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Psevdogermafroditizm davnij klinichnij termin yakim poznachayut stan za yakogo vtorinni statevi oznaki organizmu ne vidpovidayut pervinnim 1 2 Cej stan napriklad mozhe buti naslidkom deficitu 5 a reduktazi 3 chi sindromu nechutlivosti do androgeniv 4 5 i jogo ne varto plutati zi spravzhnoyu gonadalnoyu interseksnistyu spravzhnim germafroditizmom pri yakij v odnogo organizmu nayavni tkanini j yayechok i yayechnikiv Narazi cej termin ye zastarilim 6 a slovo germafrodit ne mozhe vzhivatisya stosovno lyudini 7 lishe stosovno tvarini abo roslini sho mayut yak cholovichi tak i zhinochi reproduktivni organi 8 PsevdogermafroditizmModel Hanna Gabi Odil Maye sindrom nechutlivosti do androgeniv Model Hanna Gabi Odil Maye sindrom nechutlivosti do androgeniv Inshi nazvi hibnij germafroditizmSpecialnist Ginekologiya endokrinologiyaKlasifikaciya ta zovnishni resursiDiseasesDB 14836 i 14839 Pseudohermaphroditism u VikishovishiU lyudej z cim stanom zovnishni statevi organi chasto mayut viglyad promizhnogo variantu mizh klitorom i penisom V inshih vipadkah zovnishni statevi organi mayut viglyad yakij vidpovidaye protilezhnij tkanini gonad Tomu vin mozhe ne viyavlyatisya do statevoyi zrilosti 9 Termin cholovichij psevdogermafroditizm zastosovuvavsya todi koli v osobi buli nayavni sim yaniki a termin zhinochij psevdogermafroditizm zastosovuvavsya pri nayavnosti yayechnikiv 10 ci stani takozh mozhna nazvati XY interseksnistyu ta XX interseksnistyu vidpovidno 11 12 Zmist 1 XX interseksnist 1 1 Prichini 1 2 Oznaki 2 XY interseksnist 2 1 Prichini 2 2 Oznaki 3 Genetika 4 Likuvannya 5 Terminologiya 6 Div takozh 7 Posilannya 8 PrimitkiXX interseksnist RedaguvatiPrichini Redaguvati Vinikaye yaksho pid chas pershogo trimestru vagitnosti na plid sho maye zhinochij genotip diyut veliki dozi androgeniv Ci androgeni mozhut mati yak zovnishnye tak i vnutrishnye pohodzhennya Nadmirna kilkist androgeniv zovnishnogo pohodzhennya mozhe vinikati vnaslidok prijomu matir yu anabolichnih steroyidiv nayavnosti v neyi androgen produkuyuchih puhlin nadnirnikiv chi yayechnikiv abo vrodzhenoyi giperplaziyi nadnirkovih zaloz Prichinoyu nadlishku vnutrishnih androgeniv mozhe buti vrodzhena giperplaziya nadnirkovih zaloz u samogo ploda Vrodzhena giperplaziya nadnirkovih zaloz vinikaye vnaslidok spadkovogo defektu odnogo z fermentiv neobhidnih dlya virobnictva kortizolu deficit 21 gidroksilazi deficit 11 beta gidroksilazi deficit 3 beta gidroksisteroyiddegidrogenazi vnaslidok chogo produkuyetsya nadmirna kilkist androgeniv dlya kompensaciyi nestachi kortizolu U nemovlyat zhinochoyi stati ce prizvodit do maskulinizaciyi z anomalnim rozvitkom statevih organiv todi yak u nemovlyat cholovichoyi stati do peredchasnogo statevogo rozvitku Krim togo prichinoyu mozhe buti deficit aromatazi placenti R450 sho prizvodit do nadlishkovogo produkuvannya androgeniv placentoyu 13 14 15 Oznaki Redaguvati U lyudej z cim stanom chasto sposterigayetsya zbilshennya rozmiriv klitora mozhe viglyadati yak penis prignichennya rozvitku grudej nadmirne ovolosinnya oblichchya i tila glibokij golos porushennya menstrualnogo ciklu 16 15 XY interseksnist RedaguvatiPrichini Redaguvati XY interseks osobi mozhut mati znizhenu produkciyu testosteronu vnaslidok porushennya aktivnosti chi gipoplaziyi klitin Lejdiga abo defektu receptora do lyuteyinizuvalnogo gormonu na nih porushennya biosintezu testosteronu pri defektah 20 22 desmolazi 3 beta gidroksisteroyiddegidrogenazi 17 20 gidroksilazi 17 beta gidroksisteroyiddegidrogenazi sho berut uchast u steroyidogenezi v nadnirnikah Chastoyu prichinoyu rozvitku cogo stanu ye nechutlivist do androgeniv Krim togo cej stan mozhe zumovlyuvatisya defektami rozvitku yayechok