Puberty, Disorders of Development, and Menstrual Disorders (Moulton) Flashcards
What refers to o the development of any sign of secondary sexual characteristics prior to an age 2.5 standard deviations earlier then the expected age of pubertal onset?
Precocious puberty
__1__ precocious puberty is characterized by development of secondary sexual characteristics opposite those of anticipated phenotypic sex.
__2__ precocious puberty is characterized by premature sexual maturation that is appropriate for the phenotype of the affected individual.
1) Heterosexual
2) Isosexual
What virilizing tumor can cause hetersexual precocious puberty, is exceedingly rare in childhood, and usually originates in the ovaries?
What do they secrete?
1) Sertoli-Leydig cell
2) Androgens
Congenital adrenal hyperplasia which can cause heterosexual precocious puberty, most commonly results from defect of the the adrenal enzyme ____ leading to excessive androgen production.
21-hydroxylase
True isosexual precocious puberty can be diagnosed with administration of exogenous __1__ and see a resultant rise in __2__ levels consistent with older girls who are undergoing normal puberty.
1) GnRH
2) LH
What is the treatment for true isosexual precocious puberty?
This suppresses the pituitary release of?
1) GnRH agonist (leuprolide acetate)
2) FSH and LH
Pseudoisosexual precocity results in increase __1__ levels and cause sexual characteristic maturation without activation of __2__.
1) Estrogen
2) HPO axis
A cause of pseudoisosexual precocity is ____, which presents as multiple cystic bone defects, café au lait spots, and adrenal hypercortisolism.
McCune-Albright syndrome (Polyostotic fibrous dysplasia)
A cause of pseudoisosexual precocity is ____, which is associated with a sex cord tumor that secretes estrogen, gastrointestinal polyposis, and mucocutaneous pigmentation.
Peutz-Jeghers syndrome
What conditions can cause primary amenorrhea due to hypogonadotropic hypogonadism?
1) Anorexia nervosa
2) Kallmann syndrome
Kallmann syndrome is characterized by a mutation of the KAL gene on the X chromosome that prevents the migration of the __1__ neurons into the __2__.
1) GnRH
2) Hypothalamus
What condition can cause primary amenorrhea due to hypergonadotropic hypogonadism?
Turner syndrome (45 XO)
Turner syndrome is the most common form of female ____.
Gonadal dysgenesis
What are some findings associated with Turner’s syndrome?
1) Webbing of the neck
2) Broad flat chest
3) Short stature
4) Rudimentary streaked ovaries
5) Coarctation of the aorta
What conditions can cause primary amenorrhea with breast development?
1) Androgen insensitivity syndrome (AIS)
2) Mullerian agenesis