Primary hyperaldosteronism(PA) detection test : ARR > 20 (PAC ≥ 15 ng/dL, PRA < 1 ng/mL per hour)입니다. Adrenal CT에서 nonfunctioning nodules을 나타내고 adenoma로 잘못 제시하기도 하지만 adrenal mass가 있고 현저한 aldosteronism이 있으며 35세 미만인 경우에는 추가 검사는 권고되지 않습니다[Goldman-Cecil 26th edition] https://blog.naver.com/sjloveu2/221752726496
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J Clin Endocrinol Metab.2016;101:1889-916.
Diagnosis
Although most of these conditions result from excessive aldosterone production by one or both adrenal glands, excessive production of other mineralocorticoids or constitutive activation of the renal sodium channel must be excluded.In these latter conditions, both aldosterone and renin values are low, resulting in the so-calledsyndrome of apparent mineralocorticoid excess.In this setting, diagnostic information is obtained by history (licorice ingestion) or measurement of other mineralocorticoids.
Primary hyperaldosteronism is diagnosed when there is an increased ratio (>20) of morning aldosterone to plasma renin activity. Except as noted in the figure, one of four tests (usually salt loading) is used to confirm primary hyperaldosteronism by demonstrating a lack of aldosterone suppression.
Differential Diagnosis
Having made the diagnosis of aldosterone-dependent mineralocorticoid excess, one must differentiate between the two most common adrenal causes—hyperplasia and adenoma—after excluding potential rare causes of hyperaldosteronism. Two rare autosomal dominant forms of familial hyperaldosteronism are type 1, a glucocorticoid-suppressible hyperaldosteronism, and type 2. Familial hyperaldosteronism type 1 is caused by a genetic swap of the promoter forCYP11B1(11β-hydroxylase) with that ofCYP11B2(aldosterone synthase), forming a chimeric gene in which ACTH stimulates aldosterone synthase. It should be suspected in the setting of familial disease, particularly if there is a history of early-onset cardiovascular events, and is confirmed by gene testing. Analysis of a multiplex family with familial hyperaldosteronism type 2 and 80 additional probands with unsolved early-onset primary aldosteronism revealed that eight had novel heterozygous variants in theCLCN2gene that encodes a voltage-gated chloride channel that is expressed in adrenal glomerulosa cells.
For the more common conditions,adrenal computed tomography scans may show nonfunctioning nodules and falsely suggest an adenoma.However,further testing is not recommended in a patient less than 35 years old with marked aldosteronism and an adrenal mass.The responses to physiologic maneuvers, such as upright posture, and salt loading with oral or intravenous sodium tend to be preserved in patients with hyperplasia, but there is significant overlap among groups of patients.The best diagnostic test involves the measurement of cortisol and aldosterone in bilateral adrenal venous effluent and a peripheral vein before and during an ACTH infusion. Cortisol is used to evaluate catheter placement in the adrenal veins, as levels from the two sides should be similar.When an adenoma is present, the aldosterone-to-cortisol ratio on one side is usually at least five-fold greater than the other, which may be similar to the periphery, indicating suppression. Bilateral hyperplasia tends to produce similar values on each side.
REF. Goldman-Cecil 26th edition
