Hyperreninemia and Secondary Hyperaldosteronism in a Patient with Pheochromocytoma and von Hippel-Lindau Disease

Abstract

In a 21-year-old Caucasian woman with von Hippel-Lindau disease, norepinephrine-producing adrenal pheochromocytoma was identified as the underlying cause of severe hypertension. She was found to have extremely elevated levels of circulating renin and aldosterone, and she was markedly hypokalemic. Administration of captopril further enhanced renin secretion, while her blood pressure improved. The patient became normokalemic following tumor removal, and her blood pressure decreased to normal levels with reestablishment of normal circadian blood pressure rhythm. This case demonstrates that, in the absence of renovascular or malignant hypertension, pheochromocytoma can be the underlying cause for the clinical syndrome of hypertension associated with severe hypokalemia and hyperreninemic hyperaldosteronism.

Renal artery stenosis and aneurysmatic dilatation of arteria carotis interna in tuberous sclerosis complex

“…Hyperreninemia and secondary hyperaldosteronism in a patient with pheochromocytoma and von Hippel-Lindau disease was also reported by Lenz and colleagues [24]. Renal cysts as a frequent finding in von-Hippel-Lindau disease may also cause hypertension in these patients via hyperreninemia.…”

A case of pheochromocytoma presenting as secondary hyperaldosteronism, hyperparathyroidism, diabetes and proteinuric renal disease

“…The possibility that PHEO was able to cosecrete renin-like compounds, though reported in the literature [2], was ruled out in our case, as the saline loading test normally suppressed plasma renin and ALD. This indicated that the physiological mechanisms controlling renin release were preserved and that renin was not secreted autonomously [3]. Thus, according to the reported close association between plasma catecholamines and renin [4] and the demonstration of juxtaglomerular cell hyperplasia induced by catecholamines in patients with PHEO [5], we believe that both β-receptor-mediated renin secretion [4,6] and catecholamine-mediated systemic and also renal artery vasospasm/vasoconstriction may represent the cause of RAS activation in our patient.…”

Exhaustive exercise-induced tissue hypoxia does not change endothelin and big endothelin plasma levels in normal volunteers

“…1 The increased sympathoadrenal acitivity may partly explain the stimulated renin release, which depends on ␤ 1 -receptor activation. 24 However, another compound of the renin angiotensin system, the endothelium-derived angiotensin converting enzyme, decreased in plasma during exercise. It appears, therefore, justified to conclude that this vasoregulating system is more variable with exercise than the endothelin system.…”