[Pseudohypoaldosteronism type I: systemic form]

Pseudohypoaldosteronism type 1 [PHA1] is a rare condition characterized by renal insensitivity to the action of mineralocorticoids. Patients manifest neonatal salt wasting, hyperkalemia, and metabolic acidosis despite elevated aldosterone levels. Autosomal recessive and dominant or renal forms of the disease have been described. In the recessive form, patients have salt wasting from the kidney, colon, sweat, and salivary glands; because of dramatic volume depletion, patients require massive sodium supplementation throughout life. We report a case of a one month and three weeks old who presented a systemic type 1 pseudohypoaldosteronism