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Unul din 5000, adica 99,98% sunt bebelusi nascuti normal, care se incadreaza la nastere intr-unul din sexul

feminin sau masculin. In Alaska numarul de bebelusi hermafroditi este de 17 la 5000, in timp ce de exemplu in Noua Zeelanda, acesta este cu mult mai mic de 1 la 25.000 bebelusi. Corectia chirurgicala se aplica la un bebelus din 1000 sau 2000 hermafroditi, deci se intervine foarte putin asupra "destinului" care nu este al naturii, ci al cromozomului Y. Gena din cromozomul Y, numita SRY codifica factorul determinant testicular. Aici se afla interventia nociva a omului despartit de Dumnezeu, care prin greseli imorale al planului propriu de "dezvoltare", altereaza perfectiunea licentei lui Dumnezeu. Cei 0,02% de bebelusi nefericiti trebuiesc acceptati de societate asa cum sunt sau asa cum hotaraste medicul specialist, ca este cel mai bine pentru fiecare caz medical in parte. Dar, ce te faci cu droaia "facaturilor" de transexuali, care apar ca ciupercile dupa ploaie si care isi fac scut propriilor desantari, tocmai acesti bebelusi nevinovati, victime ale degenerarii umane.
Unul din 5.000 de nou-nascuti, la nivel mondial, vine pe lume cu sex care nu poate fi determinat(hermafrodit). De multe ori, bisturiul nu poate rezolva problema hermafroditilor, transformandu-i in fiinte normale. Tocmai de aceea, in Australia, s-a decis la nivel statal recunoasterea celui de-al treilea pol sexual. In aceasta tara a fost votata o lege care permite aparitia in cartea de identitate, la sectiunea "sex", a sintagmei "nedeterminat". De-acum, nu mai este nevoie ca bebelusii hermafroditi sa fie suspusi unei operatii pentru a li se schimba unul dintre "darurile" oferite de natura, noteaza Deutsche Welle. Astfel, parintii trebuie sa constientizeze ca extirparea unui organ nu le garanteaza si un copil sanatos psihic. Avand aceasta noua lege la indemana, multi parinti ar trebui sa inteleaga ca micutii lor vor avea o viata sociala diferita decat cea considerata pana acum normala. In aceasta categorie vor fi inclusi si transsexuali, dar dupa o expertiza medicala adecvata. Aceasta lege a fost necesara dintr-un motiv destul de simplu: la nastere, copiii pot fi supusi operatiei, dar parintii trebuie sa stie ca de-acum au o alternativa. Cum un nou-nascut nu poate alege la acel moment din ce categorie sexuala vrea sa faca parte, parintele e singurul care poate spune daca isi doreste baiat sau fata. Plus ca operatia nu garanteaza transformarea micului hermafrodit intr-un om normal, implicatiile hormonale fiind mult mai profunde decat crede parintele. Organizatiile umanitare de profil fac de mult timp presiuni la nivel international pentru votarea unei astfel de legi, considerand ca aceste operatii facute la nastere reprezinta o violare crasa a drepturilor omului. De asemenea, statisticienii au stabilit la nivel global ca astfel de interventii lasa traume deosebit de grave asupra adultului nascut hermafrodit. Pe de alta parte, medicii se apara spunand ca in ultimii 15 ani, metodele chirurgicale au fost completate de o terapie hormonala adecvata. Nu se mai merge doar pe extirparea agresiva a unui organ, ci pe o intreaga schema de tratament, care include si terapii hormonale.

Hermafroditismul sau intersexualitatea este o tulburare de dezvoltare sexuala caracterizata prin existenta simultana a ambelor aparate reproducatoare; acestea pot fi prezente in totalitate sau partial, in diverse stadii de evolutie. Cuvantul "hermafrodit" s-a format din doua nume proprii grecesti: "Herma" - apartinand unei zeite de sex feminin si "Phrodite" numele unui zeu grec, barbat. Aceasta afectiune nefireasca si incomoda pentru orice individ sta la baza excluderii sociale a celor care o manifesta.

Se considera ca hermafroditismul apare ca o consecinta a secretiei excesive de hormoni masculini sau feminini produsi in organelor sexuale. organismul unei persoane, fapt ce determina o dezvoltare ambigua a

Denumiri alternative: pseudo-hermafroditism, hermafrodit, intersexualitate.

Cuprins articol
1. 2. 3. 4. 5. 6. 7.
Generalitati Cauze, incidenta, factori de risc Simptome Clasificare Diagnostic, investigatii si examinarea Tratament Cresterea unui copil

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Cauze, incidenta, factori de risc


Sus

Cauzele reale de hermafroditism implica prezenta ambelor organe sexuale - ovariene si testiculare. Din punct de vedere medical hermafroditismul presupune: ambiguitatea organelor externe; - nepotrivirea organelor genitale fata de predominanta genetica a unei persoane (de exemplu aparat reproducator feminin la un genotip masculin). Urmatoarele situatii pot determina ambiguitatea genitala si hermafroditismul: - hiperplazie congenitala suprarenala; expunerea fatului la hormoni sexuali; sindromul de feminizare testiculara; - disgenezie gonadala XY; - agenezie gonadala XY; - aberatii cromozomiale: criptoftalmia, Smith-Lemli-Opitz, sindromul Wolf-Hirschhorn (SWH) sau 4p, sindromul 13q.

Simptome
Sus

In functie de forma si gradul de dezvoltare al organelor sexuale, tulburarile specifice intersexualitatii pot fi detectate inca de la nastere. Cele mai comune simptome sunt: ambiguitatea genitala la nastere; micropenis; clitoromegalie (dimensiuni anormale ale clitorisului); dezvoltare partiala a labiilor; - testicule necoborate (care se pot dovedi a fi ovare) la baieti; - masa labiala sau inghinala suplimentara (care ar putea fi, de fapt, testicule) la fete; - hipospadias (deschiderea penisului in alta parte decat in partea superioara la barbati, la femei - uretra se deschide in vagin). Anomalii electrolitice

Intarzierea - Evolutii neasteptate in perioada pubertatii.

sau

absenta pubertatii;

Clasificare
Sus

In

principiu,

hermafroditismul

este

de

tipuri:

1. De tip XX Se manifesta la persoanele care sunt de sex feminin din punct de vedere genetic, dar organele sexuale externe au aspectul sistemului reproducator masculin (clitorisul se extinde si ofera senzatia unui penis). Aceasta anomalie se poate declansa (printre altele) din cauza expunerii in perioada de sarcina la o cantitate ridicata de hormoni masculini. Aceasta tulburare este denumita Virilism, la pubertate femeia poate manifesta caracteristici masculine (mustati, barba). 2. De tip XY Se manifesta la persoane de sex masculin din punct de vedere genetic dar organele genitale externe sunt de tip feminin. Are loc un proces denumit antivirilism care contribuie la manifestarea unor trasaturi feminine ale persoanei. 3. Hermafroditism adevarat sau gonadic In astfel de cazuri, indivizii detin ambele sisteme de reproducere (masculin si feminin), dar aspectul extern al organelor sexuale nu poate fi stabilit. Aceste cazuri sunt extrem de rare. 4. Hermafroditismul ca si tulburare genetica Acest tip este specific mai ales persoanelor care sufera de tulburari genetice precum: sindromul Down sau sindromul Edward. Se caracterizeaza prin prezenta sau absenta unuia dintre cromozomii X sau Y, in afara celor 23 de perechi de cromozomi specifici, rezultand diverse anomalii sexuale si comportamentale. Testosteronul joaca un rol cheie in determinarea naturii acestei afectiuni.

