Assessment of Platelet Aggregation and Thrombin Generation in Patients with Familial Chylomicronemia Syndrome Treated with Volanesorsen: A Cross-Sectional Study.

BACKGROUND: The antisense oligonucleotide against APOC3 mRNA volanesorsen was recently introduced to treat Familial Chylomicronemia Syndrome (FCS). Cases of decreased platelet count are reported among patients treated with volanesorsen. The aim of the study was to evaluate platelet function and thrombin generation (TG) assessment in FCS patients receiving volanesorsen. We performed a cross-sectional study on FCS patients treated with volanesorsen. METHODS: Changes in platelet count PLC were assessed from baseline to Tw12 and Tw36. To assess TG, samples were processed by CAT (with PPP-reagent LOW). The results were expressed by the thrombogram graphic (thrombin variation over time); LagTime; endogenous thrombin potential (ETP); peak; time to reach peak (ttpeak), StartTail and Velocity Index. Platelet aggregation was assessed by testing different agonists using the turbidimetry method. RESULTS: Four FCS patients and four matched healthy controls were included in the present study. Changes in PLC were 30% at Tw12 and 34% at Tw36. Thrombin generation results showed values in the normal range (for patients and controls, respectively, LagTime:10.42 ± 4.40 and 9.25 ± 0.99; ttPeak:14.33 ± 4.01 and 13.10 ± 0.67; StartTail: 32.13 ± 3.54 and 29.46 ± 1.69; Velocity Index: 20.21 ± 3.63 and 33.05 ± 13.21; ETP: 599.80 ± 73.47 and 900.2 ± 210.99; peak value: 76.84 ± 1.07 and 123.30 ± 39.45) and no significant difference between cases and controls. Platelet aggregation test showed values in range, with no significant difference compared to healthy controls. CONCLUSIONS: Our study showed for the first time that no significant changes in general hemostasis assessed by TG and in platelet function were observed in FCS patients receiving volanesorsen.

Treatment of severe hypertriglyceridemia through therapeutic plasma exchange in patients with acute pancreatitis or at risk of developing it. / Tratamiento de la hipertrigliceridemia grave mediante recambio plasmático terapéutico en pacientes con pancreatitis aguda o en riesgo de padecerla.

INTRODUCTION AND OBJECTIVES: TPE drastically reduces serum triglyceride (sTG), but its role in the treatment of hypertriglyceridemia-induced acute pancreatitis (HTG-AP) or at risk of developing it, is not well established. The objectives were to assess the effectiveness and safety of TPE in the treatment of severe HTG (sHTG), as well as to evaluate the severity of HTG-AP treated with TPE. MATERIALS AND METHODS: Observational-retrospective-single-center study, in which a descriptive analysis of sHTG treated with TPE was conducted, with the aim of treating HTG-AP or preventing its recurrence. TPE was performed if sTG≥ 1000 mg/dL after 24 hours of admission. RESULTS: 42 TPE were performed to treat 35 sHTG in 23 patients: 29 HTG-AP, and 6 sHTG with previous HTG-AP. Among the patients, 37% (13/55) were women, with 37 ± 14 years-old, 74.3% had normal BMI (25/35), 34% (12/35) were drinking > 40 g/alcohol/day and 54% (19/35) were diabetics. TPE significantly reduced the baseline sTG (4425 ± 2782 mg/dL vs. 709 ± 353 mg/dL, p < 0.001) in a single session, achieving a mean percentage reduction of 79 ± 13%; 20% (7/35) of sHTG cases required two TPE sessions to reduce sTG to < 1000 mg/dL. Adverse effects were reported in 4/42 TPE sessions (9,5%). sHTG-AP was observed in 3% of cases (1/29), and there were no deaths. sTG at 24 hours of admission showed no relation with the severity of APs. CONCLUSION: The treatment of sHTG with TPE, with the aim of treating HTG-AP or preventing its recurrence, reduces sTG quickly and safety.

Germline variant analysis from a cohort of patients with severe hypertriglyceridemia in Brazil.

