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Disorders of Sexual Differentiation Pediatric GU Review UCSD Pediatric Urology George Chiang MD Sara Marietti MD Outlined from  The Kelalis-King-Belman Textbook of Clinical Pediatric Urology 2007 (not for reproduction, distribution, or sale without consent)
 
Outline ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Chromosomal sex Gonadal sex Phenotypic sex Sexual Differentiation
Chromosomal Sex ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
“ Development” TDF SRY Testis Testosterone Müllerian Inhibiting  Substance Penis  Vas Deferns Prostate Uterus Fallopian Tubes Upper Vagina
Testicle  Differentiation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Ovarian  Differentiation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Board Questions: ,[object Object],[object Object],Gartner’s Duct Prostatic Utricle Appendix Testes Appendix Epididymis Epoophoron
 
 
 
Male Phenotypic Sexual Differentiation ,[object Object],[object Object],[object Object],[object Object],[object Object]
Female Phenotypic Sexual Differentiation ,[object Object]
 
Tanner Stages
Classification ,[object Object],[object Object],[object Object],[object Object]
Hughes IA, Houk C, Ahmed SF, Lee PA. Consensus statement on the management of intersex disorders. Archives of  Disease in Childhood 2006 ,[object Object],[object Object]
HWA Protests Intersex Genital Mutilation [Washington, DC: 21 Apr 98] A dozen members of Hermaphrodites With Attitude and Transexual Menace picketed outside the George Washington University Hospital,  protesting GWU's cutting of intersexed infants' genitals to make them resemble 'normal' male and female genitals . Hermactivists call this medical intervention Intersex Genital Mutilation (IGM).  " Not only are American doctors cutting into 7 intersexed infants every working day,  but people like GWU's Dr. Barry Belman are telling parents they have to do it for the good of the infant ,"  said Cheryl Chase, founder of HWA. "Mutilating my genitals wasn't for my good; it was to make the doctors and my parents more comfortable with my body."  After the demo, several protestors visited GWU Hospital departments that take part in IGM--OB/GYN, Endocrinology, Pediatrics and Plastic Surgery--to discuss their concerns with caregivers and, as one hermactivist put it, to speak for the infants who cannot speak for themselves.  From In Your Face on-line news, April 1998
First and foremost, we recommend avoidance of harmful or unnecessary genital surgery on infants and children. No surgery should be performed  unless it is absolutely necessary for the physical health and comfort of the intersexual child . We believe any surgery that does not meet these criteria to be essentially elective cosmetic surgery which should be deferred until the intersexual child is able to understand the risks and benefits of the proposed surgery and is able to provide appropriately informed consent.
Disorders of Sex Development ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Intersex ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Wide range of phenotypes
Disorders of Gonadal Differentiation ,[object Object],[object Object],[object Object],[object Object],[object Object]
Klinefelter’s ,[object Object],[object Object],[object Object],[object Object],[object Object]
Turner’s Syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],Mixed Gonadal Dysgenesis
[object Object],[object Object],[object Object],[object Object],[object Object],Mixed Gonadal Dysgenesis
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Mixed Gonadal Dysgenesis
Pure Gonadal Dysgenesis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Embryonic Testicular Regression and Bilateral Vanishing Testes ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
True Hermaphrodite ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Female Pseudohermaphrodite ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Female Pseudohermaphrodite ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
21-Hydroxylase deficiency Testosterone
FEMALE CAH
Female CAH
Female CAH
 
[object Object],[object Object],[object Object],[object Object],Male Pseudohermaphrodite
Male Pseudohermaphrodite ,[object Object],[object Object],[object Object]
Male Pseudohermaphrodite ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Male Pseudohermaphrodite ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Male Pseudohermaphrodite ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Male Pseudohermaphrodite ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],Common  Nenonatal  Presentations
 
 
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Case #1
Case #1
Physical Exam ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Workup? ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Workup?
Plan? ,[object Object],[object Object],[object Object]
Voiding Cystourethrogram
Case #2 ,[object Object],[object Object],[object Object],[object Object]
 
 
Workup/Plan? ,[object Object]
Case 3 ,[object Object]
Case 3
QUESTIONS
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

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PEDI GU REVIEW intersex

  • 1. Disorders of Sexual Differentiation Pediatric GU Review UCSD Pediatric Urology George Chiang MD Sara Marietti MD Outlined from The Kelalis-King-Belman Textbook of Clinical Pediatric Urology 2007 (not for reproduction, distribution, or sale without consent)
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  • 4. Chromosomal sex Gonadal sex Phenotypic sex Sexual Differentiation
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  • 6. “ Development” TDF SRY Testis Testosterone Müllerian Inhibiting Substance Penis Vas Deferns Prostate Uterus Fallopian Tubes Upper Vagina
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  • 19. HWA Protests Intersex Genital Mutilation [Washington, DC: 21 Apr 98] A dozen members of Hermaphrodites With Attitude and Transexual Menace picketed outside the George Washington University Hospital, protesting GWU's cutting of intersexed infants' genitals to make them resemble 'normal' male and female genitals . Hermactivists call this medical intervention Intersex Genital Mutilation (IGM). " Not only are American doctors cutting into 7 intersexed infants every working day, but people like GWU's Dr. Barry Belman are telling parents they have to do it for the good of the infant ," said Cheryl Chase, founder of HWA. "Mutilating my genitals wasn't for my good; it was to make the doctors and my parents more comfortable with my body." After the demo, several protestors visited GWU Hospital departments that take part in IGM--OB/GYN, Endocrinology, Pediatrics and Plastic Surgery--to discuss their concerns with caregivers and, as one hermactivist put it, to speak for the infants who cannot speak for themselves. From In Your Face on-line news, April 1998
  • 20. First and foremost, we recommend avoidance of harmful or unnecessary genital surgery on infants and children. No surgery should be performed unless it is absolutely necessary for the physical health and comfort of the intersexual child . We believe any surgery that does not meet these criteria to be essentially elective cosmetic surgery which should be deferred until the intersexual child is able to understand the risks and benefits of the proposed surgery and is able to provide appropriately informed consent.
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  • 23. Wide range of phenotypes
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