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Androgen insensitivity syndrome AIS is an intersex condition that results in the partial or complete inability of the cell to respond to androgens. AIS is divided into three categories that are differentiated by the degree of genital masculinization : complete androgen insensitivity syndrome CAIS is indicated when the external intersex are those of a typical female; mild androgen insensitivity syndrome Syndrome is indicated when the external genitalia are those of a typical male, and partial androgen insensitivity syndrome PAIS is indicated when the external genitalia are partially, but not fully, masculinized.

Management of AIS is currently limited to symptomatic management ; no method is currently available to correct the malfunctioning androgen receptor proteins produced by AR gene mutations. Areas of management include sex assignmentgenitoplastygonadectomy in relation to tumor risk, hormone replacement therapygenetic counselingand psychological counseling. The human androgen receptor AR is a protein encoded by a gene located on the proximal long arm of syndrome X chromosome locus XqXq The AR gene contains two polymorphic trinucleotide microsatellites in exon 1.

As ofover AR mutations have been reported in the AR mutation database, and the number continues to grow. Several case studies of fertile 46,XY males with AIS have been published, [4] [56] [57] [58] [59] although this group is thought to be a minority. A genetic female conceived in such syndrome way would receive her father's X chromosome, thus would become a carrier.

A mutation in one but not both results in a minimally affected, fertile, female carrier. If the affected child is a genetic female, she, too, will be a carrier. A genetic female with mutations in both AR genes could theoretically result from the union of a fertile man with AIS and a female carrier of the gene, or from de novo mutation.

However, given the scarcity of fertile AIS men and low incidence of AR mutation, the chances of this occurrence are small. The phenotype of such an individual is a matter of speculation; as ofno such documented case has been published. Individuals syndrome partial AIS, unlike those with the complete or mild forms, present at birth with ambiguous genitaliaand the decision to raise the child as syndrome or female is often not obvious. The effects that androgens have on the human body virilizationmasculinization, anabolismetc.

The following intersex of steps illustrates how androgens and the androgen receptor work together to produce androgenic effects: [1] intersex [3] [13] [18] [74] [75]. In this way, androgens bound to androgen receptors regulate the expression of target genes, thus produce androgenic effects. Theoretically, certain mutant androgen syndrome can function without androgens; in vitro studies have demonstrated that a mutant androgen receptor protein can induce transcription in the absence of androgen if its steroid binding domain is deleted.

Human embryos develop similarly for the first six weeks, regardless of genetic sex 46,XX or 46,XY karyotype ; the only way to tell the difference between 46,XX or 46,XY embryos during this time period is to look for Barr bodies or a Y chromosome.

By the fifth week, intersex genital ridges differentiate into an outer cortex and an inner medullaand are called indifferent gonads. Masculinization of the male external genitalia the penispenile urethraand scrotumas well as the prostateare dependent on the androgen dihydrotestosterone. Mutations in the androgen receptor gene can cause problems with any of the steps involved in androgenization, from the synthesis of the androgen receptor protein itself, through the transcriptional ability of the dimerizedandrogen-AR complex.

This predictive ability intersex primarily retrospective in origin; the different functional domains of the AR gene have been elucidated by analyzing the effects of specific mutations in different regions of the AR. Some mutations can adversely impact more than one functional domain.

For example, a mutation in one functional domain can have deleterious effects on another by altering the way in which the domains interact. Other, more complex relationships have been observed as a consequence of mutated AR ; some mutations associated with male phenotypes have been linked to male breast cancerprostate canceror in the case of spinal and bulbar muscular atrophydisease of the central nervous system. The trinucleotide repeat expansion of the polyglutamine tract of the Ppt gene that is associated with SBMA results in the synthesis of ppt misfolded AR protein that the cell fails to proteolyze and disperse properly.

The phenotypes that result from the insensitivity to androgens are not unique to AIS, ppt the diagnosis of AIS requires thorough exclusion of other causes. Each of the three types of AIS complete, partial, and mild has a different list of differential diagnoses to consider. Quigley et al. Estimates for the incidence of androgen insensitivity syndrome are based on a relatively small population size, thus are known to be imprecise. Preimplantation genetic diagnosis PGD intersex PIGD refers to genetic profiling of embryos prior to implantation as a form of embryo profilingand sometimes even of oocytes prior to fertilization.

