CMDT DSA--GH, acromegaly, hypopituitarism Flashcards
hypopituitarism w/o mass lesions- congenital
- congenital hypopituitarism
- congenital isolated hypogonadotropic hypogonadism (affects GnRH, LH, or FSH)
- Prader-Willi syndrome
- Kallmann syndrome (impair GnRH)
- congenital GH def
Laron syndrome
- AR
- mutations in the GH R- results in GH-resistance
- def in IGF-1- dwarfism
- prominent forehead, depressed nasal bridge, small mandible, central obesity
gonadotrophic def (hypogonadotropic hypogonadism)- causes what?
-hypogonadism, infertility
gonadotrophic def- causes
- congenital- lack of pubertal development
- isolated hypogonadotropic hypogonadism
- Prader-Willi syndrome
- acquired- gradual loss of facial, axilliary, pubic hair
Prader-Willi syndrome
-cryptorchidism, mental retardation, short stature, hyperflexibility, autonomic dysregulation, cognitive impairment, hyperphagia with obesity, hypogonadotropic hypogonadism
dx of ACTH def
- fxnal atrophy of adrenal cortex
- cosyntropic test- blood drawn for serum cortisol, ACTH, and DHEA- then synthetic ACTH is administered- cortisol < 20 indicates adrenal insufficiency
GH stimulation test
only 66% sensitive- can do a therapeutic trial of GH tx
imaging- indicated when?
-when a mass lesion is suspected
tx for hypopituitarism
-H replacement!
central diabetes insipidus- essentials of dx
- ADH def with polyuria (2-20L/day) and polydipsia
- hypernatremia occurs if fluid intake inadequate
familial diabetes insipidus
- dominant- sx’s at 2 yo
- Wolfram syndrome- AR- DIDMOAD (diabetes insipidus, type 1 DM, optic atrophy, deafness)
vasopressin challenege test
-desmopressin acetate intranasally- measure urine volume for 12 hrs b/f and after
central diabetes insipidus- tx
-desmopressin acetate- intranasally every 12-24 hrs as needed for thirst and polyuria
Acromegaly and gigantism- essentials of dx
- pit tumor
- gigantism b/f closure of epiphyses, excessive growth of hands, feet, jaw, internal organs
- amenorrhea, HAs, visual field loss, weakness
- soft, doughy, sweaty handshake
- elevated serum IGF-1
- serum GH not suppressed following oral glucose
acromegaly and gigantism- lab
-serum IGF-1, PRL, glucose, liver enzymes and serum creatinine or BUN (liver failure or kidney dz can elevate GH), T4 and TSH
acromegaly and gigantism- tx
- pituitary microsurgery- remove the adenoma
- cabergoline (dopamine agonist of choice- oral)
- octreotide and lanreotide (SST analogs)
- tamoxifen (selective estrogen R modulator)
- pegvisomant (GH R antagonist)
- stereotactic radiosurgery- in pts who dont achieve complete remission with surgery or medical therapy
hyperprolactinemia- essentials of dx
- women- oligomenorrhea, amenorrhea, galactorrhea, infertility
- men- hypogonadism, dec libido and ED, infertility
- elevated serum PRL (PRL is normally elevated during pregnancy)
- CT scan or MRI- pit adenoma
hyperprolactinemia- tx
- pregnant women w/ macroadenomas- continue tx with dopamine agonists to prevent tumor growth
- dopamine agonists
- surgical tx- for apoplexy or those that compromise visual fields