Diagnostic, investigatii si examinarea


Sus

Testul frecvent utilizat pentru diagnosticarea unei tulburari intersexuale este analiza cromozomiala. Alte investigatii pot fi cele hormonale, testarea electrolitilor si a moleculelor specifice. Examinarea clinica prin care se verifica absenta sau prezenta vaginului sau colului uterin se va face prin intermediul unui examen endoscopic. Ecografia si examenul de tip RMN vor confirma sau infirma prezenta si tipul organelor interne sexuale.

Tratament
Sus

Nu exista tratament complet pentru aceasta tulburare. Tulburarile intersexuale sunt afectiuni complexe care necesita tratament de lunga durata. Factorii care trebuie luati in considerare inainte de a decide interventia chirurgicala sau atribuirea unui gen sexual sunt: diagnosticarea corecta, investigatii cromozomiale si hormonale, testari psihologice si analiza comportamentala.

Medicii specialisti recomanda amanarea interventiei chirurgicale daca viata pacientului nu este pusa in pericol de existenta celor doua aparate de reproducere. Acestia considera ca pacientul trebuie sa decida singur care este aparatul reproducator care il reprezinta. Totodata, se recomanda ca pacientii cu hermafroditism sa beneficieze de terapie si suport, sa faca parte din grupuri de sprijin compuse din persoane cu aceeasi problema.

Cresterea unui copil


Sus

Cateva sfaturi pe care puteti sa le urmati cand cresteti un copil cu o asemenea tulburare: copiii hermafroditi trebuie sustinuti si tratati la fel ca si ceilalti; adevarul despre trasaturile sexuale nu trebuie ascuns; - accesibilitate la psihoterapeuti, preoti sau alti specialisti care pot ajuta la stabilirea unui confort psihic; - pot fi incurajati sa se inscrie in grupuri online de suport pentru a fii in contact cu cei care sufera de aceeasi tulburare; - parintii pot avea ei insisi nevoie de specialisti pentru a stii cum sa-i incurajeze si ce atitudine sa adopte; - incurajarea copilului pentru a alege un gen de apartenenta pe care sa-l poata identifica odata cu varsta; - interventia chirurgicala se va face doar la varsta adulta, cu acceptul persoanei care sufera de aceasta afectiune, pentru a nu produce prejudicii psihologice. Hermafroditii sau intersexualii traiesc deseori sentimente de confuzie, furie si tristete, se simt exclusi social si inadaptati. Cu toate acestea, uman nu se diferentiaza cu nimic de ceilalti deoarece au emotii, dorinte, motivatii, vise etc. Aceste persoane merita sa fie tratate si privite ca persoane normale!

Intersexualitate
Anomalie caracterizata prin prezenta, la acelasi individ, a tesutului ovarian si a tesutului testicular. Intersexualitatea este o afectiune congenitala exceptionala consecutiva unei anomalii a embriogenezei. Aceasta definitie grupeaza subiectii al caror aspect exterior poate fi foarte diferit: pur feminin, ambiguu sau pur masculin. Tratament - Atitudinea terapeutica da mai intai de toate posibilitatea de a alege, de acord cu parintii, sexul cel mai bine adaptat pentru fiecare caz, dupa aspectul fizic exterior si starea organelor interne. Daca diagnosticul nu a putut fi stabilit la nastere se ia in calcul educatia pe care copilul in cauza a primit-o deja. In continuare, este vorba de consolidarea sexului ales prin ablatie chirurgicala a organelor neadecvate si prin aport de hormoni daca acesta este necesar.

Hermafroditism
Anomalie caracterizata prin prezenta, la acelasi individ, a tesutului ovarian si a tesutului testicular. Un hermafrodism este o afectiune congenitala exceptionala consecutiva

unei anomalii a embriogenezei. Aceasta definitie grupeaza subiectii al caror aspect exterior poate fi foarte diferit: pur feminin, ambiguu sau pur masculin. Tratament - Atitudinea terapeutica da mai intai de toate posibilitatea de a alege, de acord cu parintii, sexul cel mai bine adaptat pentru fiecare caz, dupa aspectul fizic exterior si starea organelor interne. Daca diagnosticul nu a putut fi stabilit la nastere se ia in calcul educatia pe care copilul in cauza a primit-o deja. In continuare, este vorba de consolidarea sexului ales prin ablatie chirurgicala a organelor neadecvate si prin aport de hormoni daca acesta este necesar.
Hermafrodismul sau intersexualitatea denumeste un grup de conditii medicale in care exista o discrepanta intre organele genitale externe si organele genitale interne (testicule sau ovare). In prezent termenul mai vechi de hermafrodism a fost inlocuit cu cel de intersexualitate de catre medici, pacienti dar si familiile acestora, intrucat se considera ca cel dintai poarta o incarcatura confuza si insensibila la adresa acestei conditii medicale. Totodata, in prezent aceste anomalii sunt tot mai des supranumite tulburari de diferentiere sexuala.

CAUZELE INTERSEXUALITATII (HERMAFRODISM)


In functie de manifestarile sale, hermafrodismul cuprinde 4 tipuri majore, cauzele acestora variind de la caz la caz.

1. Intersexualitate de tip 46 XX
Intersexualitatea de tip 46 XX este caracterizata de prezenta in organismul pacientului a cromozomilor femeiesti, a tesutului ovarian, organele sexuale externe luand aspectul organelor de reproducere tipic barbatesti. Cauza care sta la baza acestei anomalii este expunerea organismului mamei la o cantitate prea mare de hormoni masculini in timpul sarcinii, fapt ce va rezulta in fuzionarea labiilor si marirea excesiva a clitorisului, acesta semanand foarte mult cu un penis. De cele mai multe ori pacientul diagnosticat cu intersexualitate de tip 46 XX detine uter si

tuburi falopiene dezvoltate normal, motiv pentru care aceasta conditie poarta denumirea de virilizare sau pseudohermafrodism. Alte cauze posibile ale intersexualitatii de tip 46 XX sunt:

hiperplazie adrenala congenitala; urmarea unui tratament pe baza de hormoni masculini (testosteron) in timpul sarcinii;

tumori ale organelor care produc hormoni masculini (testosteron), precum tumori ovariene. Daca nu exista alta cauza care duce la intersexualitate de tip 46 XX, mamele care dau nastere unui copil care se confrunta cu acest diagnostic trebuie sa efetueze teste amanuntite; deficienta de aromataza, caz in care aceasta conditie medicala nu va putea fi diagnosticata decat in momentul in care pacientul ajunge la varsta adolescentei. Aromataza este o enzima care transforma hormanii barbatesti in hormoni femeiesti. Activitatea crescuta a aromatazei duce la prezenta, in organism, a unei cantitati

mari de hormoni femeiesti (estrogen), insa deficienta acestei enzime duce la intersexualitate de tip 46 XX. La pubertate, organismele cu particulatitatea cromozomiala XX (fete) incep sa dezvolte trasaturi barbatesti.