Hypertriglyceridemia (HTG) is a common dyslipidemia associated with an increased risk of cardiovascular disease and pancreatitis. It is well stablished that the severe cases of disease often present with an underlying genetic cause. In this study, we determined the frequency and variation spectrum of genes involved in the triglyceride metabolism in a series of Brazilian patients with severe HTG. A total of 212 patients with very high HTG, defined with fasting triglycerides (TG) ≥ 880 mg/ dL, that underwent a multi-gene panel testing were included in this research. Germline deleterious variants (i.e. Pathogenic/Likely Pathogenic (P/LP) variants) were identified in 28 out of 212 patients, reflecting an overall diagnostic yield of 13% in our cohort. Variants of unknown significance (VUS) were identified in 87 patients, and represent 80% of detected variants in this dataset. We confirm the LPL as the most frequently mutated gene in patients with severe HTG, and we had only one suspected case of familial chylomicronemia syndrome, caused by a homozygous variant in LMF1, in our cohort. Notably, we report 16 distinct and novel variants (P/LP and VUS), each of them representing a single case, not previously reported in any public databases or other studies. Our data expand our knowledge of genetic variation spectrum in patients with severe HTG in the Brazilian population, often underrepresented in public genomic databases, being also a valuable clinical resource for genetic counseling and healthcare programs in the country.

Efficacy and safety of omega-3-acid ethyl acetate 90 capsules in severe hypertriglyceridemia: A randomized, controlled, multicenter study.

Omega-3-acid ethyl acetate 90 capsules (containing 465 mg of eicosapentaenoic acid and 375 mg docosahexaenoic acid) is composed of highly purified omega-3 polyunsaturated fatty acid (PUFA) ethyl esters, whose lipid-lowering effect for severe hypertriglyceridemia (HTG) treatment is unclear. This study aimed to evaluate the efficacy and safety of omega-3-acid ethyl acetate 90 capsules in patients with severe HTG. In this randomized, double-blind, placebo-controlled, multicenter study, 239 patients with severe HTG were enrolled and randomized (1:1) into omega-3 group (N = 122) and placebo group (N = 117) to receive 12-week corresponding treatments. Lipid-related indexes were obtained at treatment initiation (W0), 4 weeks (W4), W8, and W12 after treatment. Adverse events and adverse drug reactions were recorded. Triacylglycerols (TAG), total cholesterol (TC), non-high-density lipoprotein cholesterol (non-HDL-C), very-low-density lipoprotein cholesterol (VLDL-C), and apolipoprotein C-III (Apo C-III) at W4, W8, and W12 were decreased in the omega-3 group versus the placebo group (all p < 0.05). Moreover, the percentage changes of TAG, TC, non-HDL-C, and VLDL-C from W0 to W4, W8, and W12, and the percentage change of Apo C-III from W0 to W4 and W8, were more obvious in the omega-3 group compared with the placebo group (all p < 0.05). However, no difference was observed in the percentage changes of HDL-C, low-density lipoprotein cholesterol (LDL-C), and LDL-C/HDL-C ratio during follow-up between groups (all p > 0.05). Additionally, there was no discrepancy in adverse events and adverse drug reactions between groups (all p > 0.05). Omega-3-acid ethyl acetate 90 capsules exhibit satisfied lipid-lowering effect with tolerable safety profile in patients with severe HTG.

Congenital Hyperlipidemia in Infants for Congenital Cardiac Surgery.

Infants with concurrent severe hypertriglyceridemia and complex congenital heart disease are a rare occurrence and can have life-threatening consequences when undergoing surgical intervention. This case series outlines two instances involving infants undergoing total anomalous pulmonary venous connection repair and surgical closure of a ventricular septal defect. The study explores troubleshooting the effects of hypertriglyceridemia on perioperative outcomes.

Characterizing genetic profiles for high triglyceride levels in U.S. patients of African ancestry.

