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Unit 3 – GENDER DEVELOPMENT. Question in pairs. If you were a parent and your child was just born a girl but with what looked like a penis or with “unusual genitalia” what would you do?.

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    2. Question in pairs.. If you were a parent and your child was just born a girl but with what looked like a penis or with “unusual genitalia” what would you do?

    3. Disorders of sex development (DSDs).(Intersex conditions)1. Hermaphrodites2. AIS (androgen insensitivity syndrome)+ (CAIS)(PAIS)3. CAH (congenital adrenal hyperplasia)4. DHT- (Dihydrotestosterone)5. Turner’s Syndrome6. Klinefelter’s syndrome

    4. There is the view that the hormones that you are exposed to in the womb have a large impart on your brain development and therefore your gender identity.

    5. For example testosterone has been linked to the masculinisation of the brain such as the areas linked to spatial skills. Similarly the female oestrogen plays a role in feminising the brain.

    6. In rare cases, the hormones which trigger the development of sex and gender may not work properly on the brain, gonads, and genitals, causing variations between them. For example, the sex (as determined physically by the gonads and genitals) could be male, while the gender (as determined by the brain) could be female.

    7. This occurs in 1 in 4,500 live births such an individual is termed intersex.

    8. Intersex is a group of conditions where there is a discrepancy between the external genitals and the internal genitals (the testes and ovaries). click for picture of ambiguous genitalia of baby with XY sex chromosomes.

    9. Although the older terms are still included in this article for reference, they have been replaced by most experts (and patients and families) because they are misleading, confusing, and insensitive.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).

    10. Examples of Hormones Activity – Poster class teach Intersex conditions • Do poster then teach one gender condition to another group • As above • As above • As above

    11. For info

    12. Intersex can be divided into four categories: • 46, XX Intersex • 46, XY Intersex • True Gonadal Intersex • Complex or Undetermined Intersex For more details visit extra info

    13. Intersex conditions cause babies to be born with the genitalia of both sexes (or ambiguous genitalia). In such cases, it was recommended that the child’s parents should choose which gender to bring up their child. However, it is now thought to be better to wait until the child can choose their own gender identity before any surgery is carried out to confirm it. http://medlineplus.gov/

    14. 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.

    15. 1. Hermaphrodites Are very rare they have a combination of XX and XY cells in their bodies. The karyotype of a true hermaphrodite is classified into 46, XX; 46, XY. Mr Blackwell is the 303rd recorded hermaphrodite who on surgery was found to have had an active ovary on one side, with a small uterus, and an active testicle on the other.He also had a penis and something like a vaginal opening.

    16. It would only have taken a small difference in his internal ducts, (we are told by the newspaper reporter), and he would have fertilised himself without being able to control it. On attending a hospital he opted to live as a man, and the ovary was removed. This was because he had been raised as a male and identified as such.

    17. Pseudo- Hermaphrodites Are more common they have gonads they do not match their chromosomes. A state in which an individual possesses the internal reproductive organs of one sex while exhibiting some of the external physical characteristics of the opposite sex.

    18. AIS (androgen insensitivity syndrome) Are born genetically male with the sex chromosomes (XY). The presence of the Y chromosome stops the development of internal female sexual organs and causes the development of testes (two egg-shaped male reproductive organs), which also happens in normal male sexual development. The testes produce male hormones, called androgens, which normally cause male sex organs (such as the penis) to develop.

    19. However, in AIS, the body ignores the androgen or is insensitive to it, so the genitals develop along female lines and the testes usually remain inside the body.There are two forms of AIS, which are determined by the level of insensitivity to androgen.

    20. 1- Complete androgen insensitivity syndrome (CAIS) This is where the person is totally insensitive to androgen and develops external genitals that are entirely female. Most children born with CAIS are brought up as girls. They will develop breast at puberty and have a vagina.

    21. Their vagina is sometimes referred to as blind because there is no uterus or ovaries ‘attached’. Consequently they will not menstruate and cannot have children.

    22. Partial androgen insensitivity syndrome (PAIS) • This is where the person has some sensitivity to androgen. The level of sensitivity determines how the genitals develop. The person can look almost entirely male, entirely female, or somewhere in between.

    23. Some babies are born with a slightly enlarged clitoris, while others may have an almost fully formed penis. Children with PAIS can be brought up as girls or boys. The decision is often based on the type of genital development.

    24. In some cases, babies with PAIS may be raised as boys but become more female as they develop during puberty. Hormone therapy can sometimes be used to "direct" puberty towards a certain gender. Or, treatment can be used to delay puberty until a child is old enough to make an informed decision about their preferred gender identity.

    25. However all girls born with androgen insensitivity syndrome (AIS) will be infertile, as the development of the female internal organs is suppressed by male hormones from the testes in the body. Girls born with AIS have no womb, ovaries or fallopian tubes while boys are also likely to be infertile, unless their androgen sensitivity is very mild.

    26. 2. CAH (congenital adrenal hyperplasia)

    27. Individuals with CAH (congenital adrenal hyperplasia)... have an inherited disorder of the adrenal gland (two small, triangular-shaped glands located above the kidneys), They lack an enzyme needed by the adrenal gland to make the hormones cortisol which affects many different body systems.

