title: Pseudohypoaldosteronism type-I: a rare cause of hyperkalemia in neonates.
abstract: 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.
authors.forename: Areeb Sohail
authors.initials: AS
authors.lastname: Bangash
authors.forename: Nisreen Feroz
authors.initials: NF
authors.lastname: Ali
authors.forename: Sadaf
authors.initials: S
authors.lastname: Sami
authors.forename: Mohammad
authors.initials: M
authors.lastname: Iqbal
mesh_terms: D005260:Female; D006801:Humans; D006947:Hyperkalemia / Q000209:etiology* / Q000628:therapy; D007231:Infant, Newborn; D011546:Pseudohypoaldosteronism / Q000150:complications* / Q000175:diagnosis / Q000628:therapy