Areeb Sohail Bangash, Nisreen Feroz Ali, Sadaf Sami, Mohammad Iqbal.
Pseudohypoaldosteronism Type-I; a rare cause of hyperkalemia in neonates.
J Pak Med Assoc Jan ;64(4):484-6.

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.

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