Primary Aldosteronism - Hypertension with a Genetic-Basis

Gordon, Roland D., Klemm, Shelley A., Tunny, Terry J. and Stowasser, Michae. (1992) Primary Aldosteronism - Hypertension with a Genetic-Basis. Lancet, 340 8812: 159-161. doi:10.1016/0140-6736(92)93225-C

Author Gordon, Roland D.
Klemm, Shelley A.
Tunny, Terry J.
Stowasser, Michae.
Title Primary Aldosteronism - Hypertension with a Genetic-Basis
Journal name Lancet   Check publisher's open access policy
ISSN 0140-6736
Publication date 1992-07-01
Year available 1992
Sub-type Article (original research)
DOI 10.1016/0140-6736(92)93225-C
Open Access Status Not yet assessed
Volume 340
Issue 8812
Start page 159
End page 161
Total pages 3
Place of publication London United Kingdom
Publisher The Lancet Publishing Group
Language eng
Subject 2700 Medicine
Abstract Exciting developments in knowledge of primary aldosteronism include description of new subtypes and elucidation of the genetic basis of one variety. Furthermore, relatively simple biochemical screening (aldosterone/renin ratio) has disclosed that primary aldosteronism is more common than previously thought, by diagnosing patients at an earlier, normokalaemic stage. The mutant gene discovered in the glucocorticoid-suppressible variety (FHI) codes for an aldosterone biosynythetic enzyme normally controlled by angiotensin II, and now controlled by corticotropin. The zona fasciculata is hyperplastic and makes aldosterone and "hybrid steroids" 18-oxocortisol and 18-hydroxycortisol in excess, in response to ACTH but not to angiotensin II. Adrenal tumours have not yet been described in this condition. Aldosterone-producing adenomas (Conn's syndrome) are also commonly composed of zona fasciculata-like cells, make "hybrid steroids" in excess and are very sensitive to ACTH but not to angiotensin II. We have described a new variety of aldosterone-producing adenoma which is responsive to angiotensin II (AII-responsive APA), consists of at least 20% zona glomerulosa-like cells, and does not make "hybrid steroids" in excess. We have also described a new familial variety of primary aldosteronism that includes tumours and is not glucocorticoid-suppressible (FHII). We propose that primary aldosteronism is a spectrum of genetic diseases expressed as either hyperplasia or neoplasia, and that morphological and genetic diversity explains biochemical and clinical behaviour.
Keyword Suppressible Hyper-Aldosteronism
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
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