Primary aldosteronism—some genetic, morphological, and biochemical aspects of subtypes

Gordon, Richard D.; Stowasser, Michael; Klemm, Shelley A.; Tunny, Terry J.

doi:10.1016/0039-128x(94)00013-3

Cited by 49 publications

(15 citation statements)

References 38 publications

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“…This could be mainly attributable to lower basal secretion of aldosterone in PA/SCS compared to that in APA. It is well recognized that ACTH acutely stimulates aldosterone secretion [25], whereas ACTH chronically reduces aldosterone secretion [26]. Thus, the suppressed ACTH secretion due to mild cortisol excess in PA/SCS could not explain the lower PAC and higher serum potassium levels in our PA/SCS patients.…”

Section: Discussionmentioning

confidence: 73%

Clinicopathological features of primary aldosteronism associated with subclinical Cushing's syndrome

et al. 2011

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Primary aldosteronism (PA) is characterized by autonomous aldosterone hypersecretion from adrenal gland, resulting in low-renin hypertension often accompanied by hypokalemia and metabolic alkarosis. Recent epidemiologic studies have revealed that PA is more common cause of secondary hypertension than previously thought with its higher prevalence (5-15 %) in patients with hypertension [1][2][3][4][5]. Subclinical Cushing's syndrome (SCS) is characterized as subtle cortisol hypersecretion from adrenal tumor but lack of specific signs or symptoms of overt abstract. Primary aldosteronism (PA), an autonomous aldosterone hypersecretion from adrenal adenoma and/or hyperplasia, and subclinical Cushing syndrome (SCS), a mild but autonomous cortisol hypersecretion from adrenal adenoma without signs or symptoms of Cuhing's syndrome, are now well-recognized clinical entities of adrenal incidentaloma. However, the clinicopathological features of PA associated with SCS (PA/SCS) remain unknown. The present study was undertaken to study the prevalence of PA/SCS among PA patients diagnosed at our institute, and characterize their clinicopathlogical features. The prevalence of PA/SCS was 8 of 38 PA patients (21%) studied. These 8 PA/SCS patients were significantly older and had larger tumor, higher serum potassium levels, lower basal plasma levels of aldosterone, ACTH and DHEA-S as well as lower response of aldosterone after ACTH stimulation than those in 12 patients with aldosterone-producing adenoma without hypercortisolism. All 8 PA/SCS patients showed unilateral uptake by adrenal scintigraphy at the ipsilateral side, whereas the laterality of aldosterone hypersecretion as determined by adrenal venous sampling varied from ipsilateral (3), contralateral (2), and bilateral side (2). 6 PA/SCS patinets who underwent adrenalectomy required hydrocortisone replacement postoperatively. Histopathological analysis of the resected adrenal tumors from 5 PA/SCS patients revealed a single adenoma in 3, and double adenomas in 2, with varying degrees of positive immunoreactivities for steroidgenic enzymes (3β-HSD, P450 C17 ) by immunohistochemical study as well as CYP11B2 mRNA expression as measured by real-time RT-PCR. In conclusion, PA/SCS consists of a variety of adrenal pathologies so that therapeutic approach differs depending on the disease subtype.

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Section: Discussionmentioning

confidence: 73%

Clinicopathological features of primary aldosteronism associated with subclinical Cushing's syndrome

et al. 2011

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“…However, the criteria for the histopathological diagnosis of adrenal adenoma are not clearly defined and there are varying degrees of overlap between pathological morphologies -for example mixed histology, "dominant" nodules or poorly circumscribed adenomata. 11,12 Occasionally, the post-operative clinical and biochemical course of the patient is required to assess whether a cure has been achieved.…”

Section: Discussionmentioning

confidence: 99%

Adrenal venous sampling as a diagnostic procedure for primary hyperaldosteronism: experience from a tertiary referral centre

