When is the benefit greater than the risk? 
Background: Pseudohypoaldosteronism type I (PHA I) is characterized by a mineralocorticoid resistance caused by an inactivating mutation of the gene encoding ENaC. It can lead to salt wasting crises and severe hyperkalemia causing life-threatening cardiac arrhythmias. The systemic form of the disease requires a life-long salt supplementation (1).
Case report: A 6-year-old boy, weighting 16,5 Kg, diagnosed with systemic PHA I was proposed for tonsillectomy, adenoidectomy and myringotomy. Treatment included 51 mEq Na+/kg/day fractioned in 5 meals (total of 240ml NaCl 20%).
By suggestion of his paediatric endocrinologist, we request an analytic study at admission which revealed hyponatremia (129 mmol/L) being salt supplementation desirable during the surgery. After induction and orotracheal intubation, a gastric tube was placed and 50ml de NaCl 20% was administered. Metoclopramide, dexamethasone and ondansetron were administered. Surgery lasted for 90 minutes. The child was extubated uneventfully when fully awake. Postoperative plasma sodium was 145 mmol/L. The recovery was made in a pediatric intermediate care unit. He was discharged home two days after surgery, asymptomatic. 
Discussion: PHA I is a rare disease that places patient at risk of severe dehydration and ionic imbalance(2). In this case, we decided to administer NaCl through a gastric tube in order to prevent severe hyponatremia and hypercalemia. The ionic variations in this child are difficult to predict, so we decided to prompt correct the imbalances. It would be impossible to perform this amount of sodium supplementation intravenously due to the large volume of fluid needed in short time. Although there was the possibility of supplementation at the end of surgery it would still exist possibility of regurgitation and aspiration. We are aware of some conditions that we could optimize before surgery, namely oral ingestion of liquids until 2 hours before surgery and early onset of fluid therapy. We noticed that we inadvertently overcorrect the sodium level to which may contribute intraoperative use of dexamethasone and hydrocortisone. 
Learning points: Anaesthesiologist always must balance the risks and the benefits of his attitudes. Maybe sometimes it worth it to take the risks.

05 / When is the benefit greater than the risk?