FINAL Flashcards
hyperaldosteronism
-adrenal cortex
-primary- single adenoma (conns), bilateral adrenal hyperplasia
-renin independent-> low renin and high aldosterone
-low K
-polyuria, polydipsia
-BP > 150/90 3 diff times or > 140/80 on 3 diff meds or controlled on 4 diff meds
-paresthesias - metabolic alkalosis
-constipation
-palpitations
-high HCO3, low H
-saline infusion test, oral sodium loading test, CT, adrenal venous sampling
-unilateral- surgery
-bilateral- spironolactone
-secondary- high renin and high aldosterone
congenital adrenal hyperplasia (CAH)
-glucocorticoid deficiency
-low cortisol high ACTH
-21β-hydroxylasedeficiency: MC
-Hypotension
-XX GENOTYPE
-Female pseudohermaphroditism: clitoromegaly and/or male external genitalia along with a uterus and ovaries
-Precocious puberty- early puberty
-Virilization, irregular menstrual cycles, infertility, hair
-diff dx- PCOS- do US
-XY GENOTYPE
-Normal male external genitalia at birth
-Precocious puberty
-11β-hydroxylase& 17α deficiency:
-Hypertension
-XX GENOTYPE
-Normal female external genitaliaatbirth
-Delayed puberty(primary amenorrhea) or sexual infantilism
-XY GENOTYPE
-Male pseudohermaphroditism:female external genitalia with a blind ending vagina and intra-abdominal testes at birth
-Delayed puberty or sexual infantilism
CAH symptoms
-adrenal crisis
-failure to thrive
-hypoglycemia
-hyperpigmentation
-gender dysphoria
-21 beta hydroxylase deficiency -> males present with classic CAH- salt wasting crisis -> shock
-21 beta- low aldosterone and high K
CAH dx
-17 hydroxyprogesterone increase in 21 beta
-low cortisol even after cosyntropin (ACTH)
-neonatal screening
-advanced bone age in children
-non classic men presentation -> familial genetics
-CT- abdomen testes
CAH tx
-replace low cortisol
-since ACTH is high -> will increase androgens -> control this too
-hydrocortisone- prevent crisis in time of stress
-prednisone - good for androgen excess in children
-increase dose with sickness
-21- fludrocortisone, NaCl
-11- spironolactone, decrease Na
-17- spironolactone, estrogen in females, decrease Na
pheochromocytoma: adrenal medulla
-triad- headaches, tachycardia, sweating
-paragangliomas
-MEN2, von hippel-lindau syndrome, neurofibromatosis type 1, familial paragangliomas
-must screen before medullary thyroid cancer surgery
-increase appetite and weight loss
-hyperglycemia
-reynauds
-cardiomegaly, cardiomyopathy
-postural tachy, postural hypotension
-triggered by dye, glucagon inj, needle bx, anesthesia, surgery
pheochromocytoma labs
-thyroid- WNL
-leukocytosis
-ESR high
-hyperglycemia- high
-high renin
-metanephrine > 2.2
-catecholamine > 135
-plasma metanephrine- more sensitive
-urinary assay for vanillylmandelic acid (VMA)
-CT, genetic testing, MRI, nuclear, imaging
-things that interfere:
-drugs- acetaminophen, antihistamines, anti-HTN
-food- banana, peppers, caffeine
-ds- severe pain renal failure, exercise hypoglycemia, severe emotion
pheochromocytoma complications
-sudden blindness
-CVA
-sudden death
-ARDS
-severe hypotension after resection
-renal failure
-MI
-seeding of tumor
pheochromocytoma tx
-laparotomy
-preop- CCB, alpha blocker
-propranolol
-nicardapine- HTN crisis
-post op - blood transfusion, volume replacement
-follow up- recheck urine catecholamines and whole body scan
-inoperable/metastatic tumor- metyrosine
-metastatic pheochromocytoma- combo chemo and irridation
neurofibromatosis type 1
-multiple neurofibromas
-cafe au lait spots
-axillary freckling on skin
-lisch nodules of iris
-usually on adrenals
adrenal insufficiency
-high ACTH, low cortisol
-tx- hydrocortisone
-herediatary factors
-associ with other autoimmune ds (DM, hypothyroid) or mucocutaneous candida infections
-causes: autoimmune, granuloma (TB), congenital adrenal hypo/hyperplasia, B/L hemorrhage, drugs that block corticosteroid synthesis (antineoplastic, HIV meds like dronabinol, ketoconazole)
adrenal insufficiency symptoms
-salt cravings
-hypotension
-hypoglycemia
-lack of hair
-weakness
-fatigue
-dizzy
-wt loss
-nausea/vomiting
-diarrhea
-intolerance to temp
-abdominal pain
-depression/anxiety
-amenorrhea
-orthostatic hypotension
-hyperpigmentation- high ACTH
-vitiligo
-addisonian crisis-
-fever
-orthostatic hypotension
-salt craving
-sharp sudden pain in abdomen, legs, lower back
-vomiting, diarrhea
-hypoglycemia
-dehydration
-loss of consciousness
adrenal insufficiency dx
-morning cortisol, morning ACTH, ACTH stimulation test
-primary- screen for hypoaldosteronism and hypoandrogenism -> tx depends on this