2. Intersexualitate de tip XY
Intersexualitatea de tip XY se manifesta prin prezenta in organism a cromozomilor masculini, insa organele genitale masculine externe sunt incomplet dezvoltate, dezvoltate ambiguu sau de tip femeiesc.in structura interna testiculele pot fi dezvoltate normal, cu malformatii sau absente, motiv pentru care aceasta conditie medicala este denumita si subvirilizare. Alte cauze ale intersexualitatii de tip XY sunt:

disfunctii ale testiculelor. Testiculele produc in mod normal hormoni masculini, insa daca dezvoltarea acestora nu are loc in conditii normale, persoana in cauza se va confrunta cu fenomenul de subvirilizare. Cauzele dezvoltarii incomplete a tessticulelor sunt variate, insa cea mai frecventa este disgeneza gonadica pura;

disfunctii in ceea ce priveste producerea de testosteron (hormon barbatesc), acest proces necesitand un serie de pasi, in cadrul fiecarui actionand o alta enzima. Carente ale oricarei enzime implicate in procesul de formare a testosteronului conduce la fenomenul de subvirilizare, in aceasta categorie intrand diferite tipuri de hiperplazie adrenala congenitala;

disfunctii ale modului de folosire a testosteronului. In aceste situatii nivelul de testosteron produs de testicule poate sa fie normal: - deficienta de 5-alfa reductaza, caz in care enzima necesara convertirii testosteronului in dihidrotestosteron (DHT) lipseste; sindrom de insensibilitate la androgeni, reprezentand una dintre cele mai frecvente cauze ale intersexualitatii de tip 46 XY, manifestat prin functionarea anormala a receptorilor pentru hormoni masculini.

3. Intersexualitate de tip gonadal


Intersexualitate de tip gonadal se manifesta atat prin prezenta tesutului testicular, cat si al tesutului ovarian intr-un singur organism. Astfel, pacientul poate avea cromozomi de tip XX si XY, in vreme ce organele sexuale externe sunt ambigui. Medicii specialisti supranumesc aceasta forma de intersexulitate hermafrodism pur. Desi cauzele intersexualitatii de tip gonadal nu sunt cunoscute studiile realizate de experti in acest dmeniu au stabilit o legatura a acestei conditii medicale cu expunerea la pesticide in timpul sarcinii.

4. Disfunctii complexe in stabilirea sexului genetic al pacientului


In categoria disfunctiilor complexe (nedeterminate) in stabilirea sexului genetic al pacientului intra orice combinatie cromozomiala, alta decat 46 XX sau 46 XY. Acestea includ 45 X0, caz in care organismul va avea un singur cromozom, de tip X, si 47, XXX sau 47, XXY, ambele cazuri fiind caracterizate de prezenta unui cromozom in plus (fie X sau Y). Aceste conditii medicale nu sunt caractetizate de o discrepanta intre organele sexuale interne si cele externe, insa pot exista probleme in ceea ce priveste nivelul de hormoni sexuali, dezvoltarea sexuala a pacientului, sau numarul scazut de cromozomi sexuali.

Metode de tratament pentru intersexualitate (hermafrodism)


Pentru a tratarea eficienta a diagnosticului de intersexualitate este necesara interventia unei echipe de medici specialisti a caror munca sa aiba ca rezultat intelegerea si tratarea pacientului care se confrunta cu aceasta conditie medicala, precum si consilierea membrilor familiei acestora. Totodata, este necesar ca parintii sa inteleaga implicatiile intersexualitatii si a tratarii acesteia in primii ani din viata copilului. In trecut, specialistii considerau ca este extrem de important stabilirea cat mai rapida a unui gen sexual al pacientului astfel incat sa se poata interveni prompt in reconstruirea organelor sexuale externe, indepartand prin metode chirurgicale tesuturile genului secundar. Problema acestei gandiri era datorata ignorarii genului cromozomial (dat de numarul mare de hormoni barbatesti sau femeiesti), in detrimentul celui indus de organele genitale externe. In prezent, opinia expertilor s-a schimbat radical, acestia considerand ca desi organele sexuale femeiesti sunt mai usor de reconstruit, complexitatea acestora este mai mare. In concluzie, inainte de a se decide genul copilului diagnosticat cu intersexualitate, parintii sunt sfatuiti sa tina seama de o gama extrem de vasta de factori de tip cromozomial, neurologic, hormonal, psihologic si de comportament. Deasemenea, unii specialisti sustin ca tratamentul medical trebuie amanat pana in momentul in care insusi copilul va avea putere de decizie in privinta stabilirii sexului sau.

Pseudohermaphroditism
From Wikipedia, the free encyclopedia

Pseudohermaphroditism
Classification and external resources

ICD-10

Q56.1-Q56.3

ICD-9

752.7

DiseasesDB

14836 14839

MeSH

D012734

Pseudohermaphroditism, or pseudo-hermaphroditism, is the condition in which an organism is born with secondary sex characteristics[1] or aphenotype[2] that is different from what would be expected on the basis of the gonadal tissue (ovary or testis). In some cases, the external sex organs look intermediate between the typical clitoris or penis. In other cases, the external sex organs have an appearance that does not look intermediate, but rather has the appearance that would be expected to be seen with the "opposite" gonadal tissue. Because of this, pseudohermaphroditism is sometimes not identified until puberty. It is possible for the condition to be undetected until adulthood.[3] The term "male pseudohermaphrodite" is used when a testis is present, and the term "female pseudohermaphrodite" is used when an ovary is present.[4] The term "true" hermaphrodite is reserved for the very rare cases where both ovarian and testicular tissue is present. (Whether or not that term would be appropriate when ovotestes are found, or only when distinct ovaries and testes are found, is not well defined.) Associated conditions in males include 5--reductase deficiency[5] from a deficiency in the male chromosome (46 XY).[6][7]
Contents
[hide]

1 Surgery

2 Terminology 3 History 4 See also 5 References

[edit]Surgery
Surgery has sometimes been performed to alter the appearance of the genitals.[8][9]

[edit]Terminology
Use of the term "pseudohermaphroditism" can be problematic, and is now considered redundant.
[10]

The term "pseudohermaphroditism" was created by Edwin Klebs in 1876,[11][12] long before the

genetic roles of the X chromosome and Y chromosome and the social components of gender identity were well characterized, which is why the term is usually used to describe the dissonance between gonadal histology and external genital appearance. The term "intersexuality" was introduced by Richard Goldschmidt in 1923.[11][13] However, the term "intersex" has also been challenged; the Lawson Wilkins Pediatric Endocrine Society and the European Society for Pediatric Endocrinology have adopted a nomenclature system based on disorders of sex development, which covers "congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical" and thus replaces many disparate terms, including but not limited to those based on "hermaphrodite."[10][14] One example of the challenges involved in the use of the term is the case of women with Complete Androgen Insensitivity Syndrome (CAIS). These women often have primary and secondary sexual characteristics typical of other women; however, they are genetically XY and have internal testes, rather than ovaries. They have the same likelihood of a genetically XX woman of enjoying sexual pleasure but are unable to biologically reproduce. Their sexuality (homosexual, heterosexual, bisexual etc.) is unrelated to this syndrome. However scientifically precise the description "male" pseudohermaphrodite may be for such women, it is clearly socially inappropriate. CAIS is considered little better by some, as the S for syndrome in CAIS does not accord with the "normality" many CAIS women feel about their bodies. In human beings, the sex status is defined at four levels: chromosomal (XY; XX), internal organs (ovaries; testicles), external organs (breasts, vulva + vagina; penis), and psyche (sexual identity). In a XX human the default development process results in a female. In a XY human a set of genes on the Y chromosome trigger a cascade of events normally resulting in a male. A complete female or male developmental process entails the expression of female and male sex hormones, respectively, and of

their corresponding receptors in the target tissues. Without these hormones and their receptors, the internal and external sex organs, and psyche, will not develop as expected. Sex hormones, their receptors, and downstream signal transduction proteins are coded by genes that may be genetically defective. All these factors mean that genetic mutations can block the sexual development process at three stages: (a) before the development of the internal sex organs; (b) after the development of the internal sex organs but before the development of external sex organs; and (c) after the development of external sex organs but before the maturation of the sexual component of the psyche. While in (a) the XY human will be indistinguishable anatomically and psychologically from a female; in (b) the individual may either be born with ambiguous external genitals or have genitals apparently in the normal range at birth but, at pubertal age, not develop secondary sexual characteristics at all or develop secondary sexual characteristics that do not match the external genitals; and in (c) the individual will be transgendered (formerly referred to as transsexual). There are other intersexual states that are not the result of the genetic configurations above. In particular, where either the individual is a chimera - resulting from a fusion of two distinct embryos, one male and one female, during fetal development (not a genetic mosaic), or the individual contains duplicated chromosomes in the genome (XXY; XXXY). In the former case some tissues will be in the XX and others in the XY configuration; in the latter, all cells contain the Y chromosome and may or may not use it. This is a gynandromorph, which has both female and male characteristics at all four levels and may have either ambiguous sex organs (the XY/XX configuration may not be evenly distributed throughout the body) or unambiguous male and female sex organs (hermaphrodite).

