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UROGENITAL TRACT Male Development

UROGENITAL TRACT Male Development. RICHARD SCHLUSSEL, M.D. DEPARTMENT OF UROLOGY DIVISION OF PEDIATRIC UROLOGY. Genital Development. Chromosomal sex Gonadal sex Phenotypic sex. Week 5. Chromosomal sex Gonadal sex Phenotypic sex. Chromosomal sex. SRY gene S ex determining

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UROGENITAL TRACT Male Development

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  1. UROGENITAL TRACTMale Development RICHARD SCHLUSSEL, M.D. DEPARTMENT OF UROLOGY DIVISION OF PEDIATRIC UROLOGY

  2. Genital Development • Chromosomal sex • Gonadal sex • Phenotypic sex

  3. Week 5

  4. Chromosomal sex • Gonadal sex • Phenotypic sex

  5. Chromosomal sex • SRY gene Sex determining Region of the Y chromosome - transcription factor SOX 9

  6. Gonadal sex

  7. Gonadal sex • Sertoli cells • Forms week 6 • Organizes testis into cords • Secrete AMH week 8 Anti Mullerian Hormone • Leydig cell development

  8. Gonadal sex • Sertoli cells • Forms week 6 • Organizes testis into cords • Secrete AMH week 8 Anti Mullerian Hormone • Leydig cell development

  9. AMH

  10. Leydig cells Secrete Testosterone Week 9-10 Preserve mesonephric ducts Regulated by chorionic gonadotropin Placenta Pituatary Gonadal sex

  11. Chromosomal sex • Gonadal sex • Phenotypic sex • Internal genitalia (T) • External genitalia (DHT)

  12. Phenotypic sex • Androgens • Testosterone (T) • Dihydrotestosterone (DHT) • Androgen receptors

  13. Testicular descent

  14. Normal Male Genitalia

  15. Disordered development • Klinefelter’s syndrome • XX males • Male pseudohermaphrodite • Testicular feminization • Persistent Mullerian duct syndrome • Hypospadias • Undescended testes

  16. Klinefelter’s syndrome • Chromosomal sex • Failure meiosis • Disjunction • XXY • Gonadal sex • Phenotypic sex

  17. Klinefelter’s syndrome XXY • Small firm testes • Gynecomastia • Seminiferous tubular sclerosis • High gonadotropins FSH • 1 of 600 males

  18. Hermaphrodite (Intersex) • “An individual having the reproductive organs and many of the secondary sex characteristics of both sexes” • “a discrepancy between the external genitals and the internal genitals” • Hermaphroditus • the son of Hermes and Aphrodite • was fused with a nymph

  19. Male pseudohermaphrodite • Chromosomal sex • Gonadal sex • Phenotypic sex

  20. Male pseudohermaphrodite 5α-reductase deficiency XY chromosomes!!

  21. Testicular feminization(Androgen insensitivity) • Chromosomal sex • Gonadal sex • Phenotypic sex

  22. Testicular feminization(Androgen insensitivity) XY chromosomes!!

  23. Hypospadias • Abnormal androgen production • Abnormal androgen sensitivity • Arrested development • Estrogen exposure ? ? ?

  24. Hypospadias

  25. Hypospadias

  26. Hypospadias

  27. Undescended Testes 3 MONTHS 7 MONTHS • -Gubernaculum • Endocrine • - Insulin like factor3 • Genitofem nerve • CGRP (calcitonin • gene Related • Protein) 9 MONTHS

  28. Undescended Testes • Infertility • Neoplasia • Cosmesis • Testis torsion

  29. Chromosomal sex • Gonadal sex • Phenotypic sex

  30. Chromosomal sex • Gonadal sex • Phenotypic sex

  31. Persistent mullerian duct

  32. Figure 106-20 Formation of genital ridges and paramesonephric ducts. A, During the 5th and 6th weeks, the genital ridges form in the posterior abdominal wall just medial to the developing mesonephros. The primordial germ cells induce the coelomic epithelial cells lining the peritoneal cavity and the cells of the mesonephros to proliferate and form the primitive sex cords. B, During the 6th week, the paramesonephric ducts develop lateral to the mesonephros. The caudal tips of the paramesonephric ducts fuse with each other as they connect with the urogenital sinus. (Modified from Larsen WJ: Human Embryology. New York, Churchill Livingstone, 1997.)

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