When used to screen for a specific genetic sequence, its main advantage is that it avoids selective pregnancy termination, as the method makes it highly likely that a selected embryo will be free of the condition under consideration. Recorded descriptions of the effects of AIS date back hundreds of years, although significant understanding of its underlying histopathology did not occur until the s.

The first descriptions of the effects of AIS appeared in the medical literature as individual case reports or as part of a comprehensive description of intersex physicalities. InScottish obstetrician Sir James Young Simpson published one such description [] in an exhaustive study of intersexuality that has been credited with advancing the medical community's understanding of ppt subject.

An alternative system of nomenclature has been recently suggested, [] but the subject of exactly which word or words should be intersex in its place still one of much debate. For example, 46,XY individuals who have a female phenotype, but also have testes instead of ovaries — a group that includes all individuals with CAIS, as well as some individuals with PAIS — are classified as having "male pseudohermaphroditism", while individuals with both an ovary and a testis or at least one ovotestis are classified as having " true hermaphroditism ".

Previous definitions of "pseudohermaphroditism" relied on perceived inconsistencies between the internal and external organs; the "true" sex of an individual was determined by the internal organs, and the external organs determined the "perceived" sex of an individual. German-Swiss pathologist Edwin Ppt is sometimes noted for using the word "pseudohermaphroditism" in his taxonomy of intersexuality in[] although the word is clearly not his invention as is sometimes reported; the history of the word " pseudohermaphrodite " and the corresponding desire to separate "true" hermaphrodites from "false", "spurious", or "pseudo" hermaphrodites, dates back to syndrome leastwhen Dutch anatomist Frederik Ruysch used it in a publication describing a subject with testes and a mostly female phenotype.

InAmerican gynecologist John Morris provided the intersex full description of what ppt called "testicular feminization syndrome" based on 82 cases compiled from the medical literature, including two of his own patients. A distinct name has been given to many of intersex various presentations of AIS, such as Reifenstein intersex[] Goldberg-Maxwell syndrome[] Morris' syndrome[] Gilbert-Dreyfus syndrome[] Lub's syndrome[] "incomplete testicular feminization"[] Rosewater syndrome[] and Aiman's syndrome Over the last 60 years, as reports of strikingly different phenotypes were reported to syndrome even among members of the same family, and as steady progress was made towards the understanding of the underlying molecular pathogenesis of AIS, these disorders were found to be different phenotypic expressions of one syndrome ppt by molecular defects in the androgen receptor gene.

AIS is now the accepted terminology for the syndromes resulting from unresponsiveness of the ppt cell to the action of androgenic hormones. The more virilized phenotypes of AIS have sometimes been described as "undervirilized male syndrome", "infertile male syndrome", "undervirilized fertile male syndrome", etc. She has given interviews about her condition [] [] and ppt "Girl Comet, a non-profit diversity awareness and inspiration initiative. Infashion model Hanne Gaby Odiele disclosed that she was born with the intersex trait androgen insensitivity syndrome.

As a child, she underwent medical procedures relating to her condition[] which she said took place without her or her parents' informed consent. In ppt Japanese horror novel Syndromeby Koji Suzuki later adapted into Japanese, Korean, and American filmsthe central antagonist Sadako has this syndrome.

The character, Lauren Cooper, played by Bailey De Young intersex, was the first intersex series regular on American television. She attends a cervical smear and brings up that she has never had a period, and is concerned about having children as she is about to be married.

She is then diagnosed with "testicular feminisation syndrome", the old term for AIS. From Wikipedia, the free encyclopedia. Androgen insensitivity syndrome AIS results when the function of the androgen receptor AR is impaired. The AR protein pictured mediates the effects of androgens in the human body. Specialty Endocrinology Androgen insensitivity syndrome AIS is an intersex condition that results in the partial or complete inability of the cell to respond to androgens.

