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What is the most common cause fo Hyperaldosteronism? What are some other causes
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Treatment of hypoaldosteronismMineralocorticoid replacement : FludrocortisoneWhat is the most common cause of PAI in the US (MC in what age/gender)Addison's disease; autoimmune adrenalitis MC in women 30-50Other than the most common what are other causes of PAIPolyglandular autoimmune syndrome (PGA) -Type 1: autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) = childhood diagnosis, candidiasis, dystrophy of hair and nails, hypoparathyroidism -Type 2: F>M, 20-40yo adrenal/pancreas/ thyroid/more common than 1 Waterhouse-Friderichsen syndrome: thrombosis or hemorrhage in the adrenal gland (post op, trauma, sepsis, heparin associated thrombocytopenia)What is the most common cause of PAI in developing countriesInfection (TB, fungal, HIV)What are sx/signs that are unique to PAIHYPERPIGMENTATIONWhat are sign/sx of PAIFatigue, malaise, weakness, anorexia Postural dizziness, syncope, Myalgias, arthralgias, Weight loss, thinning of axillary and pubic hair, Vitiligo GI: N/V/D, abdominal pain, constipation Orthostatic HYPOTENSIONWhat is a screening/diagnostic test for PAICosyntropin 0.25mg IV (synthetic ACTH) Take serum Cortisol 45 min later Normal: rises to at least 20mcg/dL Primary AI: minimal to no response in cortisol Secondary (<18microg/dL) Increased ACTHWhat is a definite diagnostic test for PAISerum DHEA level (androgen) <1000ng/mL in 100% of Addisons (>1000 excludes the dx)Expected Chem10 values of PAIHyponatremia Hyperkalemia Hypoglycemia HypercalcemiaExpected Lab values of PAIHigh: CRH, ACTH, Renin Low: Aldosterone, Cortisol, AndrogensTx for Adrenal InsufficiencyGlucocorticoid replacement: Hydrocortisone (first line) Dexamethasone Prednisone Mineralocorticoid replacement: Fludrocortisone (only primary) High Na dietAdjustments in PAI during minor illness and pregnancyInc dose during minor illness (2x, 3x) Pregnancy (normal maintenance dose till time of delivery where they inc dose)Give the levels for CRH, ACTH and Cortisol in Primary/Secondary/Tertiary AIPrimary: CRH high, ACTH high, Cortisol low Secondary: CRH high, ACTH low, Cortisol low Tertiary: CRH low, ACTH low, Cortisol low,What is the overall cause for SAIDisorder of the pituitary; failure to secrete ACTHDiagnostic test for SAI + interpretationCRH stimulation test: absent or low ACTHWhat is the overall cause fo TAIHypothalamus disorderWhat are the renin levels in PAI, SAI, TAIPAI: Renin is High SAI: Renin is normal/low TAI: Renin is normal/lowAcute Adrenal Crisis is a what....MEDICAL EMERGENCYCommon causes of AAC in pt with Adrenal Insufficiency (AI)Abrupt withdrawal of steroids MC Failure to increase dose during stress (ex Trauma, surgery, infection, hyperthyroidism)Common causes of AAC in pt w/o A1 (2 + what med)Post adrenalectomy or removal of functioning adrenal tumor that has suppressed other adrenal Sudden destruction of pituitary (trauma, infarct) Bilateral adrenal injury (hemorrhage) Administration of etomidate (blocks cortisol production)Sign/Sx of AACShock >90%: hypotension, hypovolemia, dehydration Weakness, abdominal pain, fever, confusion, N/V/D, fatigue, headache, comaLabs of AAC (sugar, Na, K, Ca)Hyponatremia Hypoglycemia Hyperkalemia HypercalcemiaDiagnostics for AACCortisol and aldosterone levelsYou should not do what before initialing treatment for AACDont not wait for results to begin txHow do you tx AACImmediate administration of high dose dexamethasone (4mg IV bolus Q12H) or hydrocortisone 100mg IV bolus Q6H Followed by IV steroids for days then PO steroids Isotonic fluids (normal saline or D5NS) Reversal of electrolyte disorders Broad spectrum antibioticsWhat are the ACTH dependent Cortisol disturbances?Pituitary Tumors (Cushing's DISEASE) Ectopic ACTH secreting tumor (lung, pancreas, thymus, MC is SCC of the lung)What is more common ACTH dependent or independent disturbances of cortisol?ACTH