Klinefelter syndrome is a chromosome disorder in males that results in hypogonadism (small penis and small firm testicles).
Hypogonadism is the condition in which the production of sex hormones and germ cells (sperm and eggs) is inadequate.
Hypogonadism may occur at any age; however, consequences differ according to the age at onset.
If hypogonadism occurs prenatally (even if incomplete), sexual ambiguity may result.
If hypogonadism occurs before puberty, puberty does not progress.
If hypogonadism occurs after puberty, infertility and sexual dysfunction result.
The demographics of hypogonadism vary depending on the cause.
Kallman's syndrome (KS) is the most frequent cause of hypogonadotropic hypogonadism and affects approximately one in 10,000 males and one in 50,000 females.
There are a number of causes of hypogonadism, including stress, elevated prolactin levels, and several genetic disorders.
Hypogonadism most often shows up as an abnormality in boys during puberty.
The most common problems with too little hormone happen during menopause, which is normal hypogonadism.
Kallman's syndrome is a disorder of hypogonadotropic hypogonadism, delayed puberty, and anosmia (the inability to smell).
Hypogonadotropic hypogonadism (HH) occurs when the body does not produce enough of two important hormones, luteinizing hormone (LH) and follicle stimulating hormone (FSH).
Anosmia, the inability to smell, was first described with hypogonadotropic hypogonadism in 1856, but it was not until 1944 that an instance of Kallman's reported the inheritance of the two symptoms together in three separate families.
Hence, the syndrome of hypogonadotropic hypogonadism and anosmia was named Kallman's syndrome (KS).
Type 1 diabetes (diabetes mellitus) occasionally has been associated with hypogonadism.
Most cases seem to be due to the hypogonadism of malnutrition and respond to improved control.
Some specific conditions associated with diabetes mellitus, such as hemachromatosis, and the Laurence-Moon Biedl, Alstrom, and Cushing syndromes, also typically produce hypogonadism.
In normal, young males with hypogonadism, sexual acts, fantasies, and desire are significantly diminished.
However, visual and possibly tactile stimulus-bound erections are not impaired in males with hypogonadism after infancy.
In girls, hypogonadism during childhood will result in lack of menstruation and breast development and short height.
If hypogonadism occurs after puberty, symptoms include loss of menstruation, low libido, hot flashes, and loss of body hair.
In boys, hypogonadism in childhood results in lack of muscle and beard development and growth problems.
If a brain tumor is present (central hypogonadism) there may be headaches or visual loss or symptoms of other hormonal deficiencies (such as hypothyroidism).
People with anorexia nervosa (excessive dieting to the point of starvation) also may have central hypogonadism.
Parents should consult a family physician or pediatrician if their child has any signs or symptoms of hypogonadism.
Establishing the cause of hypogonadism is an important first step to getting appropriate treatment.
Ethinyl estradiol, an estrogen derivative, is sometimes used for the treatment of hypogonadism.
Many forms of hypogonadism are potentially treatable and have a good prognosis.
Adolescents with hypogonadism may have problems fitting in socially due to delayed sexual development.
In girls with hypogonadism, complications include the social implication of failing to go through puberty with peers (if hypogonadism occurs before puberty).
A supportive family that understands the diagnosis of hypogonadism is important.
Support groups can help people with hypogonadism and related conditions cope with similar situations and challenges.
Kallman's syndrome-A disorder of hypogonadotropic hypogonadism, delayed puberty, and anosmia.
Male Hypogonadism: Basic, Clinical, and Therapeutic Principles.
"Panel: Here are Twenty-Five Recommendations on Hypogonadism."
"Effect of Male Hypogonadism on Bone Architecture: Tips."
"Guidelines Take New Look at Management of Hypogonadism in Men (Testosterone Replacement Options)."
"Hypogonadism." Medline Plus, November 14, 2002.
Genitalia (hypogonadism) that is smaller than normal is common.
Hypogonadism may be corrected at puberty with hormone replacement.
The word usage examples above have been gathered from various sources to reflect current and historial usage. They do not represent the opinions of YourDictionary.com.