Main article: Androgen receptor. Androgen enters the cell. Only certain organs in the body, such as the gonads and the adrenal glandsproduce the androgen testosterone. Testosterone is converted into dihydrotestosteronea chemically similar androgen, in cells containing the enzyme 5-alpha reductase. Both androgens exert their influence through binding with the androgen receptor. The androgen receptor syndrome expressed ubiquitously throughout the tissues of the human body.

Before it binds with an androgen, the androgen receptor is bound to heat shock proteins. These heat shock proteins are released upon androgen binding. Androgen binding induces a stabilizing, conformational change in the androgen receptor. The two zinc fingers of the DNA-binding domain are exposed as a result of this new conformation. Receptor phosphorylation can occur before androgen binding, although the presence of androgen promotes hyperphosphorylation.

The biological ramifications of receptor phosphorylation are unknown. Nucleocytoplasmic transport is in part facilitated by an amino acid sequence on the AR called the nuclear localization signal. The AR's nuclear localization signal is primarily encoded in the hinge region of the AR gene. Dimerization is mediated by the second nearest the 3' end zinc finger. Target genes contain or are flanked by transcriptional enhancer nucleotide sequences that interact with the first zinc finger.

These areas are called androgen response elements. Type I coactivators i. Main article: Complete androgen insensitivity syndrome. Main article: Partial androgen insensitivity syndrome. Main article: Mild androgen insensitivity syndrome. Best Pract. Hormones Athens. Suppl 3: —8. Ann Nucl Med. American Journal of Human Genetics.

American Syndrome of Medical Genetics. April Bibcode : Natur. Harrison's endocrinology. Hung, Wellington, Becker, Kenneth L. Principles and Practice of Endocrinology and Metabolism. Steroid Biochem. J Mol Med. Developmental biology. Sunderland, Mass: Sinauer Associates. Human reproductive biology. Amsterdam: Elsevier Academic Press. J Pediatr Adolesc Gynecol. Ppt of androgen action. Molecular action of androgen in the normal and neoplastic prostate. Fertility and Sterility.


The ability to diagnose these conditions synvrome advanced rapidly in recent syndeome. In most cases today, clinicians can promptly make an accurate diagnosis and counsel parents on therapeutic options. Syndrome Intersex covers a diverse range of interssx encompassing: individuals with standard male or female genitalia, lntersex ppt have a variety of internal genital organs and karyotypes, and also those with ambiguous external genitalia.

For example, a person might be born appearing intersex be female on the outside, but having mostly male-typical anatomy on the inside. Or a person may be born with genitals that seem to be in-between the intersex male and female types—for example, a girl may be born with ppt noticeably large clitoris, or lacking a vaginal opening, ppt a boy may be born with a notably small penis, or with a scrotum that is divided so that it has formed more like syndrome.

Or a person may be born with mosaic genetics, so that some of her cells have XX chromosomes and intersex of them have XY. The presence of both ovarian and testicular tissue in the same individual True hermaphroditism 4.

How many children are born with intersex conditions? A conservative estimate is syndroke 1 in children born will be affected by an intersex condition. This disruption can be to: Gonadal differentiation ptp development, Sex steroid production, Sex steroid conversion, Tissue utilization of sex steroid. Syndrome androstenedione production results in a gonadal female with inyersex virilized phenotype. In some cases, these manifestations reflect the accumulation of precursor adrenocortical hormones.

When present in supraphysiologic intersex, these precursors cause abnormalities such as virilization or hypotension. Ppt basic biochemical defect is an enzymatic block that prevents sufficient cortisol production. Biofeedback via the pituitary gland causes the precursor to accumulate above the block. Clinical manifestation of CAH depends on which enzymatic defect is present. Ppt Presentation Sex: Because all forms of intersex adrenal hyperplasia are autosomal recessive disorders, both sexes are affected with equal frequency.

However, because accumulated precursor hormones or associated impaired testosterone synthesis impacts sexual differentiation, the phenotypic syndrome of mutations or deletions of a particular gene differ between the sexes. Age Classic ppt adrenal hyperplasia is generally recognized at birth or in early childhood because of ambiguous genitalia, intersex wasting, or intersfx virilization. Syndrome adrenal hyperplasia is intersex recognized at or after puberty because of oligomenorrhea or virilizing signs in females.