[edit]History
John Money is perhaps the best-known early researcher in this area. His doctoral thesis was titled Hermaphroditism: An Inquiry into the Nature of a Human Paradox, and awarded by Harvard University in 1952.[15] Money's general views on gender identity as something learned during childhood were later directly contradicted by a biography published in 2001 by one of his former patients, David Reimer. Among the repercussions was damage to John Money's reputation. Not only had his theory of gender plasticity been dealt a severe blow but Reimer's biography described bizarrely unpleasant childhood therapy sessions, and implied that Money had ignored or concealed the developing evidence that Reimer's reassignment to female was not going well. Money's defenders have suggested that some of the allegations about the therapy sessions may have been the result of False memory syndrome. However, Reimer's brother and mother both agreed that the therapy was not "working" in the sense

that Reimer wasn't in any way developing a female self-image during his treatment with Dr. Money. Dr. Money never publicly stated that his conclusions were incorrect. Milton Diamond has probably become the best known expert public advocate for the intersex community in the early 21st century. He is the director of the Pacific Center for Sex and Society.[16]

[edit]See

also

ntersex
Disorders of sex development; DSDs; Pseudohermaphroditism; Hermaphroditism; Hermaphrodite
Last reviewed: August 2, 2011.

Intersex is a group of conditions where there is a discrepancy between the external genitals and the internal genitals (the testes and ovaries). The older term for this condition, hermaphroditism, came from joining the names of a Greek god and goddess, Hermes and Aphrodite. Hermes was a god of male sexuality (among other things) and Aphrodite a goddess of female sexuality, love, and beauty. Although the older terms are still included in this article for reference, they have been replaced by most experts (and patients and families) because they are misleading, confusing, and insensitive. Increasingly this group of conditions is being called disorders of sex development (DSDs).

Causes, incidence, and risk factors


Intersex can be divided into four categories: 46, XX Intersex 46, XY Intersex True Gonadal Intersex Complex or Undetermined Intersex Each one is discussed in more detail below. Note: In many kids the cause of intersex may remain undetermined, even with modern diagnostic techniques. 46, XX Intersex. The person has the chromosomes of a woman, the ovaries of a woman, but external (outside) genitals that appear male. This usually is the result of a female fetus having been exposed to excess male hormones before birth. The labia ("lips" or folds of skin of the external female genitals) fuse, and the clitoris enlarges to appear like a penis. Usually this person has a normal uterus and Fallopian tubes. This condition is also called 46, XX with virilization. It used to be called female pseudohermaphroditism. There are several possible causes: Congenital adrenal hyperplasia (the most common cause). Male hormones (such as testosterone) taken or encountered by the mother during pregnancy. Male hormone-producing tumors in the mother. These are most often ovarian tumors. Mothers who have children with 46, XX intersex should be checked unless there is another clear cause.

Aromatase deficiency. This one may not be noticeable until puberty. Aromatase is an enzyme that normally converts male hormones to female hormones. Too much aromatase activity can lead to excess estrogen (female hormone); too little to 46, XX intersex. At puberty these XX children, who had been raised as girls, may begin to take on male characteristics. 46, XY Intersex. The person has the chromosomes of a man, but the external genitals are incompletely formed, ambiguous, or clearly female. Internally, testes may be normal, malformed, or absent. This condition is also called 46, XY with undervirilization. It used to be called male pseudohermaphroditism. Forming normal male external genitals depends on the appropriate balance between male and female hormones; therefore, it requires the adequate production and function of male hormones. 46, XY intersex has many possible causes: Problems with the testes. The testes normally produce male hormones. If the testes do not form properly, it will lead to undervirilization. There are a number of possible causes for this, including XY pure gonadal dysgenesis. Problems with testosterone formation. Testosterone is formed through a series of steps where each requires a different enzyme. Deficiencies in any of these enzymes can result in inadequate testosterone and produce a different syndrome of 46, XY intersex. Different types of congenital adrenal hyperplasia can fall in this category. Problems with using testosterone. Some people have normal testes and make adequate amounts of testosterone, but still have 46, XY intersex. o 5-alpha-reductase deficiency. People with 5-alpha-reductase deficiency lack the enzyme needed to convert testosterone to dihydrotestosterone (DHT). There are at least five different types of 5-alpha-reductase deficiency. Some of the babies have normal male genitalia, some have normal female genitalia, and many have something in between. Most change to external male genitalia around the time of puberty. o Androgen insensitivity syndrome (AIS). This is the most common cause of 46, XY intersex. Here the hormones are all normal, but the receptors to male hormones don't function properly. There are over 150 different defects that have been identified so far, and each causes a different type of AIS. AIS has also been called testicular feminization. True Gonadal Intersex. Here the person must have both ovarian and testicular tissue. This might be in the same gonad (an ovotestis), or the person might have one ovary and one testis. The person may have XX chromosomes, XY chromosomes, or both. The external genitals may be ambiguous or may appear to be female or male. This condition used to be called true hermaphroditism. In most people with true gonadal intersex, the underlying cause is unknown, although in some animal studies it has been linked to exposure to common agricultural pesticides. Complex or Undetermined Intersex Disorders of Sexual Development. Many chromosome configurations other than simple 46, XX or 46, XY can result in disorders of sex development. These include 45, XO (only one X chromosome), and 47, XXY, 47, XXX -- both cases have an extra sex chromosome, either an X or a Y. These disorders do not result in an intersex condition where there is discrepancy between internal and external genitalia. However, there may be problems with sex hormone levels, overall sexual development, and altered numbers of sex chromosomes.

Symptoms

The symptoms associated with intersex will depend on the underlying cause, but may include: Ambiguous genitalia at birth Micropenis Clitoromegaly (an enlarged clitoris) Partial labial fusion Apparently undescended testes (which may turn out to be ovaries) in boys Labial or inguinal (groin) masses -- which may turn out to be testes -- in girls Hypospadias (the opening of the penis is somewhere other than at the tip; in females, the urethra [urine canal] opens into the vagina) Otherwise unusual appearing genitalia at birth Electrolyte abnormalities Delayed or absent puberty Unexpected changes at puberty

Signs and tests


Chromosome analysis Hormone levels (for example, testosterone level) Hormone stimulation tests Electrolyte tests Specific molecular testing Endoscopic examination (to verify the absence or presence of a vagina or cervix) Ultrasound or MRI to evaluate whether internal sex organs are present (for example, a uterus)

Treatment
Ideally, a team of health care professionals with expertise in intersex should work together to understand and treat the child with intersex -- and to understand, counsel, and support the entire family. Parents should understand controversies and changes in treating intersex in recent years. In the past, the prevailing opinion was that it was generally best to assign a gender as quickly as possible, often based on the external genitals rather than the chromosomal gender, and to instruct the parents to have no ambiguity in their minds as to the gender of the child. Prompt surgery was often recommended. Ovarian or testicular tissue from the other gender would be removed. In general, it was considered easier to reconstruct female genitalia than functioning male genitalia, so if the "correct" choice was not clear, the child was often assigned to be a girl. More recently, the opinion of many experts has shifted. Greater respect for the complexities of female sexual functioning has led them to conclude that suboptimal female genitalia may not be inherently better than suboptimal male genitalia, even if the reconstruction is "easier." In addition,

other factors may be more important in gender satisfaction than functioning external genitals. Chromosomal, neural, hormonal, psychological, and behavioral factors can all influence gender identity. Many experts now urge delaying definitive surgery for as long as healthy, and ideally involving the child in the gender decision. Clearly, intersex is a complex issue, and its treatment has short- and long-term consequences. The best answer will depend on many factors, including the specific cause of the intersex. It is best to take the time to understand the issues before rushing into a decision. An intersex support group may help acquaint families with the latest research, and may provide a community of other families, children, and adult individuals who have faced the same issues.

