WordNet

incompetence of the gonads (especially in the male with low testosterone); results in deficient development of secondary sex characteristics and (in prepubertal males) a body with long legs and a short trunk

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/06/03 07:30:28」(JST)

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Hypogonadotropic 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.^[1]

Causes

The type of HH, based on its cause, may be classified as either primary or secondary. Primary HH, also called isolated HH, is responsible for only a small subset of cases of HH, and is characterized by an otherwise normal function and anatomy of the hypothalamus and anterior pituitary. It is caused by congenital syndromes such as Kallmann syndrome, CHARGE syndrome and gonadotropin-releasing hormone (GnRH) insensitivity. Secondary HH, also known as acquired or syndromic HH, is far more common than primary HH, and is responsible for most cases of the condition. It has a multitude of different causes, including brain or pituitary tumors, pituitary apoplexy, head trauma, ingestion of certain drugs, and certain systemic diseases and syndromes.^[1]

Primary and Secondary HH can also be attributed to a genetic trait inherited from the biologic parents. For example, the male mutations of the GnRH coding gene could result in HH. Hormone replacement can be used to initiate puberty and continue if the gene mutation occurs in the gene coding for the hormone. Chromosomal mutations tend to affect the androgen production rather than the HPG axis.

Symptoms

Examples of symptoms of hypogonadism include delayed, reduced, or absent puberty, low libido, and infertility.

Treatment

Treatment of HH may consist of administration of either a GnRH agonist or a gonadotropin formulation in the case of primary HH and treatment of the root cause (e.g., a tumor) of the symptoms in the case of secondary HH. Alternatively, hormone replacement therapy with androgens and estrogens in males and females, respectively, may be employed.

References

^ ^a ^b "Isolated Gonadotropin-Releasing Hormone (GnRH) Deficiency Overview - GeneReviews™ - NCBI Bookshelf".

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1. 先天性性腺刺激ホルモン放出ホルモン欠乏症（特発性低ゴナドトロピン性性腺機能低下） congenital gonadotropin releasing hormone deficiency idiopathic hypogonadotropic hypogonadism
2. 男性における続発性性腺機能低下の原因 causes of secondary hypogonadism in males
3. 性腺機能低下を呈する男性の臨床的特徴および診断 clinical features and diagnosis of male hypogonadism
4. 続発性無月経の病因、診断、および治療 etiology diagnosis and treatment of secondary amenorrhea
5. 続発性性腺機能低下を呈する男性における生殖能の誘導 induction of fertility in men with secondary hypogonadism

English Journal

Relation between BMD and biochemical, transfusion and endocrinological parameters in pediatric thalassemic patients.

Mohseni F1, Mohajeri-Tehrani MR, Larijani B, Hamidi Z.
Archives of osteoporosis.Arch Osteoporos.2014 Dec;9(1):174. doi: 10.1007/s11657-014-0174-3. Epub 2014 Mar 21.
Low BMDs, short stature, hypogonadism, subclinical hypothyroidism, and IFG are found in 3.3, 10, 33, 16.6, 6.6, and 26.6 % of 30 pediatric β thalassemia major patients, respectively. Age is related with low Z-scores. Short stature and hypogonadism patients were older. These patients' monitoring in
PMID 24652076

To treat or not to treat with testosterone replacement therapy: a contemporary review of management of late-onset hypogonadism and critical issues related to prostate cancer.

Kava BR.
Current urology reports.Curr Urol Rep.2014 Jul;15(7):422. doi: 10.1007/s11934-014-0422-5.
Over the last 10 years there has been a dramatic increase in the number of patients identified and treated with testosterone replacement therapy (TRT) for late-onset hypogonadism (LOH). By virtue of age, race, and family history, many of these patients are concurrently at risk for harboring indolen
PMID 24832199

MRI-based abnormalities in young adults at risk of adverse bone health due to childhood-onset metabolic & endocrine conditions.

McComb C1, Harpur A, Yacoubian C, Leddy C, Anderson G, Shepherd S, Perry C, Shaikh MG, Foster J, Ahmed SF.
Clinical endocrinology.Clin Endocrinol (Oxf).2014 Jun;80(6):811-7. doi: 10.1111/cen.12367. Epub 2013 Dec 12.
OBJECTIVE: Traditional methods of bone densitometry may not provide a comprehensive assessment of bone health. We aimed to assess bone micro-architecture and bone marrow adiposity (BMA) by MRI in adults with osteogenesis imperfecta (OI) and endocrinopathy including GH deficiency and/or hypogonadism.
PMID 24245820

Japanese Journal

臨床研究・症例報告先天性低ゴナドトロピン性性腺機能低下症の重度停留精巣に術前hCG療法が有効であった1例

三好達也,後藤正博,長谷川行洋
小児科臨床 68(2), 227-232, 2015-02
NAID 40020328306

Combined pituitary hormone deficiency with unique pituitary dysplasia and morning glory syndrome related to a heterozygous PROKR2 mutation

, , , , , , ,
Clinical Pediatric Endocrinology 24(1), 27-32, 2015
… No other mutation was present in 27 genes related to hypogonadotropic hypogonadism, pituitary hormone deficiency and optic nerve malformation. …
NAID 130004853608

2. 原発性無月経と生殖医療(クリニカルカンファレンス12(女性ヘルスケア・生殖)-原発性無月経の管理-,生涯研修プログラム,第66回日本産科婦人科学会学術講演会講演要旨)

丸山哲夫
日本産科婦人科學會雜誌 66(9), 2163-2166, 2014-09-01
NAID 110009844762

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リンク元	「続発性性腺機能低下症」「男性低性腺刺激ホルモン性性腺機能低下症」「低ゴナドトロピン性性腺機能低下症」「HHG」
拡張検索	「functional hypogonadotropic hypogonadism」
関連記事	「hypogonadotropic」