Hypertriglyceridemia (HTG) is a common cardiovascular risk factor characterized by elevated triglyceride (TG) levels. Researchers have assessed the genetic factors that influence HTG in studies focused predominantly on individuals of European ancestry. However, relatively little is known about the contribution of genetic variation of HTG in people of African ancestry (AA), potentially constraining research and treatment opportunities. Our objective was to characterize genetic profiles among individuals of AA with mild-to-moderate HTG and severe HTG versus those with normal TGs by leveraging whole-genome sequencing data and longitudinal electronic health records available in the All of Us program. We compared the enrichment of functional variants within five canonical TG metabolism genes, an AA-specific polygenic risk score for TGs, and frequencies of 145 known potentially causal TG variants between HTG patients and normal TG among a cohort of AA patients (N = 15,373). Those with mild-to-moderate HTG (N = 342) and severe HTG (N ≤ 20) were more likely to carry APOA5 p.S19W (odds ratio = 1.94, 95% confidence interval = [1.48-2.54], P = 1.63 × 10-6 and OR = 3.65, 95% confidence interval: [1.22-10.93], P = 0.02, respectively) than those with normal TG. They were also more likely to have an elevated (top 10%) polygenic risk score, elevated carriage of potentially causal variant alleles, and carry any genetic risk factor. Alternative definitions of HTG yielded comparable results. In conclusion, individuals of AA with HTG were enriched for genetic risk factors compared to individuals with normal TGs.

Early detection of severe hypertriglyceridemia using teleconsultation in a clinical laboratory setting.

BACKGROUND: Teleconsultation in the context of clinical laboratories is a valuable tool for the early detection of dyslipidemia and prevention of cardiovascular risk. Here, we describe a patient who was referred to the Lipid Unit of the Virgen Macarena Hospital due to an alert for severe hypertriglyceridemia through its teleconsultation program. CASE PRESENTATION: A comprehensive clinical and biochemical study of the patient was carried out, and genetic testing was performed on the patient and his family. The proband and his family showed mild to severe hypertriglyceridemia and various secondary factors, together with a genetic background associated with a triglyceride-raising effect. CONCLUSION: This extensive study has identified a family at high risk of cardiovascular disease and acute pancreatitis. These findings can help maximize lifestyle changes and improve the clinical management of their dyslipidemia.

Factors affecting clinical outcomes of continuous and intermittent plasmapheresis in patients with severe hypertriglyceridemia.

INTRODUCTION: Acute hypertriglyceridemia is considered a category III indication for plasmapheresis. The use of plasma as replacement fluid (RF) has been suggested to replace the consumed lipoprotein lipase. Heparin when used as an anticoagulant could possibly release lipoprotein lipase, thereby increasing triglyceride clearance. METHODS: The impact of RF (albumin vs fresh frozen plasma (FFP) and anticoagulant (ACD-A vs. heparin) on triglycerides following plasmapheresis in 27 patients with severe hypertriglyceridemia (SHTG) was investigated. A paired study of four patients with recurrent SHTG was conducted, evaluating continuous (Optia) versus intermittent flow plasmapheresis (Haemonetics). RESULTS: Shorter procedures positively impacted triglycerides (TG) drop post-sessions p < 0.05. In albumin sessions, patients who used heparin demonstrated significantly greater drop in TG and required less sessions than did those with citrate p < 0.05. In heparin sessions, patients who used albumin demonstrated significantly greater drop in triglycerides and required less sessions than did those with FFP p < 0.05. Three of six patients who used FFP and heparin showed a triglyceride drop of 11.7% following three sessions and a 50% drop with one albumin session. Compared with Haemonetics, Optia removed comparable volumes of plasma in less time, processing smaller blood volumes and using less citrate p < 0.05. Patients demonstrated significantly lower drop in TG and required more sessions with Haemonetics than they did with Optia p < 0.05. CONCLUSION: Shorter procedure was the main predictor for effective TG clearance. This can be achieved by continuous apheresis technology, particularly when using albumin as RF. TG removal via Optia seems to be optimized by using heparin.

Approach to the Management of Hypertriglyceridemia Complicated With Acute Pancreatitis in Pregnancy: A Case Report.