Salem

Hopkins

El-Gayar

et al. 2012

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cONtEXt: Adrenal vein sampling (AVs) is recommended in all patients with hyperaldosteronism to whom surgery would be offered if the results indicated unilateral hypersecretion. ObJEctIVE: to assess the performance of AVs against radiological findings and to evaluate the Endocrine society's Practice Guidelines for diagnostic cut-offs. PAtIENts: retrospective study of 41 patients with hyperaldosteronism who underwent both AVs and computed tomography (ct) imaging. rEsULts: ct and AVs results were concordant in 73.7%. Unilateral lesions on ct had a greater positive predictive value (85%) than non-unilateral lesions (50%). In patients with subsequently confirmed adrenal adenomas, a lateralisation ratio >2 when comparing cortisol-corrected aldosterone ratios from the affected versus unaffected side was 100% sensitive. Patients who were managed surgically experienced significant reductions in blood pressure and medication burden and 46% were cured. cONcLUsIONs: AVs is important in establishing unilateral or bilateral adrenal secretion of aldosterone in patients with primary hyperaldosteronism. However, it may not be essential for the work-up in patients below the age of 40, in whom adrenal incidentalomas adrenal incidentalomas are known to be rarer, and a unilateral lesion on ct therefore has a greater positive predictive value.

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“…In reality, however, there exists a much more diverse array of abnormal adrenal morphology among patients with PA (206,496). For example, in adrenals containing an APA, the remaining cortex frequently contains additional, smaller nodules.…”

Section: A Evidence From Studies On Adrenal Morphology In Pamentioning

confidence: 99%

“…Traditionally, PA has been divided into four main subtypes, three of them based on adrenocortical histopathology (APA, BAH, and aldosteroneproducing carcinoma) and one rare subtype distinctive for its familial occurrence and for being glucocorticoidremediable (FH-I). With increasing examination of adrenals removed from subjects with PA, it has become ap- parent that this classification is a gross simplification and the morphology associated with PA is far more diverse (206,496). Although APAs are usually unilateral, they may be bilateral, and their cellular composition varies from mostly ZF-like cells to mostly non-ZF (with cells resembling ZG or "hybrid" cells having characteristics of both ZF and ZG) (366,530).…”

Section: A New Concepts In the Definition Of Pa Subtypesmentioning

confidence: 99%

Primary Aldosteronism: Changing Definitions and New Concepts of Physiology and Pathophysiology Both Inside and Outside the Kidney

Stowasser

Gordon

2016

Physiological Reviews

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In the 60 years that have passed since the discovery of the mineralocorticoid hormone aldosterone, much has been learned about its synthesis (both adrenal and extraadrenal), regulation (by renin-angiotensin II, potassium, adrenocorticotrophin, and other factors), and effects (on both epithelial and nonepithelial tissues). Once thought to be rare, primary aldosteronism (PA, in which aldosterone secretion by the adrenal is excessive and autonomous of its principal regulator, angiotensin II) is now known to be the most common specifically treatable and potentially curable form of hypertension, with most patients lacking the clinical feature of hypokalemia, the presence of which was previously considered to be necessary to warrant further efforts towards confirming a diagnosis of PA. This, and the appreciation that aldosterone excess leads to adverse cardiovascular, renal, central nervous, and psychological effects, that are at least partly independent of its effects on blood pressure, have had a profound influence on raising clinical and research interest in PA. Such research on patients with PA has, in turn, furthered knowledge regarding aldosterone synthesis, regulation, and effects. This review summarizes current progress in our understanding of the physiology of aldosterone, and towards defining the causes (including genetic bases), epidemiology, outcomes, and clinical approaches to diagnostic workup (including screening, diagnostic confirmation, and subtype differentiation) and treatment of PA.

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Primary aldosteronism—some genetic, morphological, and biochemical aspects of subtypes

Cited by 49 publications

References 38 publications

Clinicopathological features of primary aldosteronism associated with subclinical Cushing's syndrome

Clinicopathological features of primary aldosteronism associated with subclinical Cushing's syndrome

Adrenal venous sampling as a diagnostic procedure for primary hyperaldosteronism: experience from a tertiary referral centre

Primary Aldosteronism: Changing Definitions and New Concepts of Physiology and Pathophysiology Both Inside and Outside the Kidney

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