TEST 1 Flashcards
hypopituitarism
-partial/complete loss of anterior pituitary
-dx- MRI*, CT, pituitary hormone levels
-primary- tumor (MC), infarction (apoplexy, sheehan’s), inflammatory (meningitis, abscess, sarcoidosis), infiltrative (langerhan’s cell histiocytosis, hemochromatosis), iatrogenic, autoimmune
-secondary- hypothalamic
-insidious onset is MC but sudden is more deadly
-congenital is deadly (GH only affects kids)
-ACTH with adrenal crisis and TSH with myxedema -> deadly
-GH is lost first -> GnRH, TSH, ACTH
-ADH rarely lost unless secondary issue -> if pt has polydipsia or SIADH -> suspect hypothalamus
hypopituitarism tx: urgent cute adrenal insufficiency, visual field loss, prolactinoma, pituitary apoplexy
-urgent acute adrenal insufficiency -> ASAP glucocorticoids -> 100mg hydrocortisone IV, then 5-100 every 6 hrs and fluid replacement
-visual field loss/tumor -> ASAP referral -> transsphenoidal removal or irradiation
-hormone replacement
-dopamine agonist- prolactinoma
-pituitary apoplexy- ASAP surgery if visual issues or coma
ddx of generalized hypopituitarism
-anorexia nervosa- maintenance of secondary sexual characteristics! (this differentiates it) despite amenorrhea -> increased GH (low IGF) and high cortisol
-alcohol liver disease of hemochromatosis- evidence of liver disease -> lab testing
-myotonia dystrophica- progressive weakness, premature balding, cataracts, facial features of accelerated aging -> lab testing
-polyglandular autoimmune disease
hypopituitarism in children
-pituitary tumor- craniopahrngioma
-langerhan’s cell histiocytosis
-cleft palate
-idiopathic, hereditary (5%)
-ht below 3rd precentile
-skeletal maturation > 2yrs behind -> left hand x-ray
-late puberty
-CT or MRI
-mid to late childhood -> IGF-1 measured
-tx- surgery, GH therapy, other hormone therapy
ACTH deficiency
-fatigue, weak
-wt loss
-hypotension
-hypoglycemia
-intolerance to stress and infection
-decreased axillary and pubic hair
-NO hyperpigmentation
-plasma and urine steroid levels are low
-dx-ACTH stimulation test- synthetic ACTH (cosyntropin) -> cortisol should rise >21 and aldosterone >4 from baseline after 30-60 mins
-insulin tolerance test- hazardous in panhypopituitarism, DM, old -> CI in CAD and epilepsy
-tx- cortisol replacement
Kallmans syndrome
-lack of GnRH
-midline facial defect- cleft
-anosmia, sensorineural deafness
-color blind
-hypogonadism
-unilateral renal agenesis (missing 1 kidney)- 50%
-cryptochidism (testes dont descend), micropenis
-bimanual synkinesis (mirror)
-ataxia, cognitive problems
-menarache with secondary amenorrhea
panhypopituitarism
-simmond’s syndrome- involves all hormones -> embolic infarction, tumor, syphilis, tuberculosis, metastatic abscess
-sheehan’s syndrome- placenta not delivered right away -> no contraction -> blood loss -> pituitary necrosis from hypovolemia and SHOCK
-no lactation after birth, fatigue
-loss of secondary sexual characteristics (pubic and axillary hair)
pituitary apoplexy
-adrenal crisis
-sudden
-pituitary outgrows blood supply
-hemorrhagic infarction of normal or tumor pituitary
-headache, neck stiff, fever, visual field defects*, oculomotor palsies
-compression on hypothalamus -> coma
-hypopituitarism results suddenly -> vascular collapse due to low ATCH and cortisol
-CSF +/- blood
-MRI- hemorrhage
gigantism
-little bony deformity
-delayed puberty
-hypogonadotropic -> eunuchoid habitus
acromegaly
-1st symptoms- coarsening of face, soft tissue swelling of hands and feet
-naso-sinus abnormality -> snoring, sinusitis, sleep apnea
-diplopia
-arthralgias - TMJ- prognathism
-gums grow -> teeth gaps
-dorsal kyphosis
-deepening of voice
-increase soft tissue- macroglossia, heel thickness (>22mm), sausage fingers
-barrel chest
-insulin resistance, glucose intolerance -> hyperglycemia
-hypertriglyceridemia
-HTN, cardiovascular abnormal
-pulmonary issues from soft tissue growth
-colonic polyposis
-hyperprolactenemia- galactorrhea
-decrease libido and impotence
-tumor- headache, visual issues -> hypopituitarism due to compression
-mortality- stress on body, prone to malignancy
-oily, doughy moist handshake, sweaty
-carpal tunnel, weakness in arms and legs
-skin tags*
