Correctable subsets of primary aldosteronism. Primary adrenal hyperplasia and renin responsive adenoma.
Among 154 cases of primary aldosteronism seen in the General Clinical Research Center at San Francisco General Hospital, twelve patients did not fulfill established characteristics of an aldosterone producing adenoma (APA) or idiopathic hyperaldosteronism (IHA). Eight patients had nodular adrenocortical hyperplasia; plasma and urinary aldosterone were elevated and responses to stimulatory and suppressive maneuvers demonstrated the same autonomy seen in patients with APA. This subset is designated primary adrenal hyperplasia. Four additional patients also had elevated aldosterone levels that were responsive to these maneuvers, similar to IHA, but had unilateral tumors. This group has been designated as aldosterone-producing renin-responsive adenoma. Eleven patients had unilateral adrenalectomy and one preferred prolonged spironolactone therapy, resulting in a sustained cure or amelioration of hypertension, hypokalemia and normalization of aldosterone production.
Irony
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San Francisco General Hospital Medical Center, California.
Kater
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Biglieri
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Shackleton
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CH
D015069:18-Hydroxycorticosterone / Q000097:blood / Q000652:urine; D000236:Adenoma / Q000097:blood / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy / Q000652:urine; D000310:Adrenal Gland Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy / Q000652:urine; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood / Q000175:diagnosis* / Q000628:therapy / Q000652:urine; D000315:Adrenalectomy; D000328:Adult; D000450:Aldosterone / Q000097:blood / Q000652:urine; D002648:Child; D003900:Desoxycorticosterone / Q000097:blood; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D006929:Hyperaldosteronism / Q000097:blood / Q000145:classification* / Q000628:therapy / Q000652:urine; D008297:Male; D008875:Middle Aged; D012083:Renin / Q000378:metabolism*; D013148:Spironolactone / Q000627:therapeutic use
D013148:Spironolactone; D003900:Desoxycorticosterone; D000450:Aldosterone; D015069:18-Hydroxycorticosterone; D012083:Renin; D006854:Hydrocortisone