This is a case of a 32-year-old woman, Gravida 3 para 2, previous two cesarean sections, who presented to our emergency department at 24+3 weeks of gestation complaining of severe epigastric pain radiating to the back. She was diagnosed with severe hypertriglyceridemia complicated with acute pancreatitis and was managed by a multi-disciplinary team, which included obstetrics, gastroenterology, endocrinology, hematology, nutrition, and ICU team. Initially, conservative treatment was employed for her management. She was placed on nil per oral status and initiated on a normal saline infusion at a rate of 150 ml/hour, along with insulin infusion at 0.1 unit/kg/hour and dextrose (D5) at 80 ml/hour. Additionally, she received omeprazole, meropenem, clexane (40 mg once daily subcutaneous injection), iron, vitamin supplements, and analgesics as required. Subsequently, due to the failure of the initial conservative medical management, the patient was admitted to the ICU. Plasmapheresis was performed after the insertion of a vascath, using 3000 ml of albumin 5% as replacement fluid and oral calcium. Following this, she was prescribed Omacor (Omega 3) at a dosage of 2 grams orally twice daily, along with a low carbohydrate and fat diet, to manage her triglyceride levels. After the removal of the central line, her triglycerides increased to 14.3 mmol/L, leading to the initiation of fenofibrate at a daily dose of one tablet. With persistent elevation to 16.4 mmol/L, Lipitor at 40 mg once daily was introduced. Following this intervention, her triglyceride levels stabilized, and her overall condition improved. She was discharged at 25+1 weeks with a prescribed regimen, and scheduled follow-ups were arranged in the endocrine and obstetrics clinics. At 36 weeks of gestation, she presented to the emergency room with abdominal, back, and leg pain. Fetal distress, indicated by fetal tachycardia (170-180 bpm) on cardiotocography, prompted an urgent category 1 cesarean section, which proceeded without complications.

Acute pancreatitis risk in multifactorial chylomicronemia syndrome depends on the molecular cause of severe hypertriglyceridemia.

BACKGROUND AND AIMS: Multifactorial chylomicronemia syndrome (MCS) is a severe form of hypertriglyceridemia (hyperTG) associated with an increased risk of acute pancreatitis (AP). Severe hyperTG is mainly polygenic in nature, either caused by the presence of heterozygous pathogenic variants (PVs) in TG-related metabolism genes or by accumulation of common variants in hyperTG susceptibility genes. This study aims to determine if the risk of AP is similar amongst MCS patients with different molecular causes of severe hyperTG. METHODS: This study included 114 MCS patients who underwent genetic testing for PVs in TG-related metabolism genes and 16 single nucleotide polymorphisms (SNPs) in hyperTG susceptibility genes. A weighted TG-polygenic risk score (TG-PRS) was calculated. A TG-PRS score ≥ 90th percentile was used to define a high TG-PRS. RESULTS: Overall, 66.7% of patients had severe hyperTG of polygenic origin. MCS patients with only a PV and those with both a PV and high TG-PRS were more prone to have maximal TG concentration ≥ 40 mmol/L (OR 5.33 (1.55-18.36); p = 0.008 and OR 5.33 (1.28-22.25); p = 0.02), as well as higher prevalence of AP (OR 3.64 (0.89-14.92); p = 0.07 and OR 11.90 (2.54-55.85); p = 0.002) compared to MCS patients with high TG-PRS alone. CONCLUSIONS: This is the first study to show that MCS caused by a high TG-PRS and a PV is associated with higher risk of AP, similar to what is seen in the monogenic form of severe hyperTG. This suggests that determining the molecular cause of severe hyperTG could be useful to stratify the risk of pancreatitis in MCS.

The pathogenic mutations of APOA5 in Chinese patients with hyperlipidemic acute pancreatitis.

