A Case of Primary Aldosteronism Due to Unilateral Adrenal Hyperplasia

Prevalence of Primary Aldosteronism among Unselected Hypertensive Patients: A Prospective Study Based on the Use of an Aldosterone/Renin Ratio above 25 as a Screening Test

“…Minor causes include familial varieties and unilateral adrenal hyperplasia (22). Glucocorticoid-remediable aldosteronism (GRA) or familial aldosteronism type I is the only subtype of PA whose underlying genetic and molecular basis is clearly understood (23)(24)(25)(26)(27)(28). The pathogenetic mechanism derives from an asymmetrical crossing-over with a nonreciprocal recombination between the 11β-hydroxylase (CYP11B1) and ALD synthase (CYP11B2) genes.…”

“…The resulting chimeric gene has a 5′ regulatory element of CYP11B1 fused to 3′ coding sequences of CYP11B2. The preferential expression of this mutant form drives a transcription process under the control of adrenocorticotropic hormone (ACTH) instead of physiological angiotensin (Ang) II stimulus (23)(24)(25)(26)(27). Subtype detection of PA, which requires one or more tests including adrenal CT, adrenal venous sampling (AVS) and genetic blood testing, is of clinical relevance since patients with APA are potentially curable with unilateral adrenalectomy while the other forms are treated medically.…”

Small Extra-Adrenal Pheochromocytoma Causing Severe Hypertension in an Elderly Patient

“…Because her hypertension could have led to fatal cardiovascular or cerebrovascular events, we considered that it was very important to provide the most effective treatment for extraadrenal pheochromocytoma (26). Furthermore, we emphasize the importance of an accurate and prompt diagnosis in elderly patients with pheochromocytoma, who often present a less florid symptomatology and have more severe cardiovascular complications in their late years than younger patients.…”

New concepts in adrenal vein sampling for aldosterone in the diagnosis of primary aldosteronism

“…Nonetheless, differentiating between APA and IHA can be extremely challenging for three reasons: 1) there are rare cases of MUAN, or unilateral autonomous hyperplasia, that can be cured by adrenalectomy [13]; 2) at histopathology the border between a microscopic adenoma and a unilateral hyperplasia or MUAN is undefined; and 3) bilateral APA and APA in the context of BAH can exist [14,15].…”