生殖可能類宦官症候群
WordNet
- capable of reproducing
- a pattern of symptoms indicative of some disease
- a complex of concurrent things; "every word has a syndrome of meanings"
- a man who has been castrated and is incapable of reproduction; "eunuchs guarded the harem" (同)castrate
PrepTutorEJDIC
- (土地が)『肥沃(ひよく)な』,作物のよくできる / (動植物が)繁殖力のある / (種子などが)生育力のある;(卵が)受精した(fertilized) / (精神などが)創造力の豊かな
- (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
- 去勢された男,(特に昔東洋の宮廷やハレムなどに仕えた)宦官(かんがん)
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/03/30 15:34:48」(JST)
[Wiki en表示]
The fertile eunuch syndrome is a cause of hypogonadotropic hypogonadism caused by a lutheinizing hormone deficiency.[1] It is characterized by hypogonadism with spermatogenesis.[2] Pasqualini and Bur published the first case of eunuchoidism with preserved spermatogenesis in 1950 in la Revista de la Asociación Médica Argentina.[3]
The hypoandrogenism with spermatogenesis syndrome included: (a) eunuchoidism, (b) testis with normal spermatogenesis and full volume, with mature spermatozoids in a high proportion of seminiferous tubes and undifferentiated and immature Leydig cells (c) full functional compensation through the administration of chorionic gonadotropin hormone, while hCG is administered (d) total urinary gonadotrophins within normal limits (e) this definition implies the normal activity of the pituitary and the absence of congenital malformations in general. In describing five other similar cases in 1953, Mc Cullagh & al [4] [5] coined the term fertile eunuch introducing it in the English literature. Unfortunately, this term is incorrect and should not be employed. Indeed, these patients are not really eunuchs. Moreover, as it will be explained later, they are not usually fertile if not treated.
A first step in the understanding of the physiopathology of Pasqualini syndrome was the absence of Lutheinizing Hormone (LH) in plasma and urine of patients. The second breakthrough was the functional and genetic studies that validated the hypothesis of a functional deficit of LH in these men. Inactivating LH mutations will then also be described in some women. Different groups [6] [7] [8] [9] [10] demonstrated in these cases a LH with varying degrees of immunological activity but biologically inactive in most of the patients, due to one or more inactivating mutations in the LHB gene. Finally, the full comprehension of Pasqualini syndrome allowed to reverse the hypoandrogenic phenotype and to restore fertility in these patients through the use of chorionic gonadotropin and the modern in-vitro fertility techniques [11]
See also
- Hypogonadism
- Delayed puberty and infertility
- Hypothalamus, pituitary gland, and HPG axis
- GnRH and gonadotropins (FSH and LH)
- Sex hormones (androgens and estrogens)
- Kallmann syndrome and GnRH insensitivity
References
- ^ Valdes-Socin H, Rubio Almanza M, Tomé Fernández-Ladreda M, Debray FG, Bours V, Beckers A. Reproduction, Smell and Neurodevelopmental disorders: Genetic defects in different hypogonadotropic hypogonadal syndromes. Frontiers in Endocrinology 2014;jul 9;5:109. doi: 10.3389/fendo.2014.00109.
- ^ Valdes-Socin H Beckers A. Pasqualini’s syndrome: hypoandrogenism with spermatogenesis. MEDICINA (Buenos Aires) 2015; 75: 53-58.
- ^ Pasqualini RQ, Bur GE. Síndrome hipoandrogénico con gametogénesis conservada. Rev Asoc Méd Argent 1950; 64: 6.
- ^ McCullagh EP, Beck JC, Schaffenburg CA: A syndrome of eunuchoidism with spermatogenesis, normal urinary FSH and low or normal ICSH: (“Fertile eunuchs”). J Clin Endocrinol Metab 1953; 13: 49.
- ^ Pasqualini RQ: Hypoandrogenic syndrome with normal spermatogenesis. J Clin Endocrinol Metab 1953; 13: 128.
- ^ Weiss J, Axelrod L, Whitcomb RW, Harris PE, Crowley WF, Jameson JL. Hypogonadism caused by a single amino acid substitution in the beta subunit of luteinizing hormone N Engl J Med. 1992; 326(3):179-83.
- ^ Valdes-Socin H, Salvi R, Daly AF, Gaillard RC, Quatresooz P, Tebeu PM et al. Hypogonadism in a patient with a mutation in the luteinizing hormone beta-subunit gene. N Engl J Med. 2004; 351(25):2619-25.