BACKGROUND AND AIMS: To study the role of gene mutations in the development of severe hypertriglyceridemia (HTG) in patients with hyperlipidemic acute pancreatitis (HLAP), especially different apolipoprotein A5 (APOA5) mutations. METHODS: Whole-exome sequencing was performed on 163 patients with HLAP and 30 patients with biliary acute pancreatitis (BAP). The pathogenicity of mutations was then assessed by combining clinical information, predictions of bioinformatics programs, information from multiple gene databases, and residue location and conservation. The pathogenic mutations of APOA5 were visualized using the software. RESULTS: 1. Compared with BAP patients, pathogenic mutations of APOA5 were frequent in HLAP patients; among them, the heterozygous mutation of p.G185C was the most common. 2. All six pathogenic mutations of APOA5 identified in this study (p.S35N, p.D167V, p.G185C, p.K188I, p.R223C, and p.H182fs) were positively correlated with severe HTG; they were all in the important domains of apolipoprotein A-V (apoA-V). Residue 223 is strictly conserved in multiple mammals and is located in the lipoprotein lipase (LPL)-binding domain (Pro215-Phe261). When Arg 223 is mutated to Cys 223, the positive charge of this residue is reduced, which is potentially destructive to the binding function of apoA-V to LPL. 3. Four new APOA5 mutations were identified, namely c.563A > T, c.667C > T, c.788G > A, and c.544_545 insGGTGC. CONCLUSIONS: The pathogenic mutations of APOA5 were specific to the patients with HLAP and severe HTG in China, and identifying such mutations had clinical significance in elucidating the etiology and subsequent treatment.

Management of Asymptomatic Severe Hypertriglyceridemia With Oral Therapy Only: A Case Study Shedding Light on Treatment Approaches and Potential Complications.

We present a rare case of a 52-year-old male with asymptomatic severe hypertriglyceridemia exceeding 11,000 mg/dL, managed initially with oral therapy without the need for an insulin drip or plasmapheresis. However, due to non-compliance at home, the patient subsequently developed pancreatitis requiring treatment with an insulin drip. He was discharged on a regimen of fenofibrate, rosuvastatin, and omega-3, with no further episodes of symptoms. Asymptomatic patients with severe hypertriglyceridemia and a low risk of developing symptoms can be safely managed through close monitoring, statin, fibrate therapy, and lifestyle modifications, but the risk of acute pancreatitis persists with elevated triglyceride levels of over 500 mg/dL and a marked increase in risk with a triglyceride level of greater than 880 mg/dL.

Severe Hypertriglyceridemia: A 10-Year Review in a Portuguese Hospital.

INTRODUCTION: Severe hypertriglyceridemia (SHTG) is a rare condition associated with serious complications, such as acute pancreatitis (AP), and the best treatment is still a matter of discussion. The aim of this study is to outline the demographics, management, and outcomes (recurrence and mortality) of complications in patients with SHTG. MATERIAL AND METHODS: A retrospective, observational, and analytical study was carried out by obtaining clinical data from the electronic health records of patients with SHTG admitted to the Internal and Intensive Medicine units from the 1st of January 2009 to the 31st of December 2020 in a university hospital. RESULTS: The cohort included 17 patients. The most common complication was AP (13/17 = 76.5%). Admission to the intensive care unit (ICU) was observed in 84.2%. Among patients with AP, the most commonly administered therapies were insulin (82.4%) and fibrates (76.5%). Plasmapheresis was used in 58.8%, and the criteria for using this technique were mainly based on clinical and laboratory abnormalities. There were no deaths. The readmission rate at 30 days was 36.3%. CONCLUSION: This study shows the morbidity profile associated with SHTG, with a high level of ICU admissions and also a high level of the use of plasmapheresis. In our population, this approach had good results, and this should be highlighted as there are no clear international guidelines for this intervention. Distinguishing between patients with familial chylomicronemia syndrome or with multifactorial chylomicronemia is important as recent specific therapy for lipoprotein lipase (LPL) genetic deficit is available. In the near future, the performance of a genetic study should be considered in patients with SHTG as an attempt to avoid the high recurrence rate of complications of this disease.

A Case of Type V Hyperlipoproteinemia Resistant to Insulin Treatment.

