A Rare Case of Pseudohypoaldosteronism Type I in a Neonate: Case Report

31 week preterm baby presented at 12 days of life with shock, hypotension, hyponatremia, hyperkalaemia and metabolic acidosis. Congenital Adrenal Hyperplasia was initially suspected. The baby was unresponsive to steroid replacement and had normal 17 OHP and elevated aldosterone levels suggesting Pseudohypoaldosteronism. The baby was aggressively managed with fluid, sodium and potassium lowering therapy. The clinical condition was complicated by bacterial sepsis resulting in the baby's death.