Flashcards in Severson's abnormal genital development; 4.7 Deck (21):
What causes Klinefelter syndrome?
Non-dysjunction of the XX homologues → 47, XXY
What is seen with Klinefelter syndrome?
Impaired sexual maturation (atrophic testes, small penis, and lack of secondary male characteristics)
What is seen with Turner syndrome?
High arched palate
Cardiac and renal anomalies
What causes Turner syndrome?
Lack of paternal sex chromosome (45, XO) → failure of ovaries to develop and infantile genitalia
True hermaphroditism is a very rare condition. What is needed to be a true hermaphrodite?
Both testicular and ovarian tissue (46, XX)
What happens with cryptorchidism?
The testicle gets "stuck" on its descent
Where is a testicular most likely to get stuck with cryptorchidism?
The inguinal canal
What causes an incomplete congenital inguinal hernia?
Obliterated portion of processus vaginalis
What causes a complete congenital inguinal hernia?
Unclosed processus vaginalis
What is seen with complete congenital inguinal hernias?
What causes a hydrocele?
Obliterated portions or unclosed processus vaginalis
What is seen with a male pseudohermaphrodite?
Reduced masculine development of the genitalia
What might be the cause of male pseudohermaphroditism?
Inadequate amount of testosterone or Mullerian inhibiting substance
What is it called when the urethra opens on the ventral surface of the penis?
What causes hypospadias?
Inadequate androgen (likely) → failure in fusion of urogenital folds and/or failure of epithelial cord to join with the spongy urethra
What is it called when the urethra opens on the dorsal surface of the penis?
What is associated with epispadias?
Exstrophy of the bladder
What happens with Congenital Adrenal Hyperplasia (CAH)?
CAH → increased androgenic hormones → female pseudohermaphroditism (46, XX)
What is seen with CAH?
Severe clitoral hypertrophy
Fusion of labia
Female internal organs
What causes Androgen Insensitivity Syndrome (AIS)...AKA Testicular Feminization Syndrome?
Ineffective (lack of) testosterone receptors