Abstract
Pseudohypoaldosteronism (PHA) type 1 is a heterogeneous group of disorder of electrolyte metabolism characterized by apparent renal tubular unresponsiveness to aldosterone action. PHA-1 is manifested by hyponatremia, hyperkalemia, metabolic acidosis and may present as salt wasting crisis with elevated levels of renin and aldosterone. PHA-1 is subdivided into two types with varying degree of severity. The earliest sign of both type of PHA-1 is poor weight gain due to dehydration and failure to thrive during infancy. Here, we report a case of renal PHA-1 in a 30 days old female infant who presented with hyponatremia, mild dehydration and hyperkalemia and was treated with dietary sodium chloride supplementation, potassium binding resin and fluid replacement therapy which proved to be lifesaving.
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