-secondary- tx not affected by this
-<3 @ 8AM, <8 @ anytime, >20 at 8AM is unlikely
-DHEA <1000
-PRIMARY- high renin*
-SECONDARY- normal renin*
-ACTH > 200 in primary
-primary- no change after ACTH stimulation
adrenal insufficiency tx
-acute:
-fluid replacement
-if still hypotensive -> dexamethasone STAT
-dose doubled during fever, infection, trauma, minor physical stress
-chronic:
-prednisone -
-double dose in summer, sickness
-fludrocortisone
-secondary adrenal insuff- tx- hydrocortisone
-alert bracelet
-emergency pack
polyglandular deficiency syndrome
-autoimmune
-Symptoms d/o the combination of deficiencies
-Three types
-Diagnosis - requires measurement of hormone levels and autoantibodies against affected endocrine glands
-Treatment - replacement of hormones
-Chronic mucocutaneous candidiasis - lifelong antifungal therapy
cushings
-cushing syndrome- exogenous vs endogenous
-other etiologies- B/L micronodular dysplasia, B/L ACTH independent macronodular hyperplasia, ectopic production of CRH
-if older suspect - ectopic lung
-associated with- DM, alcoholism, MEN type 1, use of steroids
-striae differentiates
-CT or MRI does not R/O cushing disease if not seen -> 50%
cushing work up
-urinary free cortisol -> 3 24h collections -> rules in cushings
-low dose dexamethasone given at 11PM and cortisol measured at 8AM -> suppresses ectopic and disease; if no suppression -> adrenal
-late night salivary cortisol > 5
-CRH -> disease response
-DEX .8 -> suppresses disease
-BIPSS -> disease gradient
cushing tx
-treat comorbidities of ds -> osteoporosis, psychiatric, DM, HTN, muscle weakness, hypokalemia, infection!
-bone densitometry
-metyrapone- inhibits cortisol
-adrenal enzyme inhibitors- ketoconazole or adrenal steroidogenesis blocker
-cushing disease- trans-sphenoidal resection, radiation
-adrenalectomy- mitotane-antineoplastic (adrenal carcinoma)
hyperparathyroidism
-hypercalcemia of malignancy - high PTHrP, PTH
-HPT- usually single adenoma
-severe- osteitis fibrosa- tapering of distal collar bones, salt&pepper, brown tumors on long bones, subperiosteal resorption of distal phalange
-abdominal pain
-neuro- proximal myopathy
-renal
-GI- PUD, acute pancreatitis, constipation
-cardio- bradycardia, LV hypertrophy, shortening of QT, HTN
-familial hypocalciuric hypercalcemia- Ca doesnt go down after resection
hypercalcemia causes
-parathyroid related:
-PTH*
-lithium therapy
-familial hypocalciuric hypercalcemia
-MEN1
-malignancy*:
-breast metastases
-humoral mediation of hypercalcemia (lung, kidney)
-hematologic malignancy (multiple myeloma, lymphoma, leukemia)
-PTHr
-vitamin D:
-intoxication
-sarcoidosis/granulomatous ds
-idiopathic hypercalcemia of infancy
-assoc with high bone turnover
-immobility
-hyperthyroid
-thiazides
-vitamin A intoxication
-assoc with renal failure:
-severe secondary HPT
-aluminum intoxication
-milk alkali syndrome
PTH dx and tx
-high PTH + Ca = HPT
-If low, check PTHrP, Vitamin A & D, TSH, Cortisol, ACE, 24h urine calcium clearance, review meds
-24 hour urine- <50 -> familial hypocalciuric hypercalcemia
-imaging- wrist, spine, hip DEXA, stones, 99m technetium sestamibi radionuclide scan
-surgery- Ca > 11.5, T-score < -2.5, renal stone
-replace volume
-biphosphonates
-calcitonin - in rapid emergency
-facilitate calciuresis - loops
-estrogen (post menopausal)- raloxifene
-calcimimetic agents
-mild/asymptomatic- no tx
secondary hyperparathyroid
-renal:
-high PTH
-low Ca
-high phosphate
-osteomalacia, osteitis fibrosa cystica
-vitamin D deficiency
-high PTH
-low calcium
-low phosphate
-prevention- vitamin D, phosphorus binders (sevelamer)
-tx- calcimimetic agents
-surgical- in refractory cases, severe hypercalcemia, severe bone disease, severe pruritis, calciphylaxis, severe myopathy
tertiary hyperparathyroid
-secondary hyperparathyroidism that is no longer responsive to medications
-Multiple adenomas
-calcium- phosphate product > 70
-severe bone ds and pain
-intractable pruritis
-soft tissue calcification with tumor calcinosis
-calciphylaxis- dark indented scab
-high PTH (renal)
-high calcium
-high phosphorus
hypoparathyroid
-CaPO4- 60 WNL -> >70 -> crystals in soft tissue -> arteriosclerotic ds
-causes- thyroidectomy, autoimmune, infiltrative ds (wilson, hemochromatosis, granulomas, metastasis), hypomagnesium, DiGeorge syndrome, idiopathic, acute pancreatitis
-renal manifestations of hypoparathyroidism -> due to hypercalciuria
-MC cause of hypocalcemia- hypoalbuminemia
