SUN-074 Hereditary Hypertriglyceridemia and Recurrent Pancreatitis: The Role of Insulin Drip

Abstract

Introduction Type I hyperlipoproteinemia is a rare autosomal recessive disorder stemming from homozygous deficiency of lipoprotein lipase activity (LPL) leading to accumulation of serum chylomicrons, eruptive xanthomas and recurrent pancreatitis due to severe hypertriglyceridemia with significant cardiovascular burden. The only effective therapy in these patients is strict low-fat diet, however during hospitalization aggressive medical intervention including high cost plasma apheresis in efforts to minimize multi-organ failure. Insulin drip is often used to lower TG by enhancing LPL thus decreases release of free fatty acids (FFA), however current evidence for this subtype of patients are lacking. Herein we sought to present a case of recurrent severe type 1 hypertriglyceridemia-induced pancreatitis (HTGP). Case 65 year old white male with long standing history of familial type I hyperlipoproteinemia diagnosed in 1970s, previously received multiple plasma exchange in the past for TG levels of nearly 9000, insulin dependent type 2 diabetes with complicated peripheral neuropathy, extensive vasculopathy, recently discharged from hospital following acute on chronic HTGP with associated pancreatic calcifications, pseudocysts and necrotic tissue on CT scan, presented to ED as a seventh admission within the past 7 months with recurrent epigastric pain, nausea and vomiting concerning for recurrent HTGP. Patient was found to have lactescent serum with TG levels of 1700, previously 3000+, elevated lipase with mild neutrophilic leukocytosis and pseudohyponatremia . Patient was immediately made NPO, started on aggressive IV fluid repletion therapy. Prior to commencing IV insulin drip repeat TG downtrended to nearly 1000 within a few hours of admission. Two days later his TG continued to improve at a slower rate and was subsequently discharged home on continued statin therapy, low-fat diet and tight blood sugar control. On prior admissions patient presented with TG 1200 which later downtrended to under 700 by being strictly NPO, yet continues to receive insulin. Discussion This case highlights the challenges of managing patients with recurrent HTGP refractory to lifestyle modification, resulting in excessive readmission rates, high cost associated with plasma apheresis. IV insulin drip has been used in other subclasses of lipoproteinemia, however it is continued to be used in patients with type 1 familial hyperlipoproteinemia despite lack of strong evidence in current literature. Due to absent LPL activity, the use of insulin is rendered questionable. More research is warranted in understanding the appropriate and cost-effective treatment. References Stefanutti C,Labbadia G,Morozzi C. Severe hypertriglyceridemia-related acute pancreatitis. Ther Apher Dial. 2013 Apr;17(2):130-7