CMDT DSA--GH, acromegaly, hypopituitarism Flashcards Preview

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Flashcards in CMDT DSA--GH, acromegaly, hypopituitarism Deck (18)
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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

-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