Two Cases with Pseudohypoaldosteronism / 대한소아내분비학회지

ABSTRACT

Pseudohypoaldosteronism is a disorder in which synthesis of aldosterone is normal but unresponsiveness of the target to the aldosterone activates angiotensin-renin system which in turn elevates levels of renin and aldosterone. This salt-losing syndrome causes life-threatening hyponatremia and hyperkalemia. Despite of the normal renal and adrenal function, due to deficiency of aldosterone function, reabsorption of sodium and excretion of potassium in the kidney is impaired. Sodium loss not only from the kidney but also from sweat gland, salivary gland and colon may occur in some cases. We experienced two cases of pseudohypoaldosteronism in a 3-day-old male and 6-month-old female. The hyponatremia, hyperkalemia, elevation of plasma renin activity and aldosterone concentration were observed without renal and adrenal dysfunction. Brief review and related literatures were also presented.