Support Groups
Support groups are very important for families dealing with intersex. Different support groups may differ in their thoughts regarding this very sensitive topic. Look for one that supports your thoughts and feelings on the topic. The following organizations provide further information: American Association for Klinefelter Syndrome Information and Support -- www.aaksis.org CARES Foundation -- www.caresfoundation.org Congenital Adrenal Hyperplasia Education and Support Network -- www.congenitaladrenalhyperplasia.org Hypospadias and Epispadias Association -- www.heainfo.org Intersex Society of North America -- www.isna.org Turner Syndrome Society of the US -- www.turnersyndrome.org

Expectations (prognosis)
Please see information on the individual conditions. The prognosis depends on the specific cause of intersex. With understanding, support, and appropriate treatment, overall outlook is excellent.

Calling your health care provider


If you notice that your child has unusual genitalia or sexual development, discuss this with your health care provider.

pseudohermaphroditism
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pseudohermaphroditism, a condition in which the individual has a single chromosomal and gonadal sex but combines features of both sexes in the external genitalia, causing doubt as to the true sex. Female pseudohermaphroditism refers to an individual with ovaries but with secondarysexual characteristics or external genitalia resembling those of a male. Male

pseudohermaphroditism refers to individuals whose gonads are testes but whose secondary sexual characteristics or external genitalia resemble those of a female.
Table Of Contents

Female pseudohermaphroditism
If during the first trimester of pregnancy a woman carrying a female fetus is given an androgen, ananabolic steroid, or a progestin (a substance with effects similar to those of progesterone), her fetusmay be masculinized. Androgen-producing tumours of either adrenal or ovarian origin may also lead to masculinization of a female fetus. Congenital adrenal hyperplasia, also known as adrenogenital syndrome, is a common cause of female pseudohermaphroditism. This disorder arises from an inherited defect in one of the enzymesneeded for the production of cortisol, a hormone that plays a major role in the physiological response to stress. Excessive amounts of adrenal androgens must be produced to overcome the decrease in cortisol production. In female infants this results in masculinization with anomalous development of the genital organs, whereas in male infants it results in premature sexual development (precocious puberty).
Table Of Contents

Male pseudohermaphroditism
Male pseudohermaphrodites are genetic males (45,XY) who develop female configurations and identities. These individuals have testes, but their genital ducts and external genitalia are female. Secondary sex characteristics may never appear in some, whereas others may achieve a fully feminized appearance. Male pseudohermaphroditism is rare and almost always results from autosomal recessive genetic defects (defects that must be inherited from both parents in order to be expressed). Several specific defects lead to feminization in genetic males. Each of these defects, by one mechanism or another, results in a loss of androgenic effects on body tissues. In rare instances, Leydig cells (the interstitial cells of the testes) are absent or greatly reduced in number, presumably because the receptors for luteinizing hormone (LH) are defective. Without Leydig cells, only small amounts of testosterone are produced. In other individuals there are enzyme deficiencies analogous to those that occur in female pseudohermaphrodites, although the enzyme deficiencies in males result in fetal androgen deficiency.

In some persons, tissue receptors for androgens are absent or reduced, forming a spectrum of syndromes of partial to complete resistance to androgens. The most striking example of resistance to androgens is complete testicular feminization. Affected individuals are born with female genitalia and a vagina that ends blindly (no cervix or uterus is present). Despite having testes located either in the labia or within the abdomen, these persons grow into wellproportioned females with normal breasts and scant or absent axillary and pubic hair. They have a strong female orientation, but they do not menstruate. Their hormonal aberrations are dramatic and predictable. With a loss of hypothalamic and pituitary androgen receptors, there is no inhibition of LH secretion. Serum LH concentrations are high, leading to stimulation of the Leydig cells and to high serum testosterone concentrations. The conversion of the increased amounts of testosterone to estrogen in peripheral tissues increases serum estrogen concentrations in males. In another extraordinary variant, the lesion lies not in the loss of androgen receptors but rather in the loss of 5-alpha-reductase, an enzyme necessary for the conversion of testosterone to the more potent hormone dihydrotestosterone. In this syndrome, because of a lack of testosterone directing fetal development toward a normal male configuration, genetic males are born with what appears to be female genitalia with an enlarged clitoris. These persons are often raised as females, but atpuberty an increase in testosterone secretion leads to masculinization. There then ensues a transition from the psychosocial behaviour of a prepubertal female to that of an adult male. In some fetuses there occurs, for unknown reasons, regression and disappearance of the testes, known as the vanishing testes syndrome. When this occurs early in pregnancy, before androgen-induced differentiation toward male genitalia, the child is born with female genitalia. If the testes disappear during the crucial period between 8 and 10 weeks of gestation, the child is born with ambiguous genitalia, whereas if the disappearance occurs after this key period the individual is born a male but without any testes (anorchia). Treatment must be highly individualized. In many instances, gender identity has been established by the age of 18 to 24 months, and changes in sexual identity thereafter should be attempted only after careful consideration. Intra-abdominal testes should be removed because of an increased risk of tumour formation. The patient can be treated at the appropriate time with sex hormones. Robert D. Utiger

Ambiguous genitalia is a birth defect where the outer genitals do not have the typical appearance of either a boy or a girl. See also: Genetics

Considerations
The genetic sex of a child is determined at conception. The mother's egg cell (ovum) contains an Xchromosome, while the father's sperm cell contains either an X or a Y chromosome. These X and Y chromosomes determine the child's genetic sex. Normally, an infant inherits one pair of sex chromosomes -- one X from the mother and one X or one Y from the father. The father "determines" the genetic sex of the child. A baby who inherits the X chromosome from the father is a genetic female (two X chromosomes). A baby who inherits the Y chromosome from the father is a genetic male (one X and one Y chromosome). The male and female reproductive organs and genitals both come from the same tissue in the fetus. If the process that causes this fetal tissue to become "male" or "female" is disrupted, ambiguous genitalia can develop. The genitalia makes it difficult to easily identify the infant as male or female. The extend of the ambiguity varies. In very rare instances, the physical appearance may be fully developed as the opposite of the genetic sex. For example, a genetic male may have developed the appearance of a normal female. Typically, ambiguous genitalia in genetic females (babies with two X chromosomes) has the following features: An enlarged clitoris that looks like a small penis. The urethral opening (where urine comes out) can be anywhere along, above, or below the surface of the clitoris. The labia may be fused and look like a scrotum. The infant may be thought to be a male with undescended testicles. Sometimes a lump of tissue is felt within the fused labia, further making it look like a scrotum with testicles.