Type V hyperlipoproteinemia or multifactorial chylomicronemia syndrome is a rare lipid disorder triggered mainly by uncontrolled diabetes, obesity, poor diet, or particular medications. It is associated with an increased risk of acute pancreatitis and accelerated coronary artery disease which may manifest in younger age groups. We present a case of a 42-year-old male who presented to the emergency department (ED) complaining of a non-healing hand injury. Upon laboratory workup, the patient was found to have an elevated total cholesterol (TC) of 1129 mg/dL, very low levels of high-density lipoprotein (HDL) and triglycerides (TG) > 4000 mg/dL with an inability to calculate low-density lipoprotein (LDL). Lipoprotein electrophoresis revealed an actual TG level of > 7000 mg/dL, increased chylomicrons, normal B and pre-B-lipoproteins, and increased L-lipoproteins with an elevated Apolipoprotein B. Despite these derangements, the patient did not exhibit any abdominal complaints, demonstrating a normal lipase level. The physical exam was indicative of bilateral arcus senilis and obesity. Insulin drip was initiated along with intravenous (IV) hydration and it required 12 days to bring triglycerides down to less than 1000 mg/dL. The total cholesterol was also seen to be down trending to around 500 mg/dL and the HDL improved to 22 mg/dL. We present this case as a unique presentation of asymptomatic chylomicronemia resistant to insulin treatment with an elevated ApoB but with no evidence of pancreatitis or coronary artery disease.

EFFICACY OF DOUBLE FILTRATION PLASMAPHERESIS TREATMENT IN ACUTE PANCREATITIS ASSOCIATED WITH SEVERE HYPERTRIGLYCERIDEMIA.

Background and aim: Hypertriglyceridemia is one of the leading causes of acute pancreatitis and is associated with increased morbidity and mortality. Today the recommended treatment options are fasting, hydration, if necessary antibiotics and there is not a standard recommendation to decrease triglycerides rapidly. Double Filtration Plasmapheresis (DFPP) may be an option to decrease triglycerides rapidly but its effect on the disease course is unknown. Method: In the present study, we present results of four acute pancreatitis cases associated with hypertriglyceridemia treated with DFPP. All of the patients were diagnosed as acute pancreatitis at emergency room and no complications were observed in sessions. A 76.3% reduction in triglyceride levels was observed in one or two treatment sessions. Results and conclusion: DFPP is an effective and safe option to decrease triglyceride levels rapidly but further research is needed to show the effect on mortality and morbidity.

A Case of Hypertriglyceridemia-Induced Acute Pancreatitis in the Setting of Alcohol Abuse.

Acute pancreatitis (AP) is the painful inflammation of the pancreas. It is commonly associated with gallstones, excessive alcohol use, and certain medications. We report a case of hypertriglyceridemia-induced pancreatitis in a 35-year-old African American male with a history of alcohol abuse, tobacco use, and hyperlipidemia who presented with abdominal pain and intractable vomiting. During history taking, he reported chronic alcohol abuse over the past 10 years. On physical examination, he was ill-looking, with a dry mucous membrane and reproducible epigastric tenderness. Laboratory testing indicated markedly elevated triglycerides and lipase levels. Computed Tomography imaging showed signs of pancreatic inflammation. He was treated with aggressive intravenous fluid hydration, insulin infusion, and pain control medications. He demonstrated significant improvement and then transitioned to oral fibrates. Community resources for alcohol abuse treatment were provided and a referral was made to endocrinology for outpatient follow-up. This case highlights acute pancreatitis in a person with high alcohol use with elevated triglyceride and explores possible associations between these three.

A Case of Profound Hypertriglyceridemia Causing Pseudohypobicarbonatemia.