-frontal bossing
non-pituitary tumor secretion of GH
-RARE
-Pancreatic cancer
-Small cell lung cancer
-Tumors of the adrenal gland
-Carcinoid syndrome
-Carcinoma of the thyroid
-Hypothalamic tumors - extremely rare -> Hamartoma, glioma, or gangliocytoma
acromegaly/gigantism dx
-symptoms lead to suspicion
-IGF-1*
-GOLD STANDARD- OGGT - confirms dx
-if GH fails to decrease to <1 after ingestion of glucose -> +
-give glucose- GH and IGF should decrease BUT with acromegaly it will not decrease
-MRI- pituitary adenoma
-CT*
-whole body scan, SPECT, somatostatin receptor scintigraphy - ectopic tumor
gigantism/acromegaly tx
-surgery- 1st line
-microadenomas (<10mm), macro (>10mm) -> most are macro (50% curable with resection)
-usually do med or radiation therapy with resection
-Pharmacotherapy- 2nd line
-somatostatin analogs- octreotide -> can also be give before surgery to shrink the tumor down
-dopamine agonist- cabergoline or bromocriptine
-GH receptor antagonist- pegvisomant
-radiation- adjunct or 3rd line
-used for non-resectable, pharm therapy fail, or adjunct
-usually used on its own
-risk of hypopituitarism within 10 years
-stereotactic (gamma knife) radio surgery
-decline in sweating/oily skin -> hallmark of surgical success
-soft tissue symptoms resolve with tx
-bone changes do not resolve
-if colonic polyps were an issue -> continued to be high risk
-if tx before articular cartilage damage -> joint symptoms are reversible
hypothalamic pituitary lesions
-MC tumor- hypo (compression) or hypersecretion
-headache, visual field defects
-thirst, altered appetite
-MRI
-can cause enlarged sella
pituitary adenoma
-macroadenoma- >10mm -> mass effect
-macro -> look for signs of hypopituitarism!!!!!!!!! (big enough to compress) -> pallor, hypotension
-microadenoma- <10mm -> hormones effect
-secretory- prolactinoma, GH secreting, corticotroponin secreting, thyrotropin secretion
-non-secretory- MC gonadotropin -> usually present with visual defects bc no hormone symptoms
secretion pituitary adenomas
-GH-secreting tumors: Signs of acromegaly
-Thyrotropin-secreting pituitary adenoma:
-Thyrotoxicosis
-Goiter
-Visual impairment
-Corticotropin -secreting pituitary adenoma:
-Truncal obesity
-Round face
-Dorsocervical fat pad (buffalo hump)
-Hirsutism
-Acne
-Menstrual disorders
-Hypertension
-Striae
-Bruising
-Thin skin
MEN
-autosomal dominant
-genetic testing
-MEN1- pituitary, parathyroid, pancreas
-MC prolactinoma
-Carney syndrome- spotty skin, myxomas, endocrine tumors (testicular, adrenal, pituitary)
-McCune-Albright syndrome- polyostotic fibrous dysplasia, pigmented skin patches, endocrine disorders (GH pituitary tumors, adrenal adenomas, autonomous ovarian function
-familial acromegaly
-MEN2- medullary thyroid cancers, pheochromocytoma, hirschspring disease
pituitary metastases
-rare
-POSTERIOR PUTIATRY (almost exclusively)
-diabetes insipidus!
-lung, GI, BREAST*
-MRI- hard to distinguish from pituitary adenoma
-primary or metastatic lymphoma, leukemias, plasmacytomas can occur within sella
prolactinoma S&S
-MC tumor
-usually microadenoma in women
-women - galactorrhea, amenorrhea, infertility, low libido, osteoporosis, painful sex, increase body hair, atrophic vaginitis, loss of labial fat pads, decrease breast size
-men- visual disturbances!
-usually macroadenoma- hypopituitarism
-hypogonadism, low libido, ED, infertility, decrease body hair, gynecomastia, galactorrhea (1/3), osteoporosis, decrease body hair
-apathy in women and men- ddx depression
-pharmacologic causes- amphetamines, nicotine, TCA, verapamil, estrogens*
-other causes: chronic renal failure, liver disease, ectopic production of prolactin- bronchogenic carcinoma (mostly small cell undifferentiated)*, hypernephroma
prolactinoma dx and tx
-are medications causing it?
-TSH*
-serum prolactin levels
-prolactin is episodic- stress can increase
-repeat sample should be taken if borderline
-NORMAL- <20
-if single draw is > 200 -> dx prolactinoma
-MRI*!
-tx- INDICATION- infertility or macroadenoma
-dopamine agonist- 1st line -> cabergoline, bromocriptine
-if visual issues -> transsphenoidal surgery
-radiotherapy- prior to surgery or after
-reoccurance is high after tx stops