P3D262: How High Is Too High? Management of Complications in LCH Following the Common Cold

Introduction: Langerhans cell histiocytosis (LCH) is a rare, idiopathic proliferation of aberrant antigen-presenting cells with a highly variable disease presentation ranging from single organ to multi-organ involvement. Development of diabetes insipidus (DI) is a relatively common sequela (24%) in patients with systemic LCH due to pituitary involvement. Additionally, patients with systemic LCH are often treated with glucocorticoids, potentially causing steroid-induced hyperglycemia. While both DI and steroid-induced hyperglycemia carry a risk of dehydration and electrolyte disturbances, there are few reports and evidence-based guidelines on their compounding effects in a single patient.

We discuss the unique case of a 12-year-old female with LCH, complicated by DI and steroid-induced hyperglycemia, found to have profound electrolyte disturbances requiring critical care in the setting of viral upper respiratory illness (URI). We hope this case provides insight and management for similar cases in the future.

Case Description: A 12-year-old female with LCH, complicated by steroid-induced hyperglycemia and central DI presented to our military treatment facility (MTF) emergency department for headache, fatigue, and polyuria in the setting of an viral URI. She was found to have elevated serum glucose and sodium levels, peaking at an estimated 747 mg/dL and 192 mmol/L respectively. She subsequently had a tonic-clonic seizure and altered mental status requiring intubation. She required careful correction of her electrolyte, free water, and glucose derangements with hypotonic fluids and insulin, stress dose steroids, and intravenous vasopressin. Her initial MRI was notable for new white matter injury with restricted diffusion; however after a 4-day hospitalization, she was discharged home with no neurologic deficits. At 6 months post- admission her neurologic exam remains normal.

Discussion: Our patient presents with what would otherwise be a mild self-limiting viral illness. However in a patient with tightly regulated electrolytes, glucose and fluid balance, poor PO and systemic viral inflammation can be severe stressor. For our patient this resulted in a hyperglycemic-hyperosmolar state, altered mental status and seizure. The complexity of her derangements in a critically ill patient limited our selection for a single treatment algorithm and our MTF had limited pediatric intensive care resources. Ultimately we used a stepwise approach, first prioritizing fluid resuscitation, then slowly correcting her sodium while optimizing her free water balance, and last managing her hyperglycemia.

Conclusion: This case highlights the need for close observation and monitoring even in mild illnesses such as a viral URI in patients with LCH. There are currently no evidenced based guidelines regarding the prevention and management of severe electrolyte derangements and altered mental status in patients with both DI and steroid-induced hyperglycemia. Our approach focused on careful glycemic control, slow purposeful correction of electrolytes and normalization of free water balance. Despite the extreme presentation, favorable outcomes are achievable even in a MTF with limited pediatric resources.