REI Flashcards
Sexual development
The normal and predictable sequence of sexual maturation proceeds with breast budding, then adrenarche (hair growth), a growth spurt and then menarche. In a minority of cases, pubarche (pubic hair growth) can occur before thelarche (breast/areolar development). Breast development begins around the age of 10 and average age of menarche is 12.7 years for Caucasian girls and 12.1 for Black girls. Menarche also occurs earlier for heavier girls and later for thinner, physically active girls
Weight and nml age for menarche
body weight of 85 to 106 pounds is needed before menses begin
Normal age for menarche is between nine and 17.
Turner syndrome
The genetic defect of Turner syndrome is the absence of one of the X chromosomes. These females have failure to establish secondary sexual characteristics, short stature and characteristic physical features: pterygium colli, shield chest and cubitus valgus; don’t start menses
Suspect kallman syndrome
Kallmann syndrome is characterized by olfactory tract hypoplasia and the arcuate nucleus does not secrete GnRH. Therefore, these females have no sense of smell and do not develop secondary sexual characteristics. The diagnosis is often one of exclusion found during the workup of delayed puberty. The presence of anosmia with delayed puberty should suggest Kallmann syndrome. Treatment is pulsatile GnRH therapy.
Tanner stage III breasts, tall stature and an otherwise normal examination. An MRI of the brain and a pelvic ultrasound are normal. LH and FSH levels are in the pubertal levels and she has normal DHEAS and androgen levels.
True precocious puberty is a diagnosis of exclusion where the sex steroids are increased by the hypothalamic-pituitary-gonadal axis, with increased pulsatile GnRH secretion. CNS abnormalities associated with precocious puberty include the following: tumors (e.g., astrocytomas, gliomas, germ cell tumors secreting human chorionic gonadotropin [hCG]); hypothalamic hamartomas; acquired CNS injury caused by inflammation, surgery, trauma, radiation therapy, or abscess; or congenital anomalies (e.g. hydrocephalus, arachnoid cysts, suprasellar cysts)
Abnormal sexual development
Congenital adrenal hyperplasia usually presents in the neonatal period and is associated with ambiguous genitalia. McCune Albright Syndrome is characterized by premature menses before breast and pubic hair development.
True precocious puberty treatment
treatment would include GnRH agonist to suppress pituitary production of follicular-stimulating hormone and luteinizing hormone. Observation is acceptable if the precocious puberty is within a few months of the routinely expected puberty. The process should be treated if the bone age or puberty is advanced by several years.
high DHEA and DHEAS levels and low levels of LH and FSH.
Congenital adrenal hyperplasia of the 21-hydroxylase type results in the adrenal being unable to produce adequate cortisol as a result of a partial block in the conversion of 17-hydroxyprogesterone to desoxycorticosterone, with the accumulation of adrenal androgens. This leads to precocious adrenarche. Treatment includes steroid replacement.
small vaginal opening with a blind pouch. Pelvic ultrasound reveals normal ovaries, but absence of uterus and cervix.
Renal anomalies occur in 25-35% of females with Mullerian agenesis. The uterus and cervix are absent, but the ovaries function normally and, therefore, secondary sexual characteristics are present. You would expect the karyotype in this patient to be 46,XX and testosterone levels in the female range.
severe abdominal pain. The pain is now constant and mildly uncomfortable, but every month she has a week when it is more severe. She has Tanner stage II breasts and pubic hair development. On genital examination, there is a bluish mass pushing the labia open.
Lower genital tract malformations occur in 1 in 10,000 females and are most commonly an imperforate hymen where the genital plate canalization is incomplete.