takimi yak XY disgeneziya gonad zmishanij disgenez gonad i sindromi rudimentarnogo yayechka 14 15 Okrim togo porushennya maskulinizaciyi mozhe viklikatisya deficitom 5 a reduktazi Cej ferment vidpovidaye za peretvorennya testosteronu v digidrotestosteron sho ye fiziologichno aktivnishim i ye neobhidnim dlya normalnogo rozvitku cholovichih zovnishnih statevih organiv plodu 17 Tomu lyudi z deficitom cogo fermentu narodzhuyutsya z genitaliyami duzhe podibnimi do zhinochih Ale pid chas statevogo dozrivannya riven cholovichih statevih gormoniv u cih osib pidvishuyetsya sho prizvodit do rozvitku vtorinnih statevih oznak roste m yazova masa pogliblyuyetsya golos pochinaye zbilshuvatisya yih klitoropodibnij penis pri comu ovolosinnya oblichchya v nih chasto ne virazhene Bagato z nih vihovuyutsya yak divchatka 18 19 Oznaki Redaguvati U XY interseks osib zazvichaj rozvivayutsya normalni yayechka ale maskulinizaciya sistemi volfovih protok ta zovnishnih statevih organiv ye nepovnoyu Chasto ci lyudi narodzhuyutsya z nezviznachenistyu genitalij yayechka mozhut buti zokrema vnutrishnocherevnimi chi tazovimi Mozhut rozvivatisya molochni zalozi mozhliva nayavnist klitora ta malorozvinenoyi vagini 16 15 20 21 22 Genetika RedaguvatiMutaciyi sho vplivayut na gen receptora androgeniv AR mozhut viklikati sindrom povnoyi abo chastkovoyi androgennoyi nechutlivosti Diferenciaciya plodu yak cholovichoyi stati vidbuvayetsya na shostomu chi somomu tizhni vagitnosti Rozvitok spryamovanij viznachalnim faktorom yayechok genom SRY oblastyu sho viznachaye stat na Y hromosomi Protyagom 9 13 tizhniv rozvitok statevih organiv cholovichoyi stati zalezhit vid peretvorennya testosteronu na bilsh potuzhnij androgen zavdyaki diyi 5 a reduktazi v cilovih tkaninah genitalij 23 Doslidzhennya na koshenyatah psevdogermafroditah pokazalo sho isnuye poyednannya psevdogermafroditizmu z vrodzhenimi porushennyami shlunkovo kishkovogo traktu ta sechostatevoyi sistemi 24 Likuvannya RedaguvatiInodi provodyat hirurgichne vtruchannya shob zminiti zovnishnij viglyad genitalij 25 26 Odnak u bagatoh vipadkah vidsutni chitki dokazi neobhidnosti operacij i voni mozhut sprijmatisya yak kalictvo 27 28 Terminologiya RedaguvatiVikoristannya termina psevdogermafroditizm mozhe buti problematichnim i zaraz vvazhayetsya zajvim 29 Termin psevdogermafroditizm buv zaprovadzhenij Edvinom Klebsom 1876 roku 30 Termin interseksualnist buv vvedenij Richardom Goldshmidtom u 1923 roci 31 Pediatrichne endokrinne tovaristvo Lousona Vilkinsa ta Yevropejske tovaristvo endokrinologiyi i pediatriyi prijnyali nomenklaturnu sistemu zasnovanu na porushennyah statevogo rozvitku DSD yaka ohoplyuye vrodzheni stani pri yakih rozvitok hromosomnoyi gonadnoyi abo anatomichnoyi stati ye netipovim 29 ale j sam DSD oskarzhuyetsya gromadskimi organizaciyami pravozahisnimi institutami ta naukovcyami U travni 2016 roku Interact Advocates for Intersex Youth en opublikuvav zayavu proti patologizuyuchoyi movi dlya opisu lyudej narodzhenih z interseksualnimi risami viznayuchi posilennya zagalnogo rozuminnya ta prijnyattya termina interseks 32 Div takozh RedaguvatiSpravzhnij germafroditizm Germafroditizm Interseksualnist Sindrom Morrisa Mizhnarodna asociaciya lesbijok geyiv biseksualiv transgenderiv ta interseksualivPosilannya Redaguvatihttps www britannica com science cleft palate https www sciencedirect com topics medicine and dentistry pseudohermaphroditismPrimitki Redaguvati Dorlands Medical Dictionary Arhiv originalu za 28 grudnya 2007 Procitovano 7 grudnya 2007 MESH Procitovano 7 grudnya 2007 5ARD is also known as 5 a reductase 2 deficiency because the 5 a reductase 2 gene is deficient Wilson JD Griffin JE Russell DW Oct 1993 Steroid 5 alpha reductase 2 deficiency Endocr Rev 