Primary reninism. Hypertension, hyperreninemia, and secondary aldosteronism due to renin-producing juxtaglomerular cell tumors.

Abstract

The four previously reported cases of renal juxtaglomerular (JG) cell tumors are reviewed. Together, they establish a clear clinical syndrome but, in each case, critical information is lacking. A preoperative diagnosis of a renin-producing JG cell tumor of the right kidney has provided time for intensive clinical, biochemical, morphological, and immunological studies upon an 18-year-old, severely hypertensive male and his JG cell tumor. Preoperatively, there existed severe hypokalemic aldosteronism, persistent hyperreninemia, responsive to posture but not to changes in sodium intake. Circadian rhythm of plasma renin activity (PRA) was normal despite the greatly elevated levels. Right renal vein angiotensin I (direct) and PRA levels (both by radioimmunoassay for Al) were double those of the left. Both renal arterial trees were normal and the anticipated renal cortical tumor, 15 mm in diameter, was observed roentgenographically in the right kidney. Right nephrectomy cured the entire syndrome in six days. Intense hyporeninemia was