Hypogonadism is decreased production of testosterone, sperm, or both in males.
Hypogonadism occurs when there is a problem in the testes or a problem in the pituitary gland or the hypothalamus (which signal the testes to produce testosterone and sperm).
Symptoms vary depending at which age the testosterone deficiency begins.
The diagnosis is based on an examination, blood tests, and sometimes an analysis of the chromosomes.
Treatment depends on the cause but may include hormone therapy.
The pituitary gland secretes the hormones follicle-stimulating hormone and luteinizing hormone, which are called gonadotropins. These gonadotropins stimulate the male sex organs to produce the male sex hormone testosterone and the testes to produce sperm. When testosterone is lacking, growth and sexual development may be slow, sperm production is low, and the penis may be small.
Hypogonadism can occur while a fetus is developing in the womb or can occur during early or later childhood.
There are 2 main types of hypogonadism:
Primary hypogonadism: The testes are underactive and do not produce enough testosterone.
Secondary hypogonadism: The hypothalamus or pituitary gland does not secrete the hormones that stimulate the testes.
Hypogonadism Msingi
Primary hypogonadism occurs when there is a problem in the testes.
Ugonjwa wa Klinefelter
Klinefelter syndrome is caused by a chromosome abnormality in which boys have 2 X chromosomes and 1 Y chromosome. Boys normally have 1 X and 1 Y. This syndrome is the most common cause of primary hypogonadism.
The testes are often small and firm and produce low amounts of testosterone. Boys tend to be tall with abnormally long arms and legs, may develop breast tissue (gynecomastia), and often have low muscle mass and less facial and body hair compared to other boys. Puberty (sexual maturity) may be absent, delayed, or incomplete.
The syndrome is usually first identified at puberty, when inadequate sexual development is noted, or later, when infertility is investigated.
pumbu kutoteremka kwenye korodani
In cryptorchidism (undescended testes), 1 or both of the testes remain in the abdomen. Usually the testes descend into the scrotum shortly before birth. At birth, about 3% of boys who are born at full term (9 months) have an undescended testis, whereas 30% of boys who are born prematurely have an undescended testis. In most of them, the testes spontaneously descend on their own within the first 4 months of life.
The cause of cryptorchidism is usually unknown, but it can occur in children who have other disorders such as Klinefelter syndrome or Kallman syndrome.
Undescended testes are treated with surgery within the first year of life.
Ugonjwa wa kutokuwa kwa korodani (bilateral anorchia)
In bilateral anorchia, the testes were likely present during early development in the womb but were absorbed by the body before or shortly after birth.
Without testes, boys cannot produce testosterone or sperm. Without testosterone or sperm, they cannot develop male secondary sexual characteristics (such as facial hair and muscle mass) and are infertile.
Kutokuwepo kwa seli za Leydig (Leydig cell aplasia)
Absence of Leydig cells (cells in the testes that normally produce testosterone) leads to the development of partially developed genitals or ambiguous genitals. Ambiguous genitals are not clearly male or female. These birth defects develop because not enough testosterone is produced to stimulate the fetus to develop normal male genitals.
Ugonjwa wa Noonan
Noonan syndrome is a genetic defect that causes small testes that do not produce enough testosterone. This syndrome is caused by abnormalities in certain genes that may be inherited, or it may occur spontaneously in a developing fetus.
Boys may have underdeveloped or undescended testes. Puberty may be delayed, and young men with Noonan syndrome may have low levels of testosterone and be infertile.
Hypogonadism ya Pili
Secondary hypogonadism occurs when there is a problem in the pituitary gland or the hypothalamus (the part of the brain that controls the pituitary gland) that prevents the pituitary gland from secreting the hormones that stimulate the testes. Some of the causes involve genetic disorders that cause many other abnormalities.
Ugonjwa wa Kallmann
Kallmann syndrome is a genetic disorder that causes delayed puberty and an impaired sense of smell (some have a normal sense of smell). Children who have this syndrome are lacking proper amounts of luteinizing hormone and follicle-stimulating hormone. Affected boys have an unusually small penis and undescended testes.
