Synonyms for hypogonadotropic or Related words with hypogonadotropic
Examples of "hypogonadotropic"
The terminology used when describing cases of HH can vary. The term congenital
hypogonadism (CHH) is now often used. Other terms used include idiopathic / isolated
hypogonadism (IHH), normosmic
hypogonadism (nHH) or hypothalamic hypogonadism. The term HH can be used to cover all cases, including Kallmann syndrome.
Defects in the "GNRHR" are a cause of
Mutations in the "PROKR2" (also known as "KAL3") gene have been implicated in
hypogonadism and gynecomastia.
Mutations in the "PROK2" (also known as "KAL4") gene have been implicated in
hypogonadism and gynecomastia.
In 1944, he described a congenital endocrine condition (
hypogonadism with anosmia) that has come to be known as Kallmann's syndrome.
One of the biggest problems in the diagnosis of Kallmann syndrome and other forms of HH is the ability to distinguish between a normal constitutional delay of puberty and Kallmann syndrome (KS) or
Clinically, mutation results in the X-linked form of Kallmann syndrome. Individuals with Kallmann syndrome experience anosmia (lack of smell) and do not go through puberty (hypothalamic
Administration of luteinizing hormone (LH) (or human chorionic gonadotropin) and follicle-stimulating hormone (FSH) is very effective in the treatment of male infertility due to
hypogonadism. Although controversial, off-label clomiphene citrate, an antiestrogen, may also be effective by elevating gonadotropin levels.
Kallmann syndrome (KS) and other forms of
hypogonadism (HH) are classed as pituitary or endocrine disorders. While the end result is a failure of puberty and the development of secondary sexual characteristics, the underlying cause of the disorder is located between the two endocrine glands located within the brain.
Kallmann syndrome is a form of
hypogonadism (HH). Approximately 50% of HH cases occur with no sense of smell and are classified as Kallmann syndrome. Apart from the sense of smell there is no difference in the diagnosis or treatment between a case of HH or a case of Kallmann syndrome.
The fertile eunuch syndrome is a cause of
hypogonadism caused by a lutheinizing hormone deficiency. It is characterized by hypogonadism with spermatogenesis. Pasqualini and Bur published the first case of eunuchoidism with preserved spermatogenesis in 1950 in la Revista de la Asociación Médica Argentina.
Pretesticular azoospermia is characterized by inadequate stimulation of otherwise normal testicles and genital tract. Typically, follicle-stimulating hormone (FSH) levels are low (
) commensurate with inadequate stimulation of the testes to produce sperm. Examples include hypopituitarism (for various causes), hyperprolactinemia, and exogenous FSH suppression by testosterone. Chemotherapy may suppress spermatogenesis. Pretesticular azoospermia is seen in about 2% of azoospermia
hypogonadism (IHH), also called idiopathic or congenital
hypogonadism (CHH), as well as isolated or congenital gonadotropin-releasing hormone deficiency (IGD), is a condition that results in a small subset of cases of
hypogonadism (HH) due to deficiency in or insensitivity to gonadotropin-releasing hormone (GnRH) where the function and anatomy of the anterior pituitary is otherwise normal and secondary causes of HH are not present. It presents as hypogonadism (e.g., reduced or absent puberty (Ref.1), low libido, infertility, etc.) due to an impaired release of the gonadotropins, follicle-stimulating hormone (FSH) and luteinizing hormone (LH), and a resultant lack of sex steroid and peptides production by the gonads (Ref. 2 and Ref 3). In addition, anosmia (loss of the sense of smell) occurs in instances of IHH that are the result of Kallmann syndrome, which is responsible for approximately 50% of all cases of the condition. Other causes of IHH include GnRH insensitivity, which is the second most common cause of IHH and is thought to be responsible for up to 20% of cases, and a minority (less than 5-10%) due to inactivating mutations in a variety of other genes which positively regulate GnRH secretion such as "CHD7", "KISS1R", and "TACR3". The causes of approximately 25% of all cases of IHH are still unknown.
Reversal cases have been seen in cases of both Kallmann syndrome and congenital
hypogonadism but appear to be less common in cases of Kallmann syndrome where the sense of smell is also affected. A paper published in 2016 agreed with the theory that there is a strong environmental or epigenetic link to the reversal cases. The precise mechanism of reversal is unclear and is an area of active research.
The prevalence of idiopathic
hypogonadism (IHH) and Kallmann syndrome (KS) has been estimated to be in the region of 1 in 10,000 male births. This figure comes from a 1973 study of French Foreign Legion conscripts A more recent paper published in 2011 gave the incidence in the Finnish population at 1 in 48,000, with a sex distinction of 1 in 30,000 for males and 1 in 125,000 for females.
Treatment may consist of hormone replacement therapy with androgens in either sex. Alternatively, gonadotropin-releasing hormone (GnRH)/GnRH agonists or gonadotropins may be given (in the case of "
" hypoandrogenism). The Food and Drug Administration (FDA) stated in 2015 that neither the benefits nor the safety of testosterone have been established for low testosterone levels due to aging. The FDA has required that testosterone pharmaceutical labels include warning information about the possibility of an increased risk of heart attacks and stroke.
hypogonadism (HH), also known as secondary or central hypogonadism, as well as gonadotropin-releasing hormone deficiency or gonadotropin deficiency (GD), is a condition which is characterized by hypogonadism due to an impaired secretion of gonadotropins, including follicle-stimulating hormone (FSH) and luteinizing hormone (LH), by the pituitary gland in the brain, and in turn decreased gonadotropin levels and a resultant lack of sex steroid production.
Affected males may also lack male sex hormones, which leads to underdeveloped reproductive tissues, undescended testicles (cryptorchidism), delayed puberty, and an inability to father children (infertility). These characteristics are known as
hypogonadism. Females are rarely affected by this disorder, but a few cases have been reported of adrenal insufficiency or a lack of female sex hormones, resulting in underdeveloped reproductive tissues, delayed puberty, and an absence of menstruation.
Kallmann syndrome and
hypogonadism do not exist as distinct conditions. Each case can show a different range of symptoms and a different severity of symptoms. Even family members will not always show the same degree of symptoms. Cases of KS/HH can be separated into different categories depending on the gene mutation(s) involved. Severity can range from total absence of puberty with anosmia to slightly delayed puberty with or without anosmia.
This gene encodes a protein that lacks the normal DNA-binding domain contained in other nuclear receptors. The encoded protein acts as a dominant-negative regulator of transcription of other nuclear receptors including steroidogenic factor 1. This protein also functions as an anti-testis gene by acting antagonistically to SRY. Mutations in this gene result in both X-linked congenital adrenal hypoplasia and
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