14 5 577 93 PMID 8262007 doi 10 1210 er 14 5 577 In the 20 years since it was established that impairment of dihydrotestosterone formation is the cause of a rare form of human intersex a wealth of information has accumulated about the genetics endocrinology and variable phenotypic manifestations culminating in the cloning of cDNAs encoding two 5 alpha reductase genes and documentation that mutations in the steroid 5 alpha reductase 2 gene are the cause of 5 alpha reductase deficiency Perplexing and difficult problems remain unresolved e g whether the variability in manifestations is due to variable expressions of steroid 5 alpha reductase 1 or to effects of testosterone itself It is also imperative to establish whether defects in steroid 5 alpha reductase 2 perhaps in the heterozygous state are responsible for a portion of cases of sporadic hypospadias to determine whether 5 alpha reductase plays a role in progesterone action in women and to elucidate the relation between androgen action and gender role behavior Alias AG Dec 2004 A role for 5alpha reductase activity in the development of male homosexuality Ann N Y Acad Sci New York NY USA New York Academy of Sciences 1032 237 44 PMID 15677419 doi 10 1196 annals 1314 029 Higher body hair with lower mesmorphism ratings were observed in Caucasian homosexual men compared with the general male population reflecting elevated 5alpha reductase 5alphaR activity and higher dihydrotestosterone to testosterone DHT to T ratio in sharp contrast to 46 XY 5alphaR 2 deficiency subjects who are often born with ambiguous or female genitalia but tend to grow up to be muscular heterosexual men with very little body hair or beard One study also showed them scoring around dull normal IQs A greater prevalence of liberal body hair growth in men with higher IQs and or educational levels was also observed in several samples The exceptions to this statistical trend are too unsettling however Nevertheless the results of a number of published studies including one showing higher DHT to T ratio in homosexual men done with different objectives over a span of 80 years together strongly support these findings Furthermore in an animal model cognitive enhancing effects of 5alpha reduced androgen metabolites were recently demonstrated Dreger Alice D Chase Cheryl Sousa Aron Gruppuso Phillip A Frader Joel 18 serpnya 2005 Changing the Nomenclature Taxonomy for Intersex A Scientific and Clinical Rationale Journal of Pediatric Endocrinology and Metabolism Arhiv originalu za 20 grudnya 2016 Procitovano 27 lipnya 2016 Intersex MedlinePlus Medical Encyclopedia medlineplus gov angl Procitovano 12 bereznya 2021 Zucker Kenneth J Bradley Susan J Sullivan Claire B Lowry 1992 03 Gender Identity Disorder in Children Annual Review of Sex Research angl 3 1 s 73 120 ISSN 1053 2528 doi 10 1080 10532528 1992 10559876 Procitovano 12 bereznya 2021 Michiels I Peperstraete L De Wever I Gruwez J A 1984 07 Inguinal hernia repair leading to the diagnosis of internal male pseudohermaphroditism Acta Chirurgica Belgica 84 4 s 255 258 ISSN 0001 5458 PMID 6485686 Procitovano 24 serpnya 2020 Langman Jan Thomas Sadler 2006 Langman s medical embryology Hagerstown MD Lippincott Williams amp Wilkins ISBN 0 7817 9485 4 Intersex MedlinePlus Medical Encyclopedia medlineplus gov angl Procitovano 12 bereznya 2021 Soumya Ranjan Parida 9 sichnya 2015 Intersex Procitovano 13 bereznya 2021 Cleft palate pathology Encyclopedia Britannica angl Procitovano 4 veresnya 2020 a b MarikoTheonia K BoydBradley J QuadeChristopher P CrumMana M Parast in Diagnostic Gynecologic and Obstetric Pathology Third Edition 2018 a b v g Pseudohermaphroditism an overview ScienceDirect Topics www sciencedirect com Procitovano 4 veresnya 