Puberty Disorders Flashcards
Adrenarche
Adrenal equiv of puberty
Increased production androgens from zona reticularis
Female Puberty Times
Early: 8 (under AA & hispanic)
Late: 13
Tanner Female
I: No hair or breast
II: hair just on labia, breast just where areola
III: sparsely full covered, breast round contour
IV: full covered, double contour
V: spread to thigh, round contour
Male Puberty times
Early: 9
Late: 14
Tanner Male
I: no hair II: at base III: covering more IV: triangle V: diamond
Kallman Syndrome
Defect in GnRH pulse generator or gonadotrophs + anosmia
FHx or kidney defects
Turner Syndrome
XO. Short stature, ovarian failure in 94%.
High FSH & LH
Klinefelter Syndrome
XXY. No sertoli, but have Leydig.
Microphallus, small testes, gynecomastia, infertility
High FSH & LH
Central Precocious Puberty Causes
hypothalamic hamartoma (most common); suprasellar tumor, hydrocephalus, previous CNS infection, major head trauma, cranial irradiation
Ovarian Cysts
Breast bud development but no pubic hair or bone deve
Usually present w/vaginal bleeding
McCune-Albright synd
activating mutation of alpha subunit of Gs protein. Can have excess of other hormones
Triad of precocious puberty, café- au-lait spots (restricted to dermatome) and polyostotic fibrous dysplasia
Adrenal tumor males
Small testes, more public hair
Leydig cell tumor
Unilateral increase in testes
More hair
hCG secreting tumor
bilaterl increase in testes w/ more hair growth
Familial testotoxicosis
Autosomal Dominant mutation of LH receptor so that it is constitutively active
Bilateral increase testes, penile growth, more pubic hair