ODP153 Recurrent hypertriglyceridemia-induced pancreatitis due to multifactorial chylomicronemia syndrome in a patient with ketosis-prone diabetes mellitus
Authors: Thewjitcharoen Yotsapon , Chatchomchuan Waralee , Komindr Surat , Wattanawiroon Kewalin , Nakasatien Soontaree , Himathongkam Thep 
Published: November 2022

Background

Hypertriglyceridemia-induced pancreatitis is associated with significant morbidity and mortality. The triad of diabetic ketoacidosis (DKA), severe hypertriglyceridemia, and acute pancreatitis have been occasionally described in severely obese patients with type 2 diabetes mellitus (T2DM). However, the occurrence of this triad in newly diagnosed ketosis-prone diabetes mellitus (KPDM) is rarely reported. Herein, we report a long-term follow-up of a Thai man with KPDM complicated with recurrent pancreatitis due to multifactorial chylomicronemia syndrome.

Case Description

A 33-year-old Thai male patient with a history of insulin-independent diabetes mellitus, recurrent hypertriglyceridemic pancreatitis, and recurrent DKA (three times within the past 5 years) came to our hospital in 2020 for the management of plasma triglyceride levels. At the age of 29 years (BMI 31.2 kg/m2), he experienced the first hypertriglyceridemia-induced pancreatitis (plasma triglyceride 8,189 mg/dL) in association with DKA. After resolution of DKA, insulin therapy was successfully discontinued for 3 years. Then, recurrent hypertriglyceridemic pancreatitis in association with multiple organ failures was developed during travelling to India. After treatment with plasmapheresis and continuous intravenous insulin infusion, he was able to extubate but pulmonary embolism at right pulmonary artery was discovered. Subcutaneous low molecular weight heparin was given for 6 months and then discontinued. The last hypertriglyceridemia-induced pancreatitis attack in association with DKA was aggravated by a high fat buffet meal 1 month prior to our hospital visit. He denied any tobacco, alcohol, or substance use. The patient had no known family medical history of lipid disorders. Further investigations revealed preserved beta-cell function by mixed meal stimulation test and negative pancreatic autoantibodies. Genetic testing showed no mutation in lipoprotein lipase (LPL) and its co-factors. Therefore, multifactorial chylomicronemia syndrome with KPDM was diagnosed. The patient was referred to multidisciplinary team for lifelong weight loss, limiting intake of fat and simple carbohydrates, and adherence to lipid-lowering medications. Subsequent follow-up 2 years later showed no recurrent pancreatitis.

Conclusion

The triad of acute pancreatitis, severe hypertriglyceridemia and DKA constitutes a unique subgroup of patients which insulin deficiency state is the core defect in association with underlying genetic predisposition. Molecular diagnosis of underlying genetic defect should be pursued to exclude familial chylomicronemia syndrome. In patients with multifactorial chylomicronemia syndrome, long-term management with dietary modifications together with pharmacotherapy remains the cornerstone of successful treatment.

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