Diagnostic Predicament of Persistent Electrolyte Imbalance in a Neonate, Pseudohypoaldosteronism (PHA)

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.