C onn emphasized the triad of hypertension, hypokalemia, and metabolic alkalosis in his seminal account of patients with primary aldosteronism. 1 He believed that primary aldosteronism was a very common cause of hypertension. Kaplan introduced the discussion as to the actual prevalence of primary aldosteronism, a topic still debated today. 2,3 Suffice it to say that hypokalemic hypertension is a fixed entity in the minds of clinicians as synonymous with primary aldosteronism, and perhaps this reaction is appropriate. Nonetheless, there are other diagnostic considerations. For instance, licorice gluttony looks exactly like primary aldosteronism clinically; the diagnosis requires a particularly high grade of detective work. 4 However, Mendelian disorders have become increasingly recognized, especially because elucidation of their molecular mechanisms provides reliable diagnostic tools. 5 Another confounder is the fact that contrary to prevailing clinical opinion, electrolyte and acid-base abnormalities are absent in many patients with primary aldosteronism. 6 We describe 2 remarkable patients who were clinical adventures for us and provided several important lessons.
Case 1An 18-year-old male student was admitted for further evaluation of suspected primary aldosteronism. A routine examination at the age of 15 years revealed a blood pressure of 150/90 mm Hg; however, no further workup was done at that time and no therapeutic consequences were drawn. Three years later, the patient's concerned mother consulted a nephrologist. Family history revealed hypertension in his father and brother. At that time, a 24-hour ambulatory blood pressure measurement (ABPM) revealed an average 24 h blood pressure of 156/98 mm Hg without a nocturnal dip. The nephrologist's attention was drawn to a low potassium level (3.46 mmol/L). The plasma aldosterone concentration (PAC) was 580 pg/mL (normal range: 70 to 295 pg/mL; corresponding to 58 ng/dL or 1609 pmol/L). The conversion factor for aldosterone from ng/dL to SI units (nmol/L) is 0.0349. The plasma renin concentration (PRC) was 1.2 pg/mL (normal range: 3 to 33 pg/mL). The conversion factor for renin in pg/mL to SI units (pmol/L) is 0.0237. The value corresponds to PRA suppressed 辖0.1 ng/mL/h; conversion outlined elsewhere. 7 The aldosterone/renin ratio (ARR) was 472. Furthermore, the serum sodium concentration was elevated at 149 mmol/L. Renal function was normal, except for microalbuminuria of 61 mg/24 h (normal range 辖30 mg/24 h). Blood gas analysis revealed a compensated metabolic alkalosis. Two liters of 0.9% saline solution were infused, but failed to achieve sufficient PAC suppression (辖50 pg/mL, 辖5 ng/dL). As a matter of fact, his PAC increased slightly. Magnetic resonance imaging (MRI) of the abdomen revealed an enlarged left adrenal gland with multiple small nodules (diameter 2 to 3 mm), which showed T2-weighted hyperintensity. The right adrenal gland appeared unremarkable. Unfortunately adrenal venous sampling (AVS) was unsuccessful, because only the left gland could be ca...