2020 a b Jones Richard E Lopez Kristin H 2014 Sexual Differentiation Human Reproductive Biology angl Elsevier s 87 102 ISBN 978 0 12 382184 3 doi 10 1016 b978 0 12 382184 3 00005 2 Donahoe Patricia K Schnitzer Jay J Pieretti Rafael 2006 Ambiguous Genitalia Pediatric Surgery angl Elsevier s 1911 1934 ISBN 978 0 323 02842 4 doi 10 1016 b978 0 323 02842 4 50124 8 Reference Genetics Home 5 alpha reductase deficiency Genetics Home Reference angl Procitovano 4 veresnya 2020 5 Alpha Reductase Deficiency Background Pathophysiology Epidemiology 3 travnya 2020 Procitovano 4 veresnya 2020 Lin J B Troyer D 2014 Nonneoplastic Diseases and Disorders of the Testis Pathobiology of Human Disease angl Elsevier s 2476 2479 ISBN 978 0 12 386457 4 doi 10 1016 b978 0 12 386456 7 05103 0 Rebar Robert W Erickson Gregory F 2012 Ovaries and Development Goldman s Cecil Medicine angl Elsevier s 1529 1534 ISBN 978 1 4377 1604 7 doi 10 1016 b978 1 4377 1604 7 00243 8 Male Pseudohermaphroditism an overview ScienceDirect Topics www sciencedirect com Procitovano 4 veresnya 2020 Lane Pascale H Mauer S Michael Steffes Michael W 1991 Renal Pathology in Insulin Dependent IDDM and Noninsulin Dependent Diabetes Mellitus NIDDM Nephrology Berlin Heidelberg Springer Berlin Heidelberg s 1342 1350 ISBN 978 3 540 70074 6 Vallefuoco Rosario Alleaume Charline Jardel Nicolas Maenhoudt Cindy Cordonnier Nathalie 2013 05 Type II atresia ani associated with rectovaginal fistula in a male pseudohermaphrodite kitten The Canadian Veterinary Journal La Revue Veterinaire Canadienne 54 5 s 475 478 ISSN 0008 5286 PMC 3624915 PMID 24155431 Procitovano 26 serpnya 2020 Piro C Asensio M Barcelo C Martin J A Chicaiza E 2004 07 Good results with Passerini s technique in severely masculinised female pseudohermaphroditism Cirugia Pediatrica Organo Oficial De La Sociedad Espanola De Cirugia Pediatrica 17 3 s 118 121 ISSN 0214 1221 PMID 15503946 Procitovano 24 serpnya 2020 Nihoul Fekete Claire Thibaud Elisabeth Lortat Jacob Stephen Josso Nathalie 2006 05 Long term surgical results and patient satisfaction with male pseudohermaphroditism or true hermaphroditism a cohort of 63 patients The Journal of Urology 175 5 s 1878 1884 ISSN 0022 5347 PMID 16600787 doi 10 1016 S0022 5347 05 00934 1 Procitovano 24 serpnya 2020 Zwischengeschlecht March 2014 IGM Historical Overview Hermaphrodites in the Developed World From Legal Self Determination to IGM Supplement 1 NGO Report to the 2nd 3rd and 4th Periodic Report of Switzerland on the Convention on the Rights of the Child CRC vid 2 s 49 62 Council of Europe Commissioner for Human Rights April 2015 Human rights and intersex people Issue Paper a b Lee Peter A Houk Christopher P Ahmed S Faisal Hughes Ieuan A International Consensus Conference on Intersex organized by the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology 2006 08 Consensus statement on management of intersex disorders International Consensus Conference on Intersex Pediatrics 118 2 s e488 500 ISSN 1098 4275 PMID 16882788 doi 10 1542 peds 2006 0738 Procitovano 24 serpnya 2020 Zucker KJ 1999 Intersexuality and gender identity differentiation Annual Review of Sex Research 10 1 69 PMID 10895247 doi 10 1080 10532528 1999 10559774 Goldschmidt R 1923 The Mechanism and Physiology of Sex Determination Methuen amp Co London interACT May 2016 interACT Statement on Intersex Terminology Interact Advocates for Intersex Youth Procitovano 30 travnya 2016 Cya stattya mistit pravopisni leksichni gramatichni stilistichni abo inshi movni pomilki yaki treba vipraviti Vi mozhete dopomogti vdoskonaliti cyu stattyu pogodivshi yiyi iz chinnimi movnimi standartami serpen 2020 Otrimano z https uk wikipedia org w index php title Psevdogermafroditizm amp oldid 40485791