Abstract:
:Glucocorticoid-remediable aldosteronism (GRA) is a hereditary form of primary hyperaldosteronism and the most common monogenic cause of hypertension. A chimeric gene duplication leads to ectopic aldosterone synthase activity in the cortisol-producing zona fasciculata of the adrenal cortex, under the regulation of adrenocorticotropin (ACTH). Hypertension typically develops in childhood, and may be refractory to standard therapies. Hypokalemia is uncommon in the absence of treatment with diuretics. The discovery of the genetic basis of the disorder has permitted the development of accurate diagnostic testing. Glucocorticoid suppression of ACTH is the mainstay of treatment; alternative treatments include mineralocorticoid receptor antagonists.
journal_name
Endocrinol Metab Clin North Amjournal_title
Endocrinology and metabolism clinics of North Americaauthors
Halperin F,Dluhy RGdoi
10.1016/j.ecl.2011.01.012subject
Has Abstractpub_date
2011-06-01 00:00:00pages
333-41, viiiissue
2eissn
0889-8529issn
1558-4410pii
S0889-8529(11)00013-2journal_volume
40pub_type
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journal_title:Endocrinology and metabolism clinics of North America
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journal_title:Endocrinology and metabolism clinics of North America
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journal_title:Endocrinology and metabolism clinics of North America
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journal_title:Endocrinology and metabolism clinics of North America
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