Disorders of Puberty and Sexual Dev Flashcards
Neurokinin B _____ regulates Kisspeptin
Positively
Testes Greater than ____ is puberty
3ccs
What tanner stage does Male voice chaing?
Tanner 4
2 Genetic causes of GnRH-dependent Precocious Puberty
- Kisspeptin Receptor activation
- NF-1 Mutation (neurofibromin)
(SMOOTH cafe-au-lait spots, iris hamartoma, CNS tumors)
Delayed male puberty is lack of testicular enlargement by age ____
- 14
Pregnant women who have placental aromatase deficiency are unable to produce estradiol and estriol from their precursors,_____ and_____, which come from the fetus. Due to the increase in precursor levels, both mothers with placental aromatase deficiency and their female fetuses may have some signs of androgen excess
- DHEA sulfate
- 16-alpha-DHEA sulfate
Kisspeptin is negatively regulated by _____ to inhibit GnRH firing
Dynorphin
Name 2 GnRH INdependent Activating Receptor Mutation diseases
- McCune-Albright Syndrome
2. Familial Testotoxicosis
____ Syndrome females often have lymphedema, shield chest, and HYPERgonadotropic hypogonadism
Turner’s
McCune Albright has ____ levels of FSH/LH
LOW
GnRH releases in a pulsatile manner, and the GnRH neurons express _____ receptor which is believed to regulate the firing
KISS1
Kallman syndrome is _____, where as Klinefelter is ____ hypogonadism
- HYPO
- HYPER
Kisspeptin is positively regulated by _____ to promote GnRH firing
Neurokinin B
NF-1 Precocious puberty has ____ levels of FSH/LH
HIGH
in regards to GnRH, what are the 3 components to the KNDy system?
- Kisspeptin
- Neurokini B
- Dynorphin
How to differentiate female Tanner 3 from Tanner 4
- Tanner 4 has raised areola mound
How to differentiate tanner 4 from tanner 5
- Tanner 4 has no pubic hair in thighs
Key difference of Male Tanner 3 from Tanner 4
Tanner 5 has voice change
McCune-Albright vs NF-1 brown spots comparison
- McCune has rough, irregular borders that DON’T cross the midline
- NF-1 are smooth and appear anywhere
Dynorphin ____ regulates Kisspeptin
Negatively
How many tanner stages are there?
5
The onset of breast enlargement is known as ____
Thelarche
____ Syndrome males often have failure of testes to grow normally, have eunuchoid proportions, and are tall. Often times diganosis is made at age 30. Look for small testes and ocassionally osteoporosis.
Klinefelter’s
Aside from Brown spots in McCune-Albright, what are 2 outcomes from the over-expressed GNAS gene/G-protein signaling?
- Bone dysplasia (polyostotic fibrous)
2. Sexual Precocity (GnRH-independent)
Thelarche
Onset of breast enlargement
Kisspeptin links to KISS1 receptors to regulate the ______ activity
GnRH Neuron
Delayed female puberty is lack of breast development by age ____ or lack of menarche by age ____
- 13
- 16
How to identify Tanner stage 2?
Pubic hair appears
Familial Testotoxicosis occurs from a _____ receptor Activating mutation
LH
