[Primary hypoaldosteronism and secondary pseudo-hypoaldosteronism].
We observed a 23-year-old man with pronounced hyperkalemia (max. 6.8 mmol/l) and hyponatremia (min. 112 mmol/l), which had been existent for 3 years without complaint except a transitory psychorganic syndrome due to hyponatremia. Physical examination showed no abnormality except hypotension (blood pressure 100/70 mmHg). Renal function tests were normal. Fractional clearance of sodium was significantly increased (0.8%), whereas that of potassium was decreased (2.4%). Plasma renin activity was tripled and rose after furosemide. Plasma aldosterone was lowered and showed no rise after furosemide. Suppression of plasma renin and aldosterone by saline infusion was normal. Pressor dose of angiotensin II was increased (17,9 ng AT II/kg/min). Urinary excretion of aldosterone and its conjugates was below normal, and aldosterone precursors were within normal range. The findings were interpreted as selective primary hypoaldosteronism caused by corticosterone methyl oxidase defect type II. However, neither fludrocortisone (0.5 mg/day) nor sodium chloride (200 mmol/day) led to a normalization of sodium and potassium in plasma. Additional pseudohypoaldosteronism was thus assumed. Aldosterone infusion (3 mg in 1 h) decreased renal excretion of sodium; potassium excretion failed, however, to increase in contrast to its pattern in normal man. These findings resemble additional pseudohypo-aldosteronism of type II. After 8 weeks' application of additional 80 mmol sodium (as sodium bicarbonate) plasma sodium and potassium showed normal values under combined treatment with fludrocortisone (0.1 mg/day) and sodium bicarbonate (80 mmol/day). It is to be assumed that the patient suffers from a reduced aldosterone biosynthesis in the presence of an additional transitory secondary pseudohypoaldosteronism.
D000328:Adult; D000450:Aldosterone / Q000097:blood / Q000172:deficiency*; D000804:Angiotensin II / Q000097:blood; D001639:Bicarbonates / Q000008:administration & dosage; D005438:Fludrocortisone / Q000627:therapeutic use; D006801:Humans; D006947:Hyperkalemia / Q000097:blood*; D007010:Hyponatremia / Q000097:blood*; D008297:Male; D011188:Potassium / Q000097:blood; D012083:Renin / Q000097:blood; D012964:Sodium / Q000097:blood; D017693:Sodium Bicarbonate; D012965:Sodium Chloride / Q000008:administration & dosage; D014882:Water-Electrolyte Balance / Q000187:drug effects
D001639:Bicarbonates; D000804:Angiotensin II; D012965:Sodium Chloride; D000450:Aldosterone; D017693:Sodium Bicarbonate; D012964:Sodium; D012083:Renin; D011188:Potassium; D005438:Fludrocortisone
Primärer Hypoaldosteronismus und sekundärer Pseudo-Hypoaldosteronismus.