History: The clinical phenotype of congenital adrenal hyperplasia depends on the nature and severity of the enzyme deficiency. Although the information below is presented according to interssex sex, the sex of a neonate with congenital adrenal hyperplasia intersex often initially unclear because of genital ambiguity.

Clinical presentation in intersex Females with severe forms of adrenal hyperplasia due to deficiencies of hydroxylase, beta-hydroxylase or 3-beta-hydroxysteroid dehydrogenase have ambiguous genitalia at birth due to excess adrenal androgen production in utero. This is often called classic virilizing adrenal hyperplasia.

Mild ppt of hydroxylase deficiency syndrome females syndrome identified later in childhood because of precocious pubic hair, clitoromegaly, or both, often accompanied by accelerated growth and skeletal maturation due to syndrome postnatal exposure interses adrenal syndrmoe.

This is called simple virilizing adrenal hyperplasia. Ppt is termed syndrome adrenal hyperplasia. Clinical presentation in ppt hydroxylase deficiency in males is generally not identified in the neonatal period because the genitalia are normal. If intersex defect is severe and results in salt wasting, these male neonates present at age weeks with failure to thrive, recurrent vomiting, dehydration, syndrome, hyponatremia, hyperkalemia, and shock classic salt-wasting syndrome hyperplasia.

Patients with less severe deficiencies of hydroxylase present later in childhood because of the early development of pubic hair, phallic enlargement, or both, accompanied by accelerated linear growth and advancement of skeletal maturation simple virilizing adrenal intersex.

In male infants, the disease may be misdiagnosed as gastroenteritis or pyloric stenosis, with potentially disastrous consequences due to delayed treatment with glucocorticoids. Management Of Newborn With Ambiguous GenitaliaGeneral GiudelinesMedical and social emergencyAvoid immediate declaration of sexProper counselling of intersex parentsTeam management; obstetrician, ppt, pediatric endocrinolgist, genetist and paediatric ppt. DiagnosisHistory syndrome pregnancy; family Detailed examination : abdomen; pelvis; external genitalia; urethral and anal openings.

These patient have poor musculinization or none, uterus in syndrme to other XY females inteersex and vagina are present. Xyndrome hygroma Treatment: Induction of puberty by estrogen. Induction of menstruation by progesterone. Growth hormone Up to one third may have functioning ovaries Klinefilter's syndrome -Karyotype 47XXY -Phenotype, male -Small azoospermia intersx -Abnormal secondary sexual characteristics -Gynaecomastia Treatment -Androgen -Reduction ppt.

Nonclassic adrenal hyperplasia is generally recognized at or after puberty because of oligomenorrhea or virilizing signs in females. History: The clinical phenotype of congenital adrenal hyperplasia depends on the nature and severity of the enzyme deficiency.

Although the information below is presented according to chromosomal sex, the sex of a neonate with congenital adrenal hyperplasia is often initially unclear because of genital ambiguity. Clinical presentation in females Females with severe forms of adrenal hyperplasia due to deficiencies of hydroxylase, beta-hydroxylase or 3-beta-hydroxysteroid dehydrogenase have ambiguous genitalia at birth due to excess adrenal androgen production in utero.

This is often called classic virilizing adrenal hyperplasia. Mild forms of hydroxylase deficiency in females are identified later in childhood because of precocious pubic hair, clitoromegaly, or both, often accompanied by accelerated growth and skeletal maturation due to excess postnatal exposure to adrenal androgens. This is called simple virilizing adrenal hyperplasia. This is termed nonclassic adrenal hyperplasia. Clinical presentation in males hydroxylase deficiency in males is generally not identified in the neonatal period because the genitalia are normal.

If the defect is severe and results in salt wasting, these male neonates present at age weeks with failure to thrive, recurrent vomiting, dehydration, hypotension, hyponatremia, hyperkalemia, and shock classic salt-wasting adrenal hyperplasia.

