Krisztina Lukacs, Laszlo Jozsef Barkai, Nora Hosszufalusi, Eva Palik, Attila J Szabo and Laszlo Madacsy
A case is presented of a 15-year-old boy with a past medical history of hyperlipidemia and hypertension. He attended the emergency department with a 3-day history of vomiting, acute abdominal pain, and altered mental status. Laboratory data on admission revealed metabolic acidosis (pH: 7.12, BE: -20.8 mmol/L), high blood glucose level (32.1 mmol/L) and significant hyperlipidemia (cholesterol: 16.3 mmol/L, triglycerides: 21.1 mmol/L). Treatment with electrolytes and volume replacement and intravenous insulin successfully resolved the ketoacidosis, but the abdominal pain and hyperlipidemia remained. Abdominal US and CT scan showed severe necrotizing pancreatitis with a pseudocyst. The laboratory studies showed a Frederickson type V pattern hyperlipidemia. HbA1c was 14.3% (133 mmol/mol), indicating the presence of chronic glucose elevation. Based on the lack of islet cell antibodies and the normal fasting serum C-peptide level, type 2 diabetes was diagnosed. His HLA DQB1 genotype is associated with neutral autoimmune diabetes risk. The rare and enigmatic triad of diabetic ketoacidosis, hyperlipidemia and acute pancreatitis has been reported in a few adult and childhood cases. The pathomechanism is not clear and the association among the members of the triad may have four different explanations. All previous reports in children, suggest that ketoacidosis occurring in type 1 diabetes as first symptom can cause hyperlipidemia, and consequent acute pancreatitis. However, to the best of our knowledge, this is the first report of a pediatric patient presenting with primary hyperlipidemia-induced acute necrotizing pancreatitis complicated by diabetic ketoacidosis at the onset of non-autoimmune diabetes