In a genetic male (one X and one Y chromosome), ambiguous genitalia usually include the following features: A small penis (less than 2 - 3 centimeters or 3/4 to 1 1/4 inches) that looks like an enlarged clitoris (the clitoris of a newborn female is normally somewhat enlarged at birth). The urethral opening may be anywhere along, above, or below the penis. It can be located as low as on the peritoneum, further making the infant appear to be female. There may be a small scrotum that is separated and looks like labia. Undescended testicles commonly occur with ambiguous genitalia.

Ambiguous genitalia is usually not life threatening (see Causes section for exceptions), but it can create social problems for the child and family. For this reason, a team of experienced specialists, including neonatologists, geneticists, endocrinologists, and psychiatrists or social workers will be involved in the child's care.

Causes
Pseudohermaphroditism. The genitalia are of one sex, but some physical characteristics of the other sex are present. True hermaphrodism. This is a very rare condition, in which tissue from both the ovaries and testicles is present. The child may have parts of both male and female genitals. Mixed gonadal dysgenesis (MGD). This is an intersex condition, in which there are some male structures (gonad, testis), as well as a uterus, vagina, and fallopian tubes. Congenital adrenal hyperplasia. This condition has several forms, but the most common form causes the genetic female to appear male. Many states test for this potentially lifethreatening condition during newborn screening exams. Chromosomal abnormalities, including Klinefelter's syndrome (XXY) and Turner's syndrome (XO). If the mother takes certain medications (such as androgenic steroids), they may make a genetic female look more male. Lack of production of certain hormones can cause the embryo to develop with a female body type, regardless of genetic sex Lack of testosterone cellular receptors. Even if the body makes the hormones needed to develop into a physical male, the body cannot respond to those hormones. This produces a female body-type, even if the genetic sex is male.

Home Care
Because of the potential social and psychological effects of this condition, parents should make a decision about whether to raise the child as a male or female early after diagnosis, preferably within the first few days of life. This is an important decision, so parents should not rush it.

When to Contact a Medical Professional


You are concerned about the appearance of your child's external genitalia, or your baby: Takes more than 2 weeks to regain his or her birth weight Is vomiting Looks dehydrated (dry inside of mouth, no tears when crying, less than 4 wet diapers per 24 hours, eyes look sunken in) Has a decreased appetite Has blue spells Has trouble breathing

These can all be signs of congenital adrenal hyperplasia. Ambiguous genitalia may be discovered during the first well-baby examination.

What to Expect at Your Office Visit


The doctor will perform a physical examination, which may reveal genitals that are not "typical male" or "typical female," but somewhere in between. The doctor will ask medical history questions to help identify any chromosomal disorders. Questions may include: Is there any family history of miscarriage? Is there any family history of stillbirth? Is there any family history of early death? Have any family members had infants who died in the first few weeks of life or who had ambiguous genitalia? Is there any family history of any of the disorders that cause ambiguous genitalia? What medications did the mother take before or during pregnancy (especially steroids)? What other symptoms are present?

Genetic testing can determine if the child is a genetic male or female. Often a small sample of cells can be scraped from inside the child's cheeks (this is called a buccal smear). Examining these cells is often enough to determine the genetic sex of the infant. Chromosomal analysis is a more extensive cell study that may be needed in more questionable cases. Endoscopy, abdominal x-ray, abdominal or pelvic ultrasound, and similar tests may be needed to determine the presence or absence of the internal genitals (such as undescended testes). Laboratory tests may help determine how well the reproductive organs are functioning. This can include tests for adrenal and gonadal steroids. In some cases, laparoscopy, exploratory laparotomy, or biopsy of the gonads may be needed to confirm disorders that can cause ambiguous genitalia. Depending on the cause, surgery, hormone replacement, or other treatments are used to treat conditions that can cause ambiguous genitalia. Sometimes, the parents must choose whether to raise the child as male or female (regardless of the child's chromosomes). This choice can have a big social and psychological impact on the child, so counseling is usually recommended. Note: It is often technically easier to treat (and therefore raise) the child as female (it is easier for a surgeon to make female genitalia than it is to make male genitalia), so in some cases this is recommended even if the child is genetically male. However, this is a difficult decision. You should discuss it with your family, your child's doctor, the surgeon, your child's endocrinologist, and other health care team members.

Alternative Names
Genitals - ambiguous

Intersex - Overview

Overview Symptom Treatment Prevention All Information

Alternative Names

Disorders of sex development; DSDs; Pseudohermaphroditism; Hermaphroditism; Hermaphrodite


Definition of Intersex:

Intersex is a group of conditions where there is a discrepancy between the external genitals and the internal genitals (the testes and ovaries). The older term for this condition, hermaphroditism, came from joining the names of a Greek god and goddess, Hermes and Aphrodite. Hermes was a god of male sexuality (among other things) and Aphrodite a goddess of female sexuality, love, and beauty. Although the older terms are still included in this article for reference, they have been replaced by most experts (and patients and families) because they are misleading, confusing, and insensitive. Increasingly this group of conditions is being called disorders of sex development (DSDs).
Causes, incidence, and risk factors:

Intersex can be divided into four categories:


46, XX Intersex 46, XY Intersex True Gonadal Intersex Complex or Undetermined Intersex

Each one is discussed in more detail below. Note: In many kids the cause of intersex may remain undetermined, even with modern diagnostic techniques. 46, XX Intersex. The person has the chromosomes of a woman, the ovaries of a woman, but external (outside) genitals that appear male. This usually is the result of a female fetus having been exposed to

excess male hormones before birth. The labia ("lips" or folds of skin of the external female genitals) fuse, and the clitoris enlarges to appear like a penis. Usually this person has a normal uterus and Fallopian tubes. This condition is also called 46, XX with virilization. It used to be called female pseudohermaphroditism. There are several possible causes:

Congenital adrenal hyperplasia (the most common cause). Male hormones (such as testosterone) taken or encountered by the mother during pregnancy. Male hormone-producing tumors in the mother. These are most often ovarian tumors. Mothers who have children with 46, XX intersex should be checked unless there is another clear cause. Aromatase deficiency. This one may not be noticeable until puberty. Aromatase is an enzyme that normally converts male hormones to female hormones. Too much aromatase activity can lead to excess estrogen (female hormone); too little to 46, XX intersex. At puberty these XX children, who had been raised as girls, may begin to take on male characteristics.

46, XY Intersex. The person has the chromosomes of a man, but the external genitals are incompletely formed, ambiguous, or clearly female. Internally, testes may be normal, malformed, or absent. This condition is also called 46, XY with undervirilization. It used to be called male pseudohermaphroditism. Forming normal male external genitals depends on the appropriate balance between male and female hormones; therefore, it requires the adequate production and function of male hormones. 46, XY intersex has many possible causes:

Problems with the testes. The testes normally produce male hormones. If the testes do not form properly, it will lead to undervirilization. There are a number of possible causes for this, including XY pure gonadal dysgenesis. Problems with testosterone formation. Testosterone is formed through a series of steps where each requires a different enzyme. Deficiencies in any of these enzymes can result in inadequate testosterone and produce a different syndrome of 46, XY intersex. Different types of congenital adrenal hyperplasia can fall in this category. Problems with using testosterone. Some people have normal testes and make adequate amounts of testosterone, but still have 46, XY intersex. o 5-alpha-reductase deficiency. People with 5-alphareductase deficiency lack the enzyme needed to convert

testosterone to dihydrotestosterone (DHT). There are at least five different types of 5-alpha-reductase deficiency. Some of the babies have normal male genitalia, some have normal female genitalia, and many have something in between. Most change to external male genitalia around the time of puberty. Androgen insensitivity syndrome (AIS). This is the most common cause of 46, XY intersex. Here the hormones are all normal, but the receptors to male hormones don't function properly. There are over 150 different defects that have been identified so far, and each causes a different type of AIS. AIS has also been called testicular feminization.

