El Sori (1997) (Kuwait Med J 1997; 29(3):341-4) described a Kuwaiti female infant who suffered from severe salt-wasting and life threatening hyperkalemia. She responded to salt supplements and ion-exchange resin.

Khan and Manzar (2000) reported a case of a 10-day-old male with normal male genitalia. The infant, born to consanguineous parents, presented with refusal of feeding and excessive crying. He was found to be dehydrated, with hyponatremia and hyperkalemia, which persisted despite fluid resuscitation. Further investigations revealed chloride of 87mmol/L, urea of 44umol/L, glucose of 4.4mmol/L, calcium of 3.22mmol/L, and lactate of 0.88mmol/L, while ABG showed metabolic acidosis (pH: 7.24, pCO2: 2.4 Kpa, PaO2: 9.8Kpa, HCO3: 11.8mmol/L, BE: -17.9mmol/L, and O2 sat: 92.6%). The urinary pH was 5 despite bicarbonate therapy. Renal ultrasound was normal. With the above results, a diagnosis of congenital adrenal hyperplasia was made and the patient was started on mineralocorticoids and a bolus of bicarbonate, followed by hypertonic saline. Further increase in his serum potassium (8.1mmol/L) required administration of a potassium chelator, and a beta-adrenegic receptor agonist. The above diagnosis was then changed to pseudohypoaldosteronism on the basis of findings of normal levels of 17-hydroxyprogesterone, plasma cortisol, urinary 17-ketoacids, and low urinary pregnantriol levels, along with very high levels of serum aldosterone and rennin, and the fact that there was also a family history (two cousins) of pseudohypoaldosteronism. The infant was then prescribed oral hypertonic saline, oral sodium bicarbonate, and a potassium chelator. Upon follow-up, the patient was found to be thriving well, maintaining normal levels of sodium and potassium, and had no acidosis. Khan and Manzar (2000) concluded that the diagnoses of pseudohypoaldosteronism and congenital adrenal hyperplasia should both be considered in any neonate presenting late with dehydration, hyponatremia, and hyperkalemia.