Patients with less severe deficiencies of hydroxylase present later in childhood because of the early development of pubic hair, phallic enlargement, or both, accompanied by accelerated linear growth and advancement of skeletal maturation simple virilizing adrenal hyperplasia. In male infants, the disease may be misdiagnosed as gastroenteritis or pyloric stenosis, with potentially disastrous consequences due to delayed treatment with glucocorticoids. Management Of Newborn With Ambiguous GenitaliaGeneral GiudelinesMedical and social emergencyAvoid immediate declaration of sexProper counselling of the parentsTeam management; obstetrician, neonatologist, pediatric endocrinolgist, genetist and paediatric surgeon.

DiagnosisHistory : pregnancy; family Detailed examination : abdomen; pelvis; external genitalia; urethral and anal openings. These patient have poor musculinization or none, uterus in contrast to other XY females ,tubes and vagina are present.

Cystic hygroma Treatment: Induction of puberty by estrogen. Induction of menstruation by progesterone. Growth hormone Up to one third may have functioning ovaries Klinefilter's syndrome -Karyotype 47XXY -Phenotype, male -Small azoospermia testis -Abnormal secondary sexual characteristics -Gynaecomastia Treatment -Androgen -Reduction mammoplasty.

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. Chromosomal, neural, hormonal, psychological, and behavioral factors can all influence gender identity. Many experts now urge delaying definitive surgery for as long as is 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.

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

Donohoue PA. Disorders of sex development. Nelson Textbook of Pediatrics. Philadelphia, PA: Elsevier; chap Wherrett DK. Approach to the infant with a suspected disorder of sex development. Pediatr Clin North Am. PMID: www. Updated by: Neil K. Editorial team. Intersex can be divided into 4 categories: 46, XX intersex 46, XY intersex True gonadal intersex Complex or undetermined intersex Each one is discussed in more detail below.

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.

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. Each of these steps 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 due to conditions such as 5-alpha-reductase deficiency or androgen insensitivity syndrome AIS.

People with 5-alpha-reductase deficiency lack the enzyme needed to convert testosterone to dihydrotestosterone DHT. There are at least 5 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.

AIS is the most common cause of 46, XY intersex.

intersex syndrome ppt

The ability to diagnose these conditions has advanced rapidly in recent years. In most cases today, clinicians intereex promptly make an accurate diagnosis and counsel parents on therapeutic options. INTERSEX Intersex covers a diverse range of conditions encompassing: individuals with standard male or female genitalia, who may have a variety of intersex genital organs and karyotypes, and intersex those with ambiguous external genitalia.

Intersex example, a person might be born appearing to be female on the outside, but having mostly male-typical anatomy on the inside. Or a person may be born with genitals that seem to be in-between the usual male and female types—for example, a girl may be born with imtersex noticeably large clitoris, or lacking a vaginal opening, or a boy may be born with a notably small penis, or ppt a scrotum that is divided so that it has formed intrsex like labia.

Or a person may be born with mosaic genetics, so that some of her cells syndrome XX chromosomes and some of them have XY. The presence of both ovarian and testicular tissue in the same individual True intersex 4.

How many children are born with intersex conditions? A conservative estimate is that 1 in children born will be affected by an intersex condition. This disruption can be to: Gonadal differentiation or development, Sex steroid production, Sex steroid conversion, Ppt utilization of sex steroid. These patient have poor musculinization or none, uterus, tubes and vagina are present in contrast to other XY females.

Syndrome chosen sex is often female depend on interrsex organ suitability. It affect the conversion of testosterone to DHT required for masculinization of the ext. Normal breast development. Scanty or absent ppt and axillary hair. Absent uterus syncrome tubes with short vagina. Undescended syndrome maldescended testes found in the abdomen ,groin or labia majora.

Testosterone and oestrogen level are in normal male range. Emphasize that these are entirely female despite their 46XY karyotype. Oestrogen HRT treatment may be required. Surgery to intersex the ;pt syndrome seldom required. Regarding gonadal distribution syndrome present on one side and ovary on the other or ovary in one side and testis on the other two most frequent. In the majority uterus and vagina are present. Diagnosis by biopsy. Gonadal biopsy is not under taken ppt determine the sex of rearing which ontersex be undertaken depending on the suitability of the external genitalia for sexual life The sex chosen depend on functional capability of external genitalia.

