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25 Cards in this Set

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What are the features of Constitutional Delayed Puberty?
1. Puberty not delayed beyond 16 in girls and 18 in boys.
2. Bone age is retarded appropriately for delay in puberty.
3. Prepubertal growth slows as puberty is delayed.
4. Endocrine paramets are similar to gonadotropin deficiency.
5. Pubertal progression and linear growth eventually advance normally.
6. Genetic height potential is acheived.
7. No underlying pathologic disorder is present.
What is indicative of imminent puberty in girls?
A plasma estradiol level more than 9 pg/ml.
What is indicative of immenent puberty in boys?
A plasma testosterone level more than 0.45 ng/ml.
What is the most common neoplasm assoc w/ hypthalamic-pituitary dysfunction and sexual infantalism?
Craniopharyngioma, which is a tumor of Rathke's pouch orginating from pituitary stalk and prevents hypothal releasing hormones from reaching pituitary. Assoc w/ calcifications in suprasellar region visible on X-rays.
What is Kallman Syndrome?
Olfactory tract hypoplasia that is 6x more prevalent in boys. Results in hypothalamus dysfunction (no gonad releasing hormone) w/ the clinical presentation of anosmia (lack of smell).
What are the two main causes of hypogonadotropic delayed puberty?
Craniopharyngioma and Kallman Syndrome
What is the most common form of hypergonadotropic delayed puberty in girls?
Turner's Syndrome. The genetic information that regulates rate of ovarian follicular atresia is carried on long arm of X-chromosome and somatic info is on short arm.
What is the most common form of hypergonadotropic delayed puberty in males?
Klinefelter's Syndrome. We see aortic valvular dz, berry aneurysms, breast carcinoma, and osteoporosis in these pts.
When do we use hormonal therapy for delayed puberty and for how long do we do it?
Severe constitutional delay (limit Tx to 6 mos) and pts w/ gonadal failure (long term Tx). In Turner's, combo growth hormone & low dose anabolic steroids is superior to sex hormone replacement alone.
What is the traditional definition of precocious puberty?
The development of any signs of 2ndary sexual maturation before 8 in girls and 9 in boys.
What are the two general categories of disorders that can cause precocious puberty?
GnRH-Dependent forms = involve activation of GnRH pulse generator.
GnRH-Independent forms = increased sex steroid production is not mediated by normal CNS mech.
What is true complete isosexual precocity?
Premature activation of hypothal-pituitary-ovarian axis. Usually due to hypothalamic hamartoma.
What is a hypothalamic hamartoma?
A neoplasm comprised of neurosecretory neurons that produce ectopic GnRH.
How can we determine premature activation of central mechanisms regarding precocious puberty?
The GnRH stimulation test, which involves administering IV GnRH and measuring LH and FSH secrection. Serum LH levels > 10 mIU/mL indicates central activation.
What is pseudoprecocious puberty in girls?
A condition usu due to estrogen production, which can be exogenous, from a neoplasm, or from the McCune-Albright syndrome.
What type of tumors are estrogen-secreting tumors of the ovary in children?
Granulosa-theca cell tumors (usu unilateral)
Germ cell turms that produce what can induce sexual precocity in boys?
hCG
What type of tumors almost always produce heterosexual precocity?
Adrenal tumors
What is the McCune-Albright syndrome?
aka Polyostotic Fibrous Dysplasia. Multiple cystic bone lesions prone to fracture, cafe-au-lait spots (on face, neck, shoulders, and back), more common in girls. An activating mutation that leads to autonomous hyperfunction of many endocrine organs. In the ovary, follicle cyst development and estradiol production occur independent of gonadotropins.
Waht is Familial Testotoxicosis?
A sex-limited, AD condition characterized by sexual precocity w/ testicular enlargement and maturation of Leydig cells and seminiferous tubules despite absence of gonadotropin secretion. Affected indivicduals are short but have nl fertility. Receptor mutation that leads to constitutive activation in absence of ligand.
What is Extreme Primary Hypothyroidism?
Has been reported to cause exual precocity, almost exclusively in girls. High levels of thyrotropin-releasing hormone, produced by pituitary in response to thyroid hypofunction, simulate gonadotropin secretion. Also see hyperprolactinemia and galactorrhea w/ this.
What is the significance of Peutz-Jeghers syndrome?
Has been assoc w/ ovarian tumors that may be estrogen-secreting, thus responsible for sexual precocity.
What is premature thelarche?
Isolated breast development. Usu occurs before age 4 and resolves spontaneously. Prob due to transient estrogen production from ovary.
What is premature adrenarche?
Isolated development of axillary hair. Usu occurs after age 6 and is result of premature development of adrenal androgen production. Serum DHEA-S elevated. Response to ACTH stim is normal.
What is premature pubarche?
Isolated pubic hair deleopment. More common in boys and assoc w/ CNS disorder in 50% of cases.