ESPE Abstracts

Introduction: Diabetic ketoacidosis (DKA) is a potentially fatal hyperglycemic emergency primarily associated with type 1 diabetes mellitus (T1D). The association between DKA and severe hypertriglyceridemia has already been previously discussed with a prevalence ranging between 7.1%-25%, with pancreatitis present in the majority of cases. Epidemiological data in the pediatric age are scarce and not well established. Its etiology is not yet fully known. The "cryptic triangle" (DKA, hypertriglyceridemia and pancreatitis) is a rare and serious condition.

Objective: To report a case of DKA associated with severe hypertriglyceridemia without acute pancreatitis.

Case description: Female patient, 13yo, previously healthy, diagnosed with T1D and severe DKA. A lipemic sample was identified and hypertriglyceridemia (7110 mg/dL) was confirmed. At hospitalization, levels of serum amylase, lipase and imaging exams did not suggest pancreatitis. The patient was discharged using simvastatin (20 mg/day), and a basal-bolus insulin regimen (0.8 IU/kg/day).

Discussion: Hypertriglyceridemia can lead to acute pancreatitis and, consequently, to DKA by destroying pancreatic beta cells. However, it can also be a consequence of DKA, as the lack of insulin leads to an increase in triglycerides, which in turn could lead to acute pancreatitis. The association between DKA and hypertriglyceridemia occurs due to lack of insulin, leading to enlarged lipolysis, increased production of VLDL by the liver due to increased hepatic fatty acid flow, which, in addition to ketogenesis, can be secreted as VLDL. In addition, VLDL clearance is decreased by inhibiting lipoprotein lipase activity, responsible for hydrolyzing triglycerides into fatty acids, in peripheral tissues. Two main mechanisms for pancreatitis due to changes in lipid metabolism resulting from DKA are suggested: (a) fatty acids when carried by albumin are not toxic, but if they are in large quantities, endothelial lipase degrades the fatty acids and increases the pancreatic lysolecithin activity; (b) chylomicrons would damage the distal pancreatic circulation, inducing ischaemia and altering the acinar function, in addition to exposing the tissue to triglycerides and activating pancreatic lipase. The differential diagnosis between DKA associated or not with acute pancreatitis is challenging due to the overlapping symptoms and laboratory tests. DKA is a severe complication of T1D and its association with other disorders can worsen the prognosis.

Conclusion: There is a paucity of data in the literature about treatment recommendations for acute hypertriglyceridemia, with most case reports using fibrates for handling it. The triad hypertriglyceridemia, DKA and pancreatitis is associated with increased mortality and complications.