Panhypopituitarism
Panhypopituitarism is a disorder that occurs when the pituitary gland stops producing all hormones. This disorder can occur when the pituitary gland is damaged (such as by a tumor or an injury) or is underdeveloped or poorly formed. Infections and treatment of tumors also can cause panhypopituitarism.
Without pituitary hormones, children may have delayed growth and no sexual development when puberty is expected.
(See also Hypopituitarism.)
Upungufu wa homoni ya kusisimua iliyotengwa
Isolated luteinizing hormone deficiency occurs when only one pituitary hormone, luteinizing hormone, is missing. When luteinizing hormone is missing, the testes develop and produce sperm, because these functions are also controlled by follicle-stimulating hormone. However, the testes do not produce enough testosterone, so affected boys do not develop secondary sexual characteristics. Boys do continue to grow and may have abnormally long arms and legs.
Ugonjwa wa Prader-Willi
Prader-Willi syndrome is a genetic disorder that occurs because part of a chromosome is missing or is malfunctioning. The function of the reproductive organs is abnormally decreased, which limits growth and sexual development. Boys have undescended testes and an underdeveloped penis and scrotum.
Dalili za Hipogonadismu ya Kiume kwa Watoto
Symptoms of hypogonadism vary depending at which age the testosterone deficiency begins.
In a male fetus, testosterone deficiency before the 12th week of pregnancy causes incomplete development of the genitals. The urethra may open on the underside of the penis instead of at its end (hypospadias). If testosterone deficiency develops later in pregnancy, a male fetus may have an abnormally small penis or testes that do not descend into the scrotum.
A male newborn may have genitals that appear less masculine (undervirilized) or appear more female, sometimes referred to as ambiguous genitalia.
In male children, testosterone deficiency that occurs at the time when puberty is expected results in incomplete sexual development. An affected boy retains a high-pitched voice and has poor muscle development for his age. The penis, testes, and scrotum are underdeveloped. Pubic and underarm hair is sparse, and body hair does not exist. The breasts may enlarge (gynecomastia), and the arms and legs are abnormally long.
Utambuzi wa Ugonjwa wa Hipogonadismu ya Kiume kwa Watoto
Blood tests
Chromosome analysis
Doctors suspect the diagnosis of hypogonadism when a boy has developmental abnormalities or delayed puberty. Doctors examine the boy's penis and testes to see whether they are normally developed for his age.
To confirm the diagnosis, doctors do blood tests to measure the levels of testosterone, luteinizing hormone, and follicle-stimulating hormone. The levels of luteinizing hormone and follicle-stimulating hormone help doctors determine whether hypogonadism is primary or secondary. Doctors measure levels of other hormones as well.
Doctors may do a chromosome analysis, especially if they suspect Klinefelter syndrome. Chromosomes and genes can be evaluated by analyzing a sample of blood as well as cells from other parts of the body (see Testing for Chromosome and Gene Abnormalities).
Matibabu ya Hipogonadismu ya Kiume kwa Watoto
Sometimes surgery
Replacement of hormones
Various treatments are done depending on the cause of hypogonadism.
For cryptorchidism, doctors do surgery to move the undescended testes into the scrotum, which usually enables the testes to function normally. Surgical repair helps prevent future complications.
For secondary hypogonadism, doctors treat any underlying disorder of the pituitary gland or hypothalamus. Supplements of testosterone are given, and the dose is increased over a period of 18 to 24 months. Treatment is started at an age when puberty normally begins, often around 12 years of age.
For testosterone deficiency, as in secondary hypogonadism, doctors give adolescent boys injections of testosterone every 2 to 4 weeks, and the dose is increased over a period of 18 to 24 months. As boys get older, testosterone can also be delivered through a patch on the skin or as a gel.
Genetic disorders cannot be cured, but hormone therapy may help sexual characteristics develop.