    28. It plays a role in: • Bone • Circulatory system • Immune system • Metabolism of fats, carbohydrates, and protein • Nervous system • Stress responses • and aldosterone (It helps the body regulate blood pressure). • Animation clip.

    29. Congenital adrenal hyperplasia can affect both boys and girls who produce more androgen, a type of male sex hormone. This causes male characteristics to appear early (or inappropriately). About 1 in 10,000 to 18,000 children are born with congenital adrenal hyperplasia. Girls will usually have normal female reproductive organs (ovaries, uterus, and fallopian tubes).

    30. They may also have the following changes: • Abnormal menstrual periods • Deep voice • Early appearance of pubic and armpit hair • Excessive hair growth and facial hair • Failure to menstruate • Genitals that look both male and female (ambiguous genitalia), often appearing more male than female extra information

    31. Some females with CAH have enlarged genitals to the extent that in some cases, they may be thought to be male when she is born. (Cheryl Chase). Most research evidence looks at females with this condition (Hines 2004). CAH clip

    32. Boys won't have any obvious problems at birth. However, they may appear to enter puberty as early as 2 - 3 years of age. Changes may include: • Deep voice • Early appearance of pubic and armpit hair • Early development of male characteristics • Enlarged penis • Small testes • Well-developed muscles

    33. Both boys and girls will be tall as children but much shorter than normal as adults. • The goal of treatment is to return hormone levels to normal. This is done by taking a form of cortisol (dexamethasone, fludrocortisone, or hydrocortisone) every day. People may need additional doses of medicine during times of stress, such as severe illness or surgery.

    34. The health care provider will determine the gender of a baby with ambiguous genitalia by checking the chromosomes (karyotyping). Girls with male-looking genitals will usually have surgery between ages 1 month - 3 months to correct the abnormal appearance.

    35. Parents of children with congenital adrenal hyperplasia should be aware of the side effects of steroid therapy. Report signs of infection and stress to your health care provider because the child may need more medication. Steroid medications cannot be stopped suddenly, because it may lead to adrenal insufficiency.

    36. Outlook (Prognosis) • People with this condition usually have good health. However, they may be shorter than normal, even with treatment. • Males have normal fertility. Females may have a smaller opening of the vagina and lower fertility.

    37. (Prognosis) continued • People with this disorder must take medication their entire lives. • Untreated, adrenal crisis can lead to death within 1 - 6 weeks after birth.

    38. Support Groups (these may be useful for essay information • National Adrenal Diseases Foundation: www.medhelp.org/nadf/ or The MAGIC Foundation: www.magicfoundation.org and The CARES Foundation: www.caresfoundation.org

    39. Additional info ..

    40. 4. DHT- (Dihydrotestosterone)- • deficient males have a rare genetic condition which means that they are born with what appears to be female genitalia and as a result they are raised as girls because DHT is responsible for the formation of male primary sex characteristics during embryonic life.

    41. This rare condition is mostly found in the village of Salinas in the Dominican Republic which is in the West Indies where all ‘suffers’ are thought to share a common ancestor.

    42. Dihydrotestosterone (DHT) is considered to be the essential androgenic hormone responsible for the development of male characteristics, including body hair, the genital organs and muscle mass.

    43. Babies born with this condition do not develop male characteristics until the onset of puberty when high levels of DHT are released; this causes their voices to deepen, muscle mass to increase, testes to descend and the clitoral like phallus to enlarge to a fully functional penis and their vaginal opening to close up.

    44. Babies born with this condition do not develop male characteristics until the onset of puberty when high levels of DHT are released; this causes their voices to deepen, muscle mass to increase, testes to descend and the clitoral like phallus to enlarge to a fully functional penis and their vaginal opening to close up.

    45. DHT continued This suggests that DHT is necessary for the complete emergence of male features and gender identity. This suggests that exposure to testosterone in the womb and at puberty is the dominant factor in determining gender identity. This has been suggested by Imperato- MCGinley et al (1974) as a result of their case study of 24 individuals aged 18 months to 60 years.

    46. There are a number of other chromosomal abnormalities that cause a discrepancy between the chromosomal sex and appearance of an individual.

    47. 5. Turner’s Syndrome; is a genetic condition that only affects females. • In 1938, Dr. Henry Turner published a report describing seven girls who shared a number of features, including short stature, lack of sexual development, cubitus valgus (arms that turn out slightly at the elbow), webbing of the neck and a low hairline in the back. The condition he described came to be known as Turner’s syndrome or TS.

    48. In 1959, Dr. C.E. Ford discovered that the cause of TS is a chromosomal (genetic) condition involving the sex chromosomes. He observed that most girls with TS he examined did not have all or part of one of their X chromosomes, and he suggested that the missing genetic material accounted for the physical findings observed in this condition.

    49. The diagnosis of TS is made on the basis of a blood test called a karyotype analysis, a picture taken under a microscope of the chromosomes in a person’s cells. TS is one of the most common genetic conditions, affecting 1 of every 2,000 to 2,500 girls.

    50. Fortunately, most girls with TS can expect to lead healthy, productive and happy lives. The condition is caused by an abnormal chromosome and affects about one in every 2,500 baby girls. This condition (TS ) occurs when one of the two X chromosomes that are found in females is completely or partially missing known as an XO karyotype.