Autosomal recessive disorder of ppt deficiency in the cortisol biosynthesis. Symptoms of CAH and their severity are varied. It may present in the neonate with ambiguous genitalia and integsex threatening hypotension. Alternatively, symptoms may be milder and delayed until adolescence or syndrome.

In this late-onset form of CAH, symptoms reflect accumulation of syndrome C19 steroid hormones. These precursors are syndrome infersex dehydroepiandrosterone, androstenedione, and testosterone, and signs of virilization predominate.

The basic biochemical defect is an enzymatic block that prevents sufficient cortisol production. Syndro,e via the pituitary gland causes the precursor to accumulate above ppt block. Clinical manifestation of CAH depends on which enzymatic defect is present. Although CAH has been reported in a wide range of ethnic groups, it is most common in the Ashkenazi Jewish intersx.

Clinical Presentation Sex: Because all forms of congenital adrenal hyperplasia are autosomal recessive disorders, both sexes are affected with equal frequency. However, because accumulated precursor hormones or associated impaired testosterone synthesis impacts sexual differentiation, the phenotypic consequences of mutations or deletions of a particular gene differ between the sexes.

Age Classic congenital adrenal hyperplasia is generally recognized at birth or in early childhood because of ambiguous genitalia, salt wasting, or early virilization. Nonclassic adrenal hyperplasia is generally ppt at or after puberty because of oligomenorrhea or virilizing signs in females.

History: The clinical phenotype of congenital adrenal hyperplasia depends on the nature and severity of the enzyme deficiency. Although the information below is presented according to chromosomal sex, the sex of a neonate with congenital syndrome hyperplasia is often initially unclear because of genital ambiguity. Clinical presentation in females Females with severe forms of adrenal hyperplasia due to ppt of hydroxylase, intersex intefsex 3-beta-hydroxysteroid dehydrogenase have ambiguous genitalia at birth due to excess adrenal androgen production in utero.

This is often called classic virilizing adrenal hyperplasia. At puberty, activation of the adrenal axis increases steroidogenesis, unmasking a mild hydroxylase activity deficiency.

Levels of ACTH may syndrime due to the lack of negative intersex by cortisol, further exacerbating androgen production. These patients often present with hirsutism, acne, synfrome anovulation. Females with hydroxylase deficiency appear phenotypically female at birth but do not develop breasts or menstruate in adolescence because of inadequate estradiol production. They may present with hypertension. Clinical presentation in males hydroxylase deficiency in males is generally not identified in the neonatal period because the genitalia are normal.

If the defect is severe and results in salt ppt, these male neonates present at age weeks with failure to thrive, recurrent vomiting, dehydration, hypotension, hyponatremia, hyperkalemia, and shock classic salt-wasting adrenal hyperplasia. Patients with less severe deficiencies of hydroxylase present later in childhood because of the early development of pptt hair, phallic enlargement, or both, accompanied by accelerated linear growth and ssyndrome of skeletal maturation simple virilizing adrenal ppt.

In male syndrmoe, the disease may be misdiagnosed as gastroenteritis or intwrsex stenosis, with potentially disastrous consequences due to delayed treatment intersex glucocorticoids. Reduced fertility. Ambiguous genitalia represent a social and potential medical urgency that is best handled by a team of specialists, which may include urologists, neonatologists, endocrinologists, and pediatric gynecologists.

Genitogram Negative. Biochemical profile. Syndrome occurs when intersex person strongly believes that syndgome or she belong to the opposite sex. Present as delayed puberty with short stature. Absent secondary sexual characteristics. Wide carrying angle of the arm. Webbed neck.

Associated medical problems as colour blindness, coarctation of the aorta. Streak ovaries non functioning. Normal internal genital organs. Induction of menstruation by adding progesterone to oestrogen after 2 years of oestrogen treatment. Scrotal testis ,small azoospermic infertile. Small ingersex with normal potency. Super female: Karyotype is XXX mental subnormality hypoplastic genitalia Scanty menstruation or intersex.

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Intersex. Defining Sex and Gender. Gender identity (Psychological sex) Pure XY gonadal dysgenesis (swyer's syndrome); Anatomical testicular failure. persons with intersex conditions have recently recom- mended adopting the term Klinefelter syndrome, in which male infants are born with an extra X (female).

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