Abstract
Severe hypertriglyceridemia is defined by triglycerides levels reaching 11,3 mmol/L (1000 mg/dL) or when chylomicrons are present in the circulation (1). Severe hypertriglyceridemia increases the risk of acute pancreatitis, a serious complication that can be recurrent and fatal. Severe hypertriglyceridemia is most often caused by an underlying primary chylomicronemia disease, either monogenic of multifactorial. Familial chylomicronemia syndrome is a rare autosomal recessive disease that can result from mutations in lipoprotein lipase in more than 90% of cases. Multifactorial chylomicronemia is an oligogenic or polygenic disorder characterized by a reduction in lipoprotein lipase activity. Primary chylomicronemia should be managed in a specialized lipid clinic before acute pancreatitis occur. However, primary chylomicronemia is underdiagnosed (2). This is why hospitalization for an acute episode of hypertriglyceridemia-induced pancreatitis is a window of opportunity to investigate these patients.
The first objective of our study was to investigate retrospectively the quality of the follow-up of patients hospitalized for hypertriglyceridemia-induced acute pancreatitis, specifically looking at specialized lipid clinic referrals. The second objective was to correlate triglyceride (TG) levels with severity of the pancreatitis.
A total of 1063 patients were hospitalized for acute pancreatitis at our center between 2012 and 2019. Twenty-five patients (2.35%) were diagnosed with hypertriglyceridemia-induced pancreatitis. Of those 25 patients, one died of pancreatitis complications, two were already diagnosed with primary chylomicronemia, and of the remaining twenty-two, 11 (50%) had a referral for an evaluation at a specialized lipid clinic. Those referrals resulted in three diagnoses of primary chylomicronemia, with a multidisciplinary follow-up. Regarding severity of pancreatitis, TG levels were positively correlated with patients being hospitalized in the intensive care unit (ICU) and also having longer stays in the ICU.
Only half of patients hospitalized for a hypertriglyceridemia-induced pancreatitis at our center had a referral for lipid disorder evaluation upon hospital discharge. We believe it is important to raise awareness concerning primary chylomicronemia to avoid recurrent acute pancreatitis preceding diagnosis, seeing this pathology increases morbidity and mortality.
1. Valdivielso et al. Eur J Intern Med. 2014;25(8):689-94. 2. Brown et al. J Clin Lipidol. 2018;12(2):254-263.
Management of severe hypertriglyceridemia due to lipoprotein lipase deficiency in children