True Gonadal Intersex. Here the person must have both ovarian and testicular tissue. This might be in the same gonad (an ovotestis), or the person might have one ovary and one testis. The person may have XX chromosomes, XY chromosomes, or both. The external genitals may be ambiguous or may appear to be female or male. This condition used to be called true hermaphroditism. In most people with true gonadal intersex, the underlying cause is unknown, although in some animal studies it has been linked to exposure to common agricultural pesticides. Complex or Undetermined Intersex Disorders of Sexual Development. Many chromosome configurations other than simple 46, XX or 46, XY can result in disorders of sex development. These include 45, XO (only one X chromosome), and 47, XXY, 47, XXX -both cases have an extra sex chromosome, either an X or a Y. These disorders do not result in an intersex condition where there is discrepancy between internal and external genitalia. However, there may be problems with sex hormone levels, overall sexual development, and altered numbers of sex chromosomes.

Reviewed last on: 8/2/2011 Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Allen L. Disorders of sexual development. Obstet Gynecol Clin North Am. 2009;36:25-45.

Donohoue PA. Disorders of sex development (intersex). In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 582.
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Hermaphrodite
From Wikipedia, the free encyclopedia

For other uses, see Hermaphrodite (disambiguation).

Mating Helix aspersa (garden snails).

In biology, a hermaphrodite is an organism that has reproductive organs normally associated with both male and female sexes.[1] Many taxonomic groups of animals (mostly invertebrates) do not have separate sexes[citation needed]. In these groups, hermaphroditism is a normal condition, enabling a form of sexual reproduction in which both partners can act as the "female" or "male". For example, the great majority of pulmonatesnails, opisthobranch snails and slugs are hermaphrodites. Hermaphroditism is also found in some fish species and to a lesser degree in othervertebrates. Most plants are also hermaphrodites. Historically, the term hermaphrodite has also been used to describe ambiguous genitalia and gonadal mosaicism in individuals of gonochoristic species, especially human beings. The word hermaphrodite entered the English lexicon in the late 14th century, derived

from Greek Hermaphroditos, the son of the Greek god Hermes and the goddess Aphrodite.[2] Recently, the word intersex has come into preferred usage for humans, since the word hermaphrodite is considered to be misleading and stigmatizing,[3] as well as "scientifically specious and clinically problematic".[4]
Contents
[hide]

o o o o

1 Zoology 1.1 Sequential hermaphrodites 1.2 Simultaneous hermaphrodites 1.3 Pseudohermaphroditism 1.4 Humans 2 Botany 3 Other uses of the term 4 Etymology 5 See also 6 References 7 Further reading 8 External links

[edit]Zoology [edit]Sequential

hermaphrodites

Main article: Sequential hermaphroditism

Shells of Crepidula fornicata (common slipper shell).

Sequential hermaphrodites (dichogamy) occur in species in which the individual is born as one sex, but can later change into the opposite sex.[5] This is in contrast with simultaneous hermaphrodites, in which an individual may possess fully functional male and female gonads. Sequential hermaphroditism is common in fish (particularly teleost fish) and some jellyfish, many gastropods (such as the common slipper shell), and some flowering plants. While some sequential hermaphrodites can change sex multiple times, most can only change sex once.[citation needed] Sequential hermaphrodism can best be understood in terms of behavioral ecology and evolutionary life history theory, as described in the sizeadvantage model[6] first proposed by Michael T. Ghiselin[7] which states that if an individual of a certain sex could significantly increase its reproductive success after reaching a certain size, it would be to their advantage to switch to that sex. Sequential hermaphrodites fall into two broad categories:

Protandry: Where an organism is born as a male, and then changes sex to a female.[5]

Example: The clownfish (genus Amphiprion) are colorful reef fish found living

in symbiosis with sea anemones. Generally one anemone contains a 'harem ', consisting of a large female, a smaller reproductive male, and even smaller non-reproductive males. If the female is removed, the reproductive male will change sex and the largest of the nonreproductive males will mature and become reproductive. It has been shown that fishing pressure can change when the switch from male to female occurs, since fishermen usually prefer to catch the larger fish. The populations are generally changing sex at a smaller size, due to natural selection.

Protogyny: Where the organism is born as a female, and then changes sex to a male.[5]

Example: wrasses (Family Labridae) are a group of reef fish in which protogyny is

common. Wrasses also have an uncommon life history strategy, which is termed diandry (literally, two males). In these species, two male morphs exists: an initial phase male and a terminal phase male. Initial phase males do not look like males and spawn in groups with other females. They are not territorial. They are, perhaps, female mimics (which is why they are found swimming in group with other females). Terminal phase males are territorial and have a distinctively bright coloration. Individuals are born as males or females, but if they are born males, they are not born as terminal phase males. Females and initial phase males can become terminal phase males. Usually, the most dominant female or initial phase male replaces any terminal phase male when those males die or abandon the group. Dichogamy can have both conservation-related implications for humans, as mentioned above, as well as economic implications. For instance, groupers are favoured fish for eating in many Asian countries

and are often aquacultured. Since the adults take several years to change from female to male, the broodstock are extremely valuable individuals.

[edit]Simultaneous

hermaphrodites

Earthworms are simultaneous hermaphrodites, having both male and female reproductive organs.

A simultaneous (or synchronous) hermaphrodite (or homogamous) is an adult organism that has both male and female sexual organs at the same time.[5] Usually, self-fertilization does not occur.

Reproductive system of gastropods: Pulmonate land snails and land slugs are perhaps the

best-known kind of simultaneous hermaphrodite, and are the most widespread of terrestrial animals possessing this sexual polymorphism. Sexual material is exchanged between both animals viaspermatophore, which can then be stored in the spermatheca. After exchange of spermatozoa, both animals will lay fertilized eggs after a period of gestation; then the eggs will proceed to hatch after a development period. Snails typically reproduce in early spring and late autumn.

Banana slugs are one example of a hermaphroditic gastropod. Mating with a partner is more

desirable biologically, as the genetic material of the resultant offspring is varied, but if mating with a partner is not possible, self-fertilization is practiced. The male sexual organ of an adult banana slug is quite large in proportion to its size, as well as compared to the female organ. It is possible for banana slugs, while mating, to become stuck together. If a substantial amount of wiggling fails to separate them, the male organ will be bitten off (using the slug's radula), see apophallation. If a banana slug has lost its male sexual organ, it can still mate as a female, making its hermaphroditic quality a valuable adaptation.

Hamlets, unlike other fish, seem quite at ease mating in front of divers, allowing observations

in the wild to occur readily. They do not practice self-fertilization, but when they find a mate, the pair takes turns between which one acts as the male and which acts as the female through multiple matings, usually over the course of several nights.

Earthworms are another example of a simultaneous hermaphrodite. Although they possess

ovaries and testes, they have a protective mechanism against self-fertilization. Sexual

reproduction occurs when two worms meet and exchange gametes, copulating on damp nights during warm seasons. Fertilized eggs are protected by a cocoon, which is buried on or near the surface of the ground.

[edit]Pseudohermaphroditism
Main article: Pseudohermaphroditism

A photograph by Nadar of an intersex person displaying genitalia, one of a nine-part series. The series may be the earliest medical photographic documentation ofintersexuality.[8]

When spotted hyenas were first discovered by explorers, they were thought to be hermaphrodites. Early observations of spotted hyenas in the wild lead researchers to believe that all spotted hyenas, male and female, were born with what appeared to be a penis. The apparent penis in females is in fact an enlarged clitoris, which contains an external birth canal.[9][10] It can be difficult to determine the sex of wild spotted hyenas until sexual maturity, when they may become pregnant. When a female spotted hyena gives birth, they pass the cub through the cervix internally, but then pass it out through the elongated clitoris.[11]

[edit]Humans
Main article: Intersex True hermaphroditism in humans differs from pseudohermaphroditism in which the person's karyotype has both XX and XY chromosome pairs, having both testicular and ovarian tissue, and having ambiguous-looking external genitalia. One possible pathophysiologic explanation of this rare phenomenon is a parthenogenetic division of a haploid ovum into two haploid ova. Upon fertilization of the two ova by two sperm cells (one carrying an X and the other carrying

a Y chromosome), the two fertilized ova are then fused together resulting in a person having dual genitalial, gonadal and genetic sex.

