WordNet

of first rank or importance or value; direct and immediate rather than secondary; "primary goals"; "a primary effect"; "primary sources"; "a primary interest"
a preliminary election where delegates or nominees are chosen (同)primary_election
one of the main flight feathers projecting along the outer edge of a birds wing (同)primary feather, primary quill
(astronomy) a celestial body (especially a star) relative to other objects in orbit around it
not derived from or reducible to something else; basic; "a primary instinct"
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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『第一の』,『主要な』 / 『初期の』,『初等の』,初級の / 『根本的な』,基本的な,本来の,直接的な / 《名詞の前にのみ用いて》(回路・コイル・巻き・電流などが)一次の / 原色(三原色の一つ) / 《米》=primary election

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/09/24 18:04:39」(JST)

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Hypergonadotropic hypogonadism (HH), also known as primary or peripheral/gonadal hypogonadism, is a condition which is characterized by hypogonadism due to an impaired response of the gonads to the gonadotropins, follicle-stimulating hormone (FSH) and luteinizing hormone (LH), and in turn a lack of sex steroid production and elevated gonadotropin levels (as an attempt of compensation by the body). HH may present as either congenital or acquired, but the majority of cases are of the former nature.^[1]^[2]

Causes

There are a multitude of different etiologies of HH. Congenital causes include the following:^[1]^[3]^[4]

Chromosomal abnormalities (resulting in gonadal dysgenesis) - Turner's syndrome, Klinefelter's syndrome, Swyer's syndrome, XX gonadal dysgenesis, and mosaicism.
Defects in the enzymes involved in the gonadal biosynthesis of the sex hormones - 17α-hydroxylase deficiency, 17,20-lyase deficiency, 17β-hydroxysteroid dehydrogenase III deficiency, and lipoid congenital adrenal hyperplasia.
Gonadotropin resistance (e.g., due to inactivating mutations in the gonadotropin receptors) - Leydig cell hypoplasia (or insensitivity to LH) in males, FSH insensitivity in females, and LH and FSH resistance due to mutations in the GNAS gene (termed pseudohypoparathyroidism type 1A).

Acquired causes (due to damage to or dysfunction of the gonads) include gonadal torsion, vanishing/anorchia, orchitis, premature ovarian failure, ovarian resistance syndrome, trauma, surgery, autoimmunity, chemotherapy, radiation, infections (e.g., sexually-transmitted diseases), toxins (e.g., endocrine disruptors), and drugs (e.g., antiandrogens, opioids, alcohol).^[1]^[3]^[4]

Symptoms

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

Treatment

Treatment of HH is usually with hormone replacement therapy, consisting of androgen and estrogen administration in males and females, respectively.^[3]

References

^ ^a ^b ^c John P. Mulhall (22 April 2011). Cancer and Sexual Health. Springer. pp. 207–208. ISBN 978-1-60761-915-4. Retrieved 10 June 2012.
^ Ramón Piñón (1 January 2002). Biology of Human Reproduction. University Science Books. p. 363. ISBN 978-1-891389-12-2. Retrieved 10 June 2012.
^ ^a ^b ^c Marschall Stevens Runge; Cam Patterson (20 June 2006). Principles of Molecular Medicine. Humana Press. p. 463. ISBN 978-1-58829-202-5. Retrieved 10 June 2012.
^ ^a ^b J. Larry Jameson (1998). Principles of Molecular Medicine. Humana Press. p. 601. ISBN 978-0-89603-529-4. Retrieved 10 June 2012.

UpToDate Contents

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1. 男性における原発性性腺機能低下の原因 causes of primary hypogonadism in males
2. 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
3. 原発性早期卵巣障害（早期卵巣機能不全）の病因と原因 pathogenesis and causes of spontaneous primary ovarian insufficiency premature ovarian failure
4. 性腺機能低下を呈する男性に対するテストステロン治療 testosterone treatment of male hypogonadism
5. 原発性早期卵巣障害（早期卵巣機能不全）のマネージメント management of spontaneous primary ovarian insufficiency premature ovarian failure

English Journal

Acromegaly and bone disease.

Anthony JR1, Ioachimescu AG.
Current opinion in endocrinology, diabetes, and obesity.Curr Opin Endocrinol Diabetes Obes.2014 Dec;21(6):476-82. doi: 10.1097/MED.0000000000000109.
PURPOSE OF REVIEW: To provide an update on current understanding of osteoporosis associated with acromegaly.RECENT FINDINGS: Patients with acromegaly have an increased risk of morphometric vertebral fractures. This seems to correlate with acromegaly activity and its duration, but it persists after b
PMID 25354045

The effect of testosterone on cardiovascular disease: a critical review of the literature.

Su JJ1, Park SK1, Hsieh TM2.
American journal of men's health.Am J Mens Health.2014 Nov;8(6):470-91. doi: 10.1177/1557988314522642. Epub 2014 Feb 20.
Cardiovascular disease is the leading cause of death in the United States. Testosterone is the principal male sex hormone and plays an important role in men's health and well-being. Historically, testosterone was believed to adversely affect cardiovascular function. However, contemporary literature
PMID 24563500

Factors affecting spermatogenesis upon gonadotropin-replacement therapy: a meta-analytic study.

Rastrelli G1, Corona G, Mannucci E, Maggi M.
Andrology.Andrology.2014 Nov;2(6):794-808. doi: 10.1111/andr.262. Epub 2014 Oct 1.
A meta-analysis was performed to systematically analyse the results of gonadotropin and GnRH therapy in inducing spermatogenesis in subjects with hypogonadotropic hypogonadism (HHG) and azoospermia. An extensive Medline and Embase search was performed including the following words: 'gonadotropins' o
PMID 25271205

Japanese Journal

A Case of Marinesco-Sjögren Syndrome: MRI Observations of Skeletal Muscles, Bone Metabolism, and Treatment with Testosterone and Risedronate

Fujitake Junko,Komatsu Yasato,Hataya Yuji,Nishikawa Atsuko,Eriguchi Makoto,Mizuta Haruo,Hayashi Michiyuki
Internal Medicine 50(2), 145-149, 2011
… Marinesco-Sjögren syndrome (MSS) is a rare autosomal recessive disorder characterized by cerebellar ataxia, congenital cataracts, mental retardation, primary hypogonadism, skeletal abnormalities and myopathy, and patients with MSS are considered to be at risk of falls and bone fractures. …
NAID 130000413214