mineralocorticoid excess hyperaldostronism. epidemiology first description of a patient with an...
TRANSCRIPT
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Mineralocorticoid ExcessHyperaldostronism
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Epidemiology
• first description of a patient with an aldosterone-producing adrenal adenoma (Conn's syndrome)
• mineralocorticoid excess was thought to represent a rare cause of hypertension
• a much higher prevalence is now recognized, ranging from 5 to 12%
• The prevalence is higher when patients are preselected for hypokalemic hypertension
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• The most common cause of mineralocorticoid excess is primary hyperaldosteronism, reflecting excess production of aldosterone by the adrenal zona glomerulosa.
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Causes of Mineralocorticoid Excess
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Etiology• Bilateral micronodular hyperplasia is more
common than unilateral adrenal adenomas • Bilateral adrenal hyperplasia is usually
micronodular but can also contain larger nodules that might be mistaken for a unilateral adenoma.
• rare instances, caused by an adrenocortical carcinoma.
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Causes of Mineralocorticoid Excess
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Clinical Manifestations
• Excess activation of the mineralocorticoid receptor leads to potassium depletion and increased sodium retention, with the latter causing an expansion of extracellular and plasma volume.
• Increased ENaC activity also results in hydrogen depletion that can cause metabolic alkalosis.
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• Aldosterone also has direct effects on the vascular system, where it increases cardiac remodeling and decreases compliance.
• Aldosterone excess may cause direct damage to the myocardium and the kidney glomeruli, in addition to secondary damage due to systemic hypertension.
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• The clinical hallmark of mineralocorticoid excess is hypokalemic hypertension
• serum sodium tends to be normal due to the concurrent fluid retention, which in some cases can lead to peripheral edema.
• Hypokalemia can be exacerbated by thiazide drug treatment, which leads to increased delivery of sodium to the distal renal tubule, thereby driving potassium excretion.
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• Severe hypokalemia can be associated with 1. muscle weakness2. overt proximal myopathy3. even hypokalemic paralysis• Severe alkalosis contributes to muscle cramps
and, in severe cases, can cause tetany
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Diagnosis
• should be restricted to those who exhibit 1. hypertension associated with drug resistance2. Hypokalemia3. an adrenal mass4. hypertension before the age of 40 years
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• The accepted screening test is concurrent 1. measurement of plasma renin and
aldosterone 2. calculation of the aldosterone-renin ratio
(ARR) • serum potassium needs to be normalized prior
to testing.
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Screening
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Confirmatory Tests
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Intravenous Saline Infusion Test
• Two liters of 0.9% sodium chloride solution is infused intravenously with an infusion pump over 4 hours with the patient recumbent.
• Blood pressure and heart rate are monitored during the infusion.
• At the completion of the infusion, blood is drawn for measurement of PAC.
1. normal subjects :PAC levels decrease to less than 5 ng/dL,2. primary aldosteronism : more than 10 ng/dL. 3. Indeterminate : PAC values between 5 and 10 ng/dL
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Oral Sodium Loading Test
• hypertension and hypokalemia controlled• high-sodium diet (supplemented with sodium chloride
tablets if needed) for 3 days, with a goal sodium intake of 5000 mg (equivalent to 218 mEq of sodium or 12.8 g sodium chloride
• On the third day of the highsodium diet, a 24-hour urine specimen is collected for measurement of aldosterone, sodium, and creatinine.
• Urinary aldosterone excretion of more than 12 μg/24 hours is consistent with autonomous aldosterone secretion
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Fludrocortisone Suppression Test
• fludrocortisone acetate is administered for 4 days (0.1 mg every 6 hours) in combination with sodium chloride tablets (2 g three times daily with food).
• Blood pressure and serum potassium levels must be monitored daily.
• In the setting of low PRA, failure to suppress the upright 10 a.m. PAC to less than 6 ng/dL on day 4 is diagnostic of primary aldosteronism
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Localization
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Adrenal Venous Sampling
• cortisol-corrected aldosterone lateralization ratios greater than 4.0: unilateral source of aldosterone like APA 95% sensitivity and 100% specificity
• zone of overlap :ratios greater than 3.0 but less than 4.0 represent
• bilateral aldosterone secretion : Ratios less than 3.0
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Treatment
• Patients younger than 40 years with confirmed mineralocorticoid excess and a unilateral lesion can go straight to surgery
• Laparoscopic adrenalectomy is the preferred approach.
• Patients who are not surgical candidates, or with evidence of bilateral hyperplasia based on CT or AVS, should be treated medically
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Medical treatment mineralocorticoid receptor antagonist • Spironolactone: started at 12.5–50 mg bid and
titrated up to a maximum of 400 mg/d to control blood pressure and normalize potassium.
• Side effects include 1. menstrual irregularity2. decreased libido3. gynecomastia• eplerenone : more selective MR antagonist . Doses
start at 25 mg bid and it can be titrated up to 200 mg/d.
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• Another useful drug is the sodium channel blocker amiloride (5–10 mg/bid).
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