DEPENDANT (70%)What the ACTH independent Cortisol disturbances?Adrenal Neoplasm (cortisol secreting, MC adenomas, W>M 25-45yo); 1/2 in kids are carcinomas Bilateral Micro/Macronodular HyperplasiaWhat does hyperplasia mean?Increase in the number of cells due to hormonal and/or compensatory changesWhat is an exogenous cause of hypercortisolism/Cushing Syndrome ?Long term use of high dose glucocorticoidsSign/Sx of Hypercorticolism/Cushing SyndromeWeight Gain, Moon Face, Purple/Red Striae, Retrocervical/supraclavicular fat pad - Buffalo Hump, HTN, Truncal/Central Obesity w/ thin extremities, Acanthosis Nigricans, Hirsutism, Acne, Skin AtrophyWhat is Acanthosis Nigricans?Epidermal hyperplasia + thickening with darkening of the skin (velvet-like skin) that often involved axilla or groinWhat is the most specific screening tests for Hypercorticolism/Cushing Syndrome + others? (interpret LD)MS: 24hr urinary free cortisol Nighttime (11pm) salivary cortisol Low dose (1mg) overnight - low dose Dexamethasone suppression test (elevated cortisol or no suppression = Cushing's syndrome)What is the differential test for determine the cause of Hypercorticolism/Cushing Syndrome?Baseline ACTH + High-Dose Dexamethasone suppression test3 Results of the Differential Test for Hypercorticolism/Cushing SyndromeCushing Disease: increased ACTH + suppression of cortisol Ectopic ACTH-producing tumor: increased ACTH + no suppression of cortisol Adrenal tumor & steroids: decreased ACTH + no suppression of cortisolWhat is the only cause of Hypercorticolism/Cushing Syndrome that suppresses cortisol on high-dose dexamethasone??Cushing's DiseaseExpected labs (sugar, wbcs, K, cortisol) + Acid/Base condition with Hypercorticolism/Cushing SyndromeHyperglycemia, Glycosuria, Leukocytosis, Hypokalemia, High Cortisol Metabolic AlkalosisTx of adrenal adenoma/mass Hypercortisolism/Cushing's syndrome?Surgical resectionTx of Cushing's DiseaseTranssphenoidal resectionTx of inoperable or bilateral hyperplasia Hypercortisolism/Cushing's syndrome?Medical inhibition of steroid production with adrenal enzyme inhibitors (ketoconazole, mitotane)What is a Phenochromocytoma? Describe the PathophysiologyAdrenal (chromaffin cells) tumor of the sympathetic nervous system that secretes catecholamines (norepi/epi/dopamine)Sign/Sx of PheochromocytomaTriad: Episodic headache, sweating, and tachycardia 5 P's: Pressure (paroxysmal HTN) Pain (head, chest, abd) Perspiration Palpitations/Panic PallorAre most Pheochromocytoma's unilateral or bilateral? are most benign or malignant?Most are unilateral and benign (MENS syndrome: most are bilateral)Two weeks prior to testing for Pheochromocytoma what should the pt do?Discontinue meds (except anti HTN)What is the low risk test for Pheochromocytoma?24hr urine catecholamine + metanephrine which confirms dxTest for high risk PheochromocytomaPlasma fractioned free metanephrineImaging for PheochromocytomaImaging: MRI or CT abd & pelvis (No mass?? consider ectopic source = extra-adrenal (paraganglioma) Nuclear Imaging: MIBG (can detect tumors outside adrenal gland if CT/MRI negative)Step wise process for medication administration for PheochromocytomaFirst: Alpha blocker PO (PHEnoxybenzamine/nonselective or selective alpha 1 blocker/prazosin, tetrazosin, doxazosin) FOLLOWED by Beta Blockers (metoprolol)Surgical tx for PheochromocytomaResection; Complete adrenalectomy after 1-2 weeks of medsWhat should your consider post surgery in PheochromocytomaHypotensionWho/What sx should you test for Pheochromocytoma?Classic Triad (episodic HA, sweating, tachycardia), onset HTN <20yo, RESISTANT HTN, fhxAre most Adrenal Carcinoma unilateral or bilateral?UnilateralWhat is the MC adrenal nodule?Benign Adrenal AdenomaWhat should you do with a nodule >6cm??SurgeryWhat should you do with a nodule <4cm non-secretory benign nodule?ObserveYou can perform _____ ______ _______ for hyperaldosteronism/pituitary conditions which can help differentiate unilateral or bilateral hyperplasiaadrenal venous sampling