Usmle 1 Genitalia

• ▪ Testes
• ▪ Vas deferens
• ▪ Seminal vesicles
• ▪ Epididymis

• ▪ Ovaries
• ▪ Fallopian tube
• ▪ Uterus
• ▪ Upper vagina

• ▪ Urogenital Sinus
•   ▪ Prostate
•   ▪ Prostatic urethra
•   ▪ Bulbourethral Gland

• ▪ Urogenital tubercule
•   ▪ Penis

• ▪ Labioscrotal swelling
•   ▪ Scrotum

• ▪ Urogenital Sinus
•   ▪ Labia vagina
•   ▪ Labia minora

• ▪ Urogenital tubercule
•   ▪ Clitoris

• ▪ Labioscrotal swelling
•   ▪ Labia majora

▪ A male requires MIF in order to develop INTERNAL GENITALIA

▪ Any XY with internal female genitalia has an MIF deficiency

• ▪ A male must have testosterone to have EXTERNAL GENITALIA
•    ▪ If  XY with external female genitalia has testosterone problem

▪ Remember male/female is based on GENOTYPE

▪ Penis one day → gone the next!!!

▪ Disorder of the androgen receptor

• ▪ The internal genitalia does not equal the genotype
•     ▪ MIF problem
•         ▪ XX with male internal genitalia

• ▪ External genitalia does not match genotype
•     ▪ XX with external genitalia

• ▪ Testes never descended → commonly found in the inguinal canal
•     ▪ If not descended into scrotum by 15 months = permanently injured

• ▪ NO Leydig cells à no external genitalia à sterile
• ▪ Management
•     ▪ Observe for 6 months
•     ▪ IF not descended refer to urologist for GnRH injections
•     ▪ IF not down in 1 year → go to surgery
•        ▪ Staple the teste down → orcypecsy.
•        ▪ Risk of testicular cancer
•          ▪ Yolk Sac cancer è most common testicular cancer in a child
•          ▪ (­↑ a-feta protein)

• ▪ Orchitis
•     ▪ d/t no more testosterone

▪ Needs 5 α-reductase

• ▪ Deficiency of 5α-reductase is inherited as autosomal recessive disease
•    ▪ DHT receptor not working

• ▪ Will present with testicular feminization
•     ▪ Drugs that could cause the same thing:
•       ▪ Fenesteride (for BPH)
•       ▪ Ketoconazole
•       ▪ Spirolactone

▪ These drugs also can cause gynecomastia

• ▪ Block DHT (dihydrotestosterone) receptors
•     ▪ Flutamide (used for testicular CA)
•     ▪ Cypropterone

• ▪ Testicular Feminization
•    ▪ Blind pouch vagina
•    ▪ Problem will not occur until puberty
•    ▪ (Sexual identity is set by age 3)
•        ▪ Jamie Lee Curtis

• ▪ Sertoli Only syndrome
•     ▪ No Leydig cells to make testosterone