高ゴナドトロピン性性腺機能低下症
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
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/09/05 21:58:45」(JST)
[Wiki en表示]
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]
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Contents
- 1 Causes
- 2 Symptoms
- 3 Treatment
- 4 See also
- 5 References
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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
See also: hypogonadism
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]
See also
- Hypogonadism
- Hypogonadotropic hypogonadism
- Delayed puberty and infertility
- Hypothalamus, pituitary gland, and HPG axis
- Gonads (testicles and ovaries)
- GnRH and gonadotropins (FSH and LH)
- Sex hormones (androgens and estrogens)
References
- ^ a b c John P. Mulhall (22 April 2011). Cancer and Sexual Health. Springer. pp. 207-208. ISBN 978-1-60761-915-4. http://books.google.com/books?id=GpIadil3YsQC&pg=PA207. Retrieved 10 June 2012.
- ^ Ramón Piñón (1 January 2002). Biology of Human Reproduction. University Science Books. p. 363. ISBN 978-1-891389-12-2. http://books.google.com/books?id=2iz-zfSh56AC&pg=PA363. 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. http://books.google.com/books?id=j-_rAKRf3WwC&pg=PA463. Retrieved 10 June 2012.
- ^ a b J. Larry Jameson (1998). Principles of Molecular Medicine. Humana Press. p. 601. ISBN 978-0-89603-529-4. http://books.google.com/books?id=u8ivGiRBkokC&pg=PA601. Retrieved 10 June 2012.
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Endocrine pathology: endocrine diseases (E00–E35, 240–259)
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Pancreas/
glucose
metabolism |
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Hypofunction
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Diabetes mellitus
types: (type 1, type 2, MODY 1 2 3 4 5 6) · complications (coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy, cardiomyopathy)
insulin receptor (Rabson–Mendenhall syndrome) · Insulin resistance
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Hyperfunction
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Hypoglycemia · beta cell (Hyperinsulinism) · G cell (Zollinger–Ellison syndrome)
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Hypothalamic/
pituitary axes |
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Hypothalamus
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gonadotropin (Kallmann syndrome, Adiposogenital dystrophy) · CRH (Tertiary adrenal insufficiency) · vasopressin (Neurogenic diabetes insipidus) · general (Hypothalamic hamartoma)
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Pituitary
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Hyperpituitarism
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anterior (Acromegaly, Hyperprolactinaemia, Pituitary ACTH hypersecretion) · posterior (SIADH) · general (Nelson's syndrome)
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Hypopituitarism
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anterior (Kallmann syndrome, Growth hormone deficiency, ACTH deficiency/Secondary adrenal insufficiency, GnRH insensitivity, FSH insensitivity, LH/hCG insensitivity) · posterior (Neurogenic diabetes insipidus) · general (Empty sella syndrome, Pituitary apoplexy, Sheehan's syndrome, Lymphocytic hypophysitis)
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Thyroid
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Hypothyroidism
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Iodine deficiency · Cretinism (Congenital hypothyroidism) · Myxedema · Euthyroid sick syndrome
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Hyperthyroidism
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Hyperthyroxinemia (Thyroid hormone resistance, Familial dysalbuminemic hyperthyroxinemia) · Hashitoxicosis · Thyrotoxicosis factitia · Graves' disease
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Thyroiditis
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Acute infectious · Subacute (De Quervain's, Subacute lymphocytic) · Autoimmune/chronic (Hashimoto's, Postpartum, Riedel's)
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Goitre
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Endemic goitre · Toxic nodular goitre · Toxic multinodular goiter
Thyroid nodule
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Parathyroid
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Hypoparathyroidism
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Hypoparathyroidism · Pseudohypoparathyroidism · Pseudopseudohypoparathyroidism
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Hyperparathyroidism
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Primary · Secondary · Tertiary · Osteitis fibrosa cystica
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Adrenal
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Hyperfunction
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aldosterone: Hyperaldosteronism/Primary aldosteronism (Conn syndrome, Bartter syndrome, Glucocorticoid remediable aldosteronism) · AME · Liddle's syndrome · 17α CAH
cortisol: Cushing's syndrome (Pseudo-Cushing's syndrome)
sex hormones: 21α CAH · 11β CAH
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Hypofunction/
Adrenal insufficiency
(Addison's, WF) |
aldosterone: Hypoaldosteronism (21α CAH, 11β CAH)
cortisol: CAH (Lipoid, 3β, 11β, 17α, 21α)
sex hormones: 17α CAH
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Gonads
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ovarian: Polycystic ovary syndrome · Premature ovarian failure
testicular: enzymatic (5α-reductase deficiency, 17β-hydroxysteroid dehydrogenase deficiency, aromatase excess syndrome) · Androgen receptor (Androgen insensitivity syndrome)
general: Hypogonadism (Delayed puberty) · Hypergonadism (Precocious puberty) · Hypoandrogenism · Hypoestrogenism · Hyperandrogenism · Hyperestrogenism
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| Height |
Gigantism · Dwarfism/Short stature (Laron syndrome, Psychosocial)
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| Multiple |
Autoimmune polyendocrine syndrome (APS1, APS2) · Carcinoid syndrome · Multiple endocrine neoplasia (1, 2A, 2B) · Progeria (Werner syndrome, Acrogeria, Metageria) · Woodhouse-Sakati syndrome
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noco(d)/cong/tumr, sysi/epon
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proc, drug (A10/H1/H2/H3/H5)
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UpToDate Contents
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English Journal
- Differential effects of strength training and testosterone treatment on soluble CD36 in aging men: Possible relation to changes in body composition.
