Abstract
Background: Severe hypertriglyceridemia (SHTG) in children is a rare but clinically significant disorder associated with recurrent acute pancreatitis and substantial morbidity. Early identification and prompt management are essential to prevent pancreatic and systemic complications. Methods: We report the case of an 11-year-old female with a history of xanthogranulomatous pancreatitis who presented with extreme hypertriglyceridemia, with fasting triglyceride levels exceeding 4000 mg/dL. Results: The patient was treated acutely with continuous intravenous aspart insulin (0.1 U/kg/hour) and adjusted 10% glucose infusion, with hourly glucose and potassium monitoring, leading to a rapid and marked reduction in triglyceride levels-55% reduction within the first 24 h, 76% at 48 h, and 82% after 96 h of treatment. No hypoglycemia or other adverse effects were observed. Nutritional management included a low-long-chain triglyceride (LCT) diet enriched with medium-chain triglycerides (MCTs) and omega-3 fatty acids, providing essential calories while minimizing chylomicron production. Over a 12-month follow-up, the patient remained asymptomatic, with sustained lipid normalization and no recurrence of pancreatitis. Conclusions: This case underscores the therapeutic value of combining pharmacologic and dietary strategies in pediatric SHTG. Evidence from pediatric and adult studies supports the role of insulin infusion for acute triglyceride lowering and MCT-based nutritional therapy for long-term control. Our findings highlight the need for early, individualized, and multidisciplinary management and emphasize the potential future role of emerging targeted therapies in addressing refractory pediatric hypertriglyceridemia.