The light-scattering effect of hypertriglyceridemia may interfere with the photometric analysis of the electrolytes, leading to errors in laboratory values. We present a case of erroneously low bicarbonate levels due to the presence of severe hypertriglyceridemia. A 49-year-old male was admitted for knee cellulitis. A comprehensive metabolic panel showed very low bicarbonate of <5 mmol/L, and an elevated anion gap of 26 mmol/L. The lactic acid, salicylic acid, ethanol, and methanol levels were normal. The lipid panel showed a remarkably high triglyceride level of 4846 mg/dL. An arterial blood gas (ABG) showed a normal pH of 7.39 and a bicarbonate level of 28 mmol/L, which was inconsistent with the metabolic acidosis seen in the blood test. The discrepancy between acidosis seen in the metabolic panel and ABG was explained by a lab error in the measured bicarbonate levels, which occurs in the presence of elevated triglyceride levels. Most laboratories use either an enzymatic/ photometric or an indirect ion-selective electrode method to measure bicarbonate. Hyperlipidemia interferes with photometric analysis due to its light-scattering effect. An ABG analyzer uses a direct ion-selective electrode method that is free of the errors of a photometric analyzer. Knowing about conditions like hypertriglyceridemia, which can interfere with the measurement of electrolytes, is important in everyday clinical medicine, as it can prevent unnecessary investigation and intervention.

Hypertriglyceridemia-Induced Acute Pancreatitis During Pregnancy: A Case Report.

Hypertriglyceridemia-induced acute pancreatitis is a rare and serious condition that places both the mother and the fetus at severe risk for morbidity and mortality. The goal of this case report is to describe the management of a pregnant patient with severely elevated triglycerides in the setting of acute pancreatitis. A 28-year-old female G2P1001 at 29 weeks of gestational age presented with epigastric abdominal pain. A computed tomography scan of the abdomen and pelvis with contrast demonstrated acute interstitial edematous pancreatitis. A lipid panel was performed, revealing a serum triglyceride level of 3,949 mg/dL. Insulin and maternal bowel rest reduced her serum triglyceride levels; however, additional medical therapy including fibrate and statin drugs were initiated to achieve goal levels of triglycerides and improve patient symptoms. The patient ultimately recovered and remained on treatment until delivery. Initial management addresses acute pancreatitis and involves fluid resuscitation, pain control, and bowel rest. Triglyceride-lowering drug therapies are rarely used during pregnancy due to the potential for fetal teratogenicity; however, given the severity of hypertriglyceridemia fenofibrate and atorvastatin were prescribed. Additional medical treatment included insulin, omega-3, and ethyl eicosapentaenoic acid.

Recurrent Pancreatitis in a Pregnant Woman with Severe Hypertriglyceridemia Successfully Managed by Multiple Plasmapheresis.

Hypertriglyceridemia (HTG) is a state of increased serum triglyceride (TG) affected by multigenetic and multifactorial causes. Serum TG concentration can be markedly elevated if exposed to precipitating factors, such as estrogen hormone and pregnancy. We report the case of a patient with severe HTG who suffered from recurrent pancreatitis during the second trimester of pregnancy conceived with in vitro fertilization-embryo transfer (IVF-ET) and was successfully controlled by multiple sessions of plasmapheresis.A 24-year-old pregnant woman was admitted because of a sudden onset of severe abdominal pain at 26 weeks of gestation conceived by IVF-ET. She has experienced recurrent pancreatitis despite low-fat diet and dyslipidemia medications allowed in pregnancy. At admission, serum amylase and lipase were elevated to 347 and 627 U/L, respectively, along with fasting TG to 4809 mg/dL. A clinical diagnosis of HTG-induced acute pancreatitis was made, and plasmapheresis was performed. After plasmapheresis, serum TG, amylase, and lipase levels decreased to 556 mg/dL, 60 U/L, and 69 U/L, respectively, along with subsequent pain relief. The patient underwent a total of nine sessions of plasmapheresis to retain serum TG lower than 1,000 mg/dL during pregnancy, with no further recurrence of acute pancreatitis. After delivery, the serum TG level was maintained below 500 mg/dL with a combination treatment of fenofibrate, statin, and ezetimibe.Although severe HTG is usually asymptomatic, if exposed to precipitating factors, it can cause acute pancreatitis, a fatal complication. Early application of plasmapheresis may be a useful option in HTG-induced acute pancreatitis intractable to medical treatment; however, its indications, risks, and benefits should be carefully evaluated.

Management of asymptomatic severe hypertriglyceridemia.

Severe hypertriglyceridemia is an urgent presentation that requires acute treatment. We present a rare case that could not be controlled by medical management and required plasmapheresis.