- Glintborg D1, Christensen LL1, Kvorning T2, Larsen R3, Højlund K1, Brixen K1, Hougaard DM4, Handberg A5,6, Andersen M1.
- Scandinavian journal of clinical and laboratory investigation.Scand J Clin Lab Invest.2015 Dec;75(8):659-66. Epub 2015 Aug 12.
- PURPOSE: We measured soluble CD36 (sCD36) and body composition to determine the effects of testosterone treatment (TT) and/or strength training (ST) on cardiovascular risk in men with low normal testosterone levels.METHODS: Double-blinded, placebo-controlled study in 54 men aged 60-78 years with bio
- PMID 26264425
- 10q26.1 Microdeletion: Redefining the critical regions for microcephaly and genital anomalies.
- Choucair N1,2,3, Abou Ghoch J1, Fawaz A4, Mégarbané A1,5, Chouery E1.
- American journal of medical genetics. Part A.Am J Med Genet A.2015 Nov;167(11):2707-13. doi: 10.1002/ajmg.a.37211. Epub 2015 Jun 26.
- Distal 10q deletion syndrome is a well-characterized chromosomal disorder consisting of neurodevelopmental impairment, facial dysmorphism, cardiac malformations, genital and urinary tract defects, as well as digital anomalies. Patients with interstitial deletions involving band 10q26.1 present a phe
- PMID 26114870
- A girl with incomplete Prader-Willi syndrome and negative MS-PCR, found to have mosaic maternal UPD-15 at SNP array.
- Morandi A1, Bonnefond A2,3, Lobbens S2,3, Carotenuto M4, Del Giudice EM5, Froguel P2,6, Maffeis C1.
- American journal of medical genetics. Part A.Am J Med Genet A.2015 Nov;167(11):2720-6. doi: 10.1002/ajmg.a.37222. Epub 2015 Jun 24.
- The Prader-Willi syndrome (PWS) is caused by lack of expression of paternal allele of the 15q11.2-q13 region, due to deletions at paternal 15q11.2-q13 (<70%), maternal uniparental disomy of chromosome 15 (mat-UPD 15) (30%) or imprinting defects (1%). Hyperphagia, intellectual disabilities/behavio
- PMID 26109092
Japanese Journal
- Hypergonadotropic hypogonadism and hypersegmented neutrophils in a patient with ataxia-telangiectasia-like disorder: Potential diagnostic clues?
- Yoshida Takeshi,Awaya Tomonari,Shibata Minoru,Kato Takeo,Numabe Hironao,Kobayashi Junya,Komatsu Kenshi,Heike Toshio
- American journal of medical genetics. Part A 164(7), 1830-1834, 2014-07
- … We report a female ATLD patient presenting with hypergonadotropic hypogonadism and hypersegmented neutrophils, previously undescribed features in this disorder, and potential diagnostic clues to differentiate ATLD from other conditions. …
- NAID 120005456013
- Plasma kisspeptin levels in male cases with hypogonadism
- Kotani Masato,Katagiri Fumihiko,Hirai Tsuyoshi,Kagawa Jiro
- Endocrine Journal 61(11), 1137-1140, 2014
- … In this study, we measured plasma kisspeptin levels in four male cases with hypogonadism and seven normal male controls using enzyme immunoassay (EIA) to elucidate the clinical implications of kisspeptin levels in male hypogonadism. …
- NAID 130004677956
- Achievement of pregnancies in women with primary ovarian insufficiency using close monitoring of follicle development: case reports
- Maruyama Tetsuo,Miyazaki Kaoru,Uchida Hiroshi,Uchida Sayaka,Masuda Hirotaka,Yoshimura Yasunori
- Endocrine Journal 60(6), 791-797, 2013
- … Women with primary ovarian insufficiency (POI)/premature ovarian failure exhibit hypergonadotropic hypogonadism due to follicle dysfunction and depletion before the age of 40 years. …
- NAID 130004443899
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