[edit]Botany

Hylocereus undatus, a hermaphrodite plant with both carpels and stamens

Main article: Sexual reproduction in plants Hermaphrodite is used in botany to describe a flower that has both staminate (male, pollenproducing) and carpellate (female, ovule-producing) parts. This condition is seen in many common garden plants. A closer analogy to hermaphroditism in botany is the presence of separate male and female flowers on the same individualsuch plants are called monoecious. Monoecy is especially common in conifers, but occurs in only about 7% of angiosperm species.[12]

[edit]Other

uses of the term

Main article: Intersex

Hermaphroditus, the son of the Greek god Hermes and the goddess Aphrodite, origin of the word "hermaphrodite".

Hermaphrodite was used to describe any person incompatible with the biological gender binary, but has recently been replaced by intersex in medicine. Humans with typical reproductive organs but atypical clitoris/penis are called pseudohermaphrodites in medical literature. Pseudohermaphroditism also refers to a human possessing both the clitoris and testicles.[13] People with intersex conditions sometimes choose to live exclusively as one sex or the other, using clothing, social cues, genital surgery, and hormone replacement therapy to blend into the sex they identify with more closely. Some people who are intersex, such as some of those with androgen insensitivity syndrome, outwardly appear completely female or male already, without realizing they are intersex. Other kinds of intersex conditions are identified immediately at birth because those with the condition have a sexual organ larger than a clitoris and smaller than a penis. Intersex is thought by some to be caused by unusual sex hormones; the unusual hormones may be caused by an atypical set of sex chromosomes. Sigmund Freud (based on work by his associate Wilhelm Fliess) held fetal hermaphroditism to be a fact of the physiological development of humans.[citation needed] He based much of his theory of innate sexuality on that assumption.[citation needed] Similarly, in contemporary times, fetuses before sexual differentiation are sometimes described as female by doctors explaining the process.[14] Neither concept is technically true. Before this stage, humans are simply undifferentiated and possess a Mllerian duct, a Wolffian duct, and a genital tubercle.

[edit]Etymology
The term "hermaphrodite" derives from Hermaphroditus, the son of Hermes and Aphrodite in Greek mythology. According to Ovid, he fused with anymph, Salmacis, resulting in one individual possessing physical traits of both sexes.[15] Although, according to Diodorus, who mentions at an earlier period, he was born with a physical body combining both sexes.[16]

[edit]See

also

True hermaphroditism
From Wikipedia, the free encyclopedia

True hermaphroditism
Classification and external resources

OMIM

400045

DiseasesDB

29664

eMedicine

med/1702

MeSH

D050090

True hermaphroditism is a medical term for an intersex condition in which an individual is born with ovarian and testicular tissue. There may be an ovary underneath each testicle on the other, but more commonly one or both gonads is an ovotestis containing both types of tissue. There are no documented cases in which both types of gonadal tissue function. Encountered karyotypes are 47XXY, 46XX/46XY, or 46XX/47XXY, and various degrees of mosaicism (with one interesting case of a XY predominant (96%) mosaic giving birth).[1] Although similar in some ways to mixed gonadal dysgenesis, the conditions can be distinguished histologically.[2] Although fertility is possible in true hermaphrodites (as of 2008 there have been at least 11 reported cases of fertility in true hermaphrodite humans in scientific literature),[2] there has yet to be a documented case where both gonadal tissues function; contrary to folk rumors of hermaphrodites being able to impregnate themselves. [edit]Presentation External genitalia are often ambiguous, the degree depending mainly on the amount of testosterone produced by the testicular tissue between 8 and 16 weeks of gestation. [edit]Causes This condition is very rare. There are several ways in which this may occur. It can be caused by the division of one ovum, followed by fertilization of each haploid ovum and fusion of the two zygotes early in development. Alternately, an ovum can be fertilized by two sperm followed by trisomic rescue in one or more daughter cells. Two ova fertilized by two sperm will occasionally fuse to form a tetragametic chimera. If one male zygote and one female zygote fuse, a hermaphroditic individual may result. It can be associated with mutation in the SRY gene.[3]

[edit]References

Wikimedia Commons has media related to: Hermaphroditism in humans

1. 2.

^ http://www.ncbi.nlm.nih.gov/pubmed/18394621 ^ a b Kim KR, Kwon Y, Joung JY, Kim KS, Ayala AG, Ro JY (October 2002). "True

hermaphroditism and mixed gonadal dysgenesis in young children: a clinicopathologic study of 10 cases". Mod. Pathol. 15 (10): 1013 9. doi:10.1097/01.MP.0000027623.23885.0D. PMID 12379746.

3.

^ Braun A, Kammerer S, Cleve H, Lhrs U, Schwarz HP, Kuhnle U (March 1993). "True

hermaphroditism in a 46,XY individual, caused by a postzygotic somatic point mutation in the male gonadal sex-determining locus (SRY): molecular genetics and histological findings in a sporadic case". Am. J. Hum. Genet. 52 (3): 57885. PMC 1682159.PMID 8447323.
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Female congenital anomalies of the genitalia, including Intersex and DSD: (Q

Uterine malformationMllerian agenesis Unicornuate uterus Uterus didelphys Bicornuate uterus Uterine septum Arcuate ut Internal VaginaVaginal septum Vaginal atresia External Clitoromegaly Progestin-induced virilisation Pseudohermaphroditism

M: FRS

anat/phys/devp

noco/cong/n

True hermaphroditic humans do not exist, but pseudohermaphrodism does, where an individual has both male and female external genital organs, sometimes at the same time. Female embryos exposed to high levels of androgens (the male hormones) develop female internal reproductive organs but male external genitalia. Alternately, genetic defects cause children to be born with female external genital organs,

which change at puberty, with the development of a penis and the closure of the false vagina.

Resources
Books

Campbell, N., J. Reece, and L. Mitchell. Biology. 5th ed. Menlo Park: Benjamin Cummings, Inc. 2000. Elia, Irene. The Female Animal. New York: Henry Holt, 1988. Jorde, L.B., J.C. Carey, M.J. Bamshad, and R.L. White. Medical Genetics 2nd ed. New York: Year Book, Inc., 2000. Stern, Kingsley R. Introductory Plant Biology. Dubuque, IA: Wm. C. Brown, 1991.
Periodicals

Berreby, D. "Sex and the Single Hermaphrodite." Discover 13 (1992): 88-93.

David L. Brock

KEY TERMS
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Gonad An organ that produces sex cells. Protandry A change in sex from male to female. Protogyny

A change in sex from female to male. Pseudohermaphrodite A person who has the physical traits of one sex, while having the genetic instructions of the other. Sequential hermaphrodite An organism that has male or female reproductive organs at one time, and which develops the opposite sex organs. Simultaneous hermaphrodite An organism which develops both male and female reproductive organs at the same time.

Read more: Hermaphrodite - Hermaphrodism In Humans - Female, Male, Sex, and Organs - JRank Articles http://science.jrank.org/pages/3310/HermaphroditeHermaphrodism-in-humans.html#ixzz29rYkbNRO