Abstract

Abstract Background Acute pancreatitis (AP) during pregnancy is a rare, potentially life-threatening disease, with an incidence of 1 case per 1,000-10,000. Hypertriglyceridemia (HTG)-induced pancreatitis accounts for only 5% of cases. Here we describe a rare case of HTG-induced AP during the second trimester of pregnancy, which was treated with concurrent insulin and plasmapheresis. Clinical Case A 35-year-old multigravida female was admitted for preterm premature rupture of membranes at 26 weeks gestation. Symptoms began on day 8 of admission and included severe epigastric pain radiating to the back. On exam, there was significant epigastric tenderness on palpation. There were no xanthelasmas or tendon xanthomas seen. She was afebrile but tachycardic. Laboratory evaluation noted a triglyceride (TG) level of >5,680 mg/dL (n<150), amylase 201 U/L (n<100) and lipase 277 U/L (n<60), consistent with AP. She was euglycemic. CT of the abdomen showed interstitial edematous pancreatitis with a peripancreatic collection. She was placed on NPO and started on intravenous (IV) fluid therapy, in combination with IV insulin and 10% dextrose. The patient also underwent one session of plasmapheresis. TG levels downtrended rapidly to 3,585 mg/dL within 24 hours. Once TG levels decreased below 1,000 mg/dL, insulin and dextrose infusions were stopped, and combination therapy of fenofibrate, omega-3 fatty acids, and low-fat low-carbohydrate diet was started. At 28 weeks and 5 days gestation, a cesarean section was performed due to suspected fetal distress. The patient was discharged on fenofibrate and Omega-3 fatty acids with a TG level of 456 mg/dL. Genetic testing revealed a heterozygous variant in the LPL gene mutation (c.644G>A; p. Gly215Glu) and a heterozygous variant of uncertain significance (VUS) in the ABCA1 gene (c.1765G>A; p. Val589Ile). She had no known family history of hyperlipidemia or cardiovascular disease. Conclusion Lipoprotein lipase (LPL) deficiency is an autosomal recessive disorder caused by biallelic pathogenic mutations in the LPL gene. The heterozygous c.644G>A variant has been previously described in several patients with LPL deficiency, however its impact on the function of the LPL protein has not yet been clearly defined. The ABCA1 gene is associated with Tangier disease, characterized by very low HDL levels and HTG. The ABCA1 gene variant detected in this case is classified as VUS as its effect on the gene function is not yet known. Further investigation is warranted to understand the role of these variants in familial dyslipidemia. Aside from the described patient, only one other case report of HCT-induced AP treated with concomitant plasmapheresis and IV insulin therapy has been reported in the literature. There are no published guidelines regarding the optimal treatment for HTG-induced AP in pregnancy. Combined treatment modality with plasmapheresis and IV insulin seems to be effective, however, its risks and benefits should be carefully assessed. Presentation: No date and time listed

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