Pseudohypoaldosteronism Type-I; a rare cause of hyperkalemia in neonates

Pseudohypoaldosteronism type i (pha-i) is a rare disorder with only a few cases reported worldwide. it appears early in life with salt-wasting, failure to thrive, dehydration, hypotension, hyperkalaemia and metabolic acidosis. there is a resistance to aldosterone by the mineralocorticoid receptors. we describe one such case of a 14-day-old female neonate who presented with frequent episodes of dehydration, hyperkalaemia and hyponatraemia. on further workup, she proved to be a case of pha-i. the aim of this report is to discuss the evaluation and to highlight the difficulties associated with the management of this rare disorder.