- ^ Lofrano-Porto A, Barra GB, Giacomini LA, Nascimento PP, Latronico AC, Casulari LA et al. Luteinizing hormone mutation and hypogonadism in men and women. N Engl J Med 2007 357:897–904.
- ^ Achard C, Courtillot C, Lahuna O, Méduri G, Soufir JC, Liere P et al. Normal spermatogenesis in a man with mutant luteinizing hormone. N Engl J Med 2009 361:1856–1863.
- ^ Basciani S, Watanabe M, Mariani S, Passeri M, Persichetti A, Fiore D, Scotto d’Abusco A, Caprio M, Lenzi A, Fabbri A, Gnessi L. Hypogonadism in a Patient with Two Novel Mutations of the Luteinizing Hormone–Subunit Gene Expressed in a Compound Heterozygous Form J. Clin Endocrinol Metab 2012; 97: 3031–3038.
- ^ Valdes-Socin H, Salvi R, Thiry A, Daly AF, Pralong FP, Gaillard R et al. Testicular effects of isolated luteinizing hormone deficiency and reversal by long-term human chorionic gonadotropin treatment. J Clin Endocrinol Metab. 2009;94(1):3-4.
UpToDate Contents
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English Journal
- [Pasqualini's syndrome: hypoandrogenism with spermatogenesis].
- Valdés Socin H1, Beckers A.
- Medicina.Medicina (B Aires).2015;75(1):53-8.
- Pasqualini and Bur published the first case of eunuchoidism with preserved spermatogenesis in 1950 in Revista de la Asociación Médica Argentina. The hypoandrogenism with spermatogenesis syndrome included: (a) eunuchoidism, (b) testis with normal spermatogenesis and full volume, with mature spermat
- PMID 25637904
- Was Akhenaten really sick?
- Codaccioni JL1, Conte-Devolx B, Argémi B.
- Annales d'endocrinologie.Ann Endocrinol (Paris).2013 Jul;74(3):231-3. doi: 10.1016/j.ando.2013.04.003. Epub 2013 Jun 25.
- The depictions of Akhenaten have long interested medicine and above all endocrinology because of the eunuch gynoid morphology of this pharaoh. These depictions call to mind various diagnostic hypotheses that have been successively considered as endocrinology progresses, with emphasis on the three di
- PMID 23810106
- Fertile eunuch syndrome in association with biventricular noncompaction, bicuspid aortic valve, severe aortic stenosis, and talipes equinovarus.
- Ozcan KS1, Osmonov D, Altay S, Gungor B, Eren M.
- Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital.Tex Heart Inst J.2013;40(2):204-6.
- Noncompaction of the ventricular myocardium is a congenital cardiomyopathy characterized by prominent ventricular trabeculations and deep intertrabecular recesses. In most cases, noncompaction is an isolated disease confined to the left ventricular myocardium. Fertile eunuch syndrome is a hypogonado
- PMID 23678225
Japanese Journal
- Fertile Eunuch Syndrome with the Mutations (Trp8Arg and lle15Thr) in the β Subunit of Luteinizing Hormone
Related Links
- A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Fertile eunuch syndrome The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. ...
- Although the term “fertile eunuchism” has been considered a misnomer (the definition of eunichism includes azoospermia) it could conceviably refer to a condition of eunichism with preserved spermatogenesis (i.e. a post-puberal hypogonadism with Leydig cell deficiency (13, 12, 15, 14, 18, 20, 7).
- 黄体形成ホルモン（LH）単独欠損症（妊性宦官症候群[fertile eunuch syndrome]）は，男児におけるLH分泌のモノトロピックな欠失に起因する性腺機能低下症のまれな原因である；卵胞刺激ホルモン（FSH）の値は正常である。思春
★リンクテーブル★
[★]
- 英
- fertile eunuch syndrome
- 同
- パスカリーニ症候群 Pasqualini syndrome
[★]
- (土地が)肥えた、肥沃な、豊穣な(opp.sterile)。多産な。方策をもたらす
- [fig]豊かな、実り多い。想像力に富む、創意豊かな
- (生)繁殖力のある。(生)受精した(fertilized)
- 関
- fertility
[★]
- 去勢された男。[fig]いくじなしの/無能な男、ふぬけ。(医)宦官
[★]