The biologic effects of testosterone may be mediated directly by testosterone or by its metabolites 5-dihydrotestosterone or estradiol (Fig. 2). Male hypogonadism is caused by a primary (hypergonadotropic) testicular disorder or is secondary (hypo- or normogonadotropic) to hypothalamic-pituitary dysfunction, as illustrated in Figure 3. Combined disorders also occur. Examples of the major causes of male hypogonadism are shown in Boxes 1 and 2. Box 1 Primary (Hypergonadotropic) Hypogonadism: Major CausesGenetic: Klinefelters syndromeCongenital: anorchiaToxins: alcohol, heavy metalsOrchitisTraumaInfarctionAging Box 2 Secondary (Hypogonadotropic) Hypogonadism: Major CausesPubertal delayHypogonadotropism Congenital or acquired Isolated or combined pituitary disease Space-occupying lesions of pituitary, hypothalamus Hyperprolactinemia per se Infiltrative, infectious Suppression Sex steroids Gonadotropin-releasing hormone analogues