IMPE Abstracts

Introduction: Central diabetes insipidus (CDI) affects 2 to 15% of children with Langerhans cell histiocytosis due to destruction of the neurohypophysis. Management includes access to free water and desmopressin (dDAVP), a synthetic analog of AVP, which has a longer half-life than vasopressin. dDAVP can be administered orally, intranasally, or parenterally, depending on the clinical setting. Protocols describing continuous infusion of dDAVP have been reported for children with ICD and malignancies, however, no reports have been described for affected children requiring liver transplantation.

Objective: To describe the clinical management and evolution of a 3-year-old boy with multiple pituitary hormone deficiencies due to Langerhans cell histiocytosis and liver failure that required transplantation.

Clinical case: 3-year-old boy with LCH with CNS and liver involvement diagnosed at 6 months of age; who was admitted for a liver transplant. He received replacement therapy with levothyroxine, hydrocortisone, growth hormone, and oral dDAVP 3 times daily. During anesthetic induction, he received stress doses of hydrocortisone and oral desmopressin 50 mg. During surgery, he maintained diuresis (DU) between 3 and 4 cc/K/h with neutral balance and normal sodium. At 10 hours after surgery, he is hemodynamically stable on mechanical ventilation and presents with polyuria and hypernatremia. A continuous infusion of dDAVP was administered for 18 hours, which consisted of hourly blood sodium measurement, and an initial dDAVP dose of 0.05 mU/kg/hr to maintain diuresis between 3 and 4 cc/K/h. If DU < 2 cc/K/H, the infusion is suspended; DU 2-3 reduce infusion rate by 25%; DU >4-5 cc/K/h increase infusion rate by 25%; DU >5 cc/K/h increase infusion rate by 50% to maintain eunatremia (135-145 mEq/L). The child was extubated and was switched to oral treatment without polyuria, dysnatremia, or other hemodynamic or electrolyte disorders.

Conclusion: This report demonstrates the usefulness of continuous infusion of dDAVP for the management of patients with ICD who require hyperhydration, allowing strict management of sudden osmolar changes caused by polyuria and dysnatremia, avoiding secondary damage during this period, which becomes a therapeutic challenge for clinicians.