Acute pancreatitis due to hypertriglyceridemia: report of 2 cases.

Hypertriglyceridemia (HTG) is a rare but well known cause of acute pancreatitis (AP), which can be a life- threatening complication if the degree of HTG is severe enough. It might be primary in origin or secondary to alcohol abuse, diabetes mellitus, pregnancy, or drugs. A serum triglyceride (TG) level of more than 1,000 to 2,000 mg/dL in patients with type I, IV, or V hyperlipidemia (Fredrickson's classification) is the identifiable risk factor. HTG-induced AP typically presents as an episode of AP or recurrent AP. The clinical course of HTG-induced AP is not different from other causes. Routine management of HTG-induced AP should be similar to other causes. A thorough family history of lipid abnormalities should be obtained, and an attempt to identify secondary causes should be made. The mainstay of treatment includes dietary restriction of fatty meal and lipid-lowering medications (mainly fibric acid derivatives). Although there are limited experiences with plasmapheresis, lipid apheresis, heparinization and insulin application, these can support the treatment of HTG- induced AP. We report two cases of HTG-induced AP which were successfully treated by plasmapheresis.     

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 within 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.     

Subsets associated with developing acute pancreatitis in patients with severe hypertriglyceridemia and the severity of pancreatitis

Background and objectivesHypertriglyceridemia (HTG) is a rare but well-recognized cause for acute pancreatitis (AP). This study aimed to determine subsets related to development of AP in patients with severe HTG and the severity of HTG-induced AP (HTG-AP).MethodsPatients who had severe HTG (serum triglyceride level >1,000 mg/dL) more than once between Jan. 2010 and Dec. 2017 in a single institute were evaluated retrospectively. Patients were divided into two groups, with AP or without AP, and were compared. HTG-APs in patients with severe HTG were compared to APs due to other causes during the same period.ResultsSixty-three patients (19.3%) presented with AP of a total 326 patients with severe HTG. The AP group displayed younger age, more alcohol consumption and diabetes mellitus, and higher initial/maximum serum levels of triglyceride, glucose, HbA1c, total cholesterol, and calculated non-high-density lipoprotein cholesterol (p < 0.05). HTG-APs were clinically more severe compared with 277 APs due to other causes in terms of CRP (p < 0.001), CT severity index (p = 0.002), revised Atlanta classification (p < 0.001), and hospital stay (p = 0.011). In logistic regression analysis, maximum serum triglyceride level (OR 2.706, p = 0.015), alcohol consumption amount (OR 5.292, p < 0.001), and age (OR 0.358, p = 0.017) were independently associated with development of AP in patients with severe HTG.ConclusionsDevelopment of AP in patient with severe HTG was independently associated with younger age, higher serum TG level, and more alcohol consumption. HTG-APs are clinically more severe than APs due to other causes.     

Hypertriglyceridemia-induced pancreatitis]

Hypertriglyceridemia (HTG) is a rare cause of pancreatitis. However, the relationship between acute pancreatitis and severe HTG is well recognized. We report a case of necrotizing pancreatitis due to severe HTG (type IV) in a patient with poorly controlled diabetes. It was of particular interest that serum pancreatic enzymes were normal even though the imaging studies indicated the presence of necrotizing pancreatitis. Our case clearly demonstrates the various indices of HTG-induced necrotizing pancreatitis with a normal pancreatic enzyme level despite there being a serum triglyceride level 相似文献   

Secondary Causes of Hypertriglyceridemia are Prevalent Among Patients Presenting With Hypertriglyceridemia Induced Acute Pancreatitis

BackgroundHypertriglyceridemia induced acute pancreatitis (HIAP) is the third common cause of acute pancreatitis. HIAP can result in recurrent attacks of severe AP with significant morbidity and mortality. Hypertriglyceridemia (HTG) could be primary or secondary. Although genetic causes of HTG are well studied, the prevalence of secondary causes of HTG in patients presenting with HIAP is not well characterized. This study aimed to identify the prevalence of risk factors for secondary hypertriglyceridemia among patients presenting with HIAP in a tertiary referral center in a large metropolitan area.MethodsThis is a retrospective analysis of all patients admitted with AP from August 2012–2017. A subgroup of patients with triglycerides >880 mg/dl were included for analysis. Secondary causes of HTG were identified. Secondary analysis evaluating the severity of pancreatitis was performed.ResultsThere were 3,746 patients admitted for AP of which 57 patients had AP and HTG. Of these 57 patients, 70.2% had history of diabetes mellitus, 26.3% had history of heavy alcohol use, 22.8% had chronic kidney disease, 47.3% with obesity, and 21.1% with metabolic syndrome. Two patients were classified as unexplained HTG. Secondary analysis showed a total of 45.6% of patients requiring ICU admission. 26.3% of patients with severe inflammatory pancreatitis and 17.5% of patients with severe necrotizing pancreatitis.ConclusionsIn our cohort of HIAP, 55 out of 57 patients had secondary causes for HTG. Identifying secondary causes of HTG during acute hospitalization is important to tailor outpatient treatment in order to prevent future admissions with HIAP.     

高脂血症性急性胰腺炎脂蛋白脂酶基因多态性研究

目的 探讨三酰甘油分解代谢酶脂蛋白脂酶(LPL)的表达和基因多态性与高脂血症性急性胰腺炎(HLP)的相关性.方法 2005年5月至2006年12月HLP住院患者20例,急性胰腺炎患者50例,另选取血脂正常患其他疾病者50例为对照.测定血清三酰甘油(TG)、胆固醇(Ch)、游离脂肪酸(FFA)、脂蛋白数值、血清LPL/肝脂酶(HL)活性;RT-PCR检测LPL mRNA表达;聚合酶链-限制性酶切片段多态性分析(PCR-RFLP)法分析LPL基因内含子8 Hind Ⅲ基因多态性变化.结果 HLP组血清TG、胆固醇(Ch)和FFA测定值均显著高于AP组和对照组,差异均有统计学意义(P值均<0.05);HLP组ApoE值显著高于AP组和对照组(P<0.05);HLP组高密度脂蛋白(HDL)显著低于对照组(P<0.05).HLP组LPL值为(5.98±2.28)U/L,均显著高于AP组和对照组[(1.97±0.76)U/L和(1.04±0.53)U/L,P值分别=0.046和0.031];HLP组HL值为(8.15±2.86)U/L,均显著高于AP组和对照组(1.64±0.59)U/L和(0.86±0.39)U/L,P值分别=0.002和0.001].HLP组LPL mRNA表达高于AP组,差异有统计学意义(P=0.0325).LPL基因内含子8 Hind Ⅲ分析,H2等位基因频率HLP组显著高于对照组(0.90比0.72,P<0.05);H1等位基因频率在HLP和AP组均显著低于对照组(0.10比0.28和0.14比0.28,P<0.05).HLP组H2H2基因型患者TG和Apo E值均显著高于H2H1/H1H1基因型(P值分别=0.043和0.046);AP组H2H2基因型患者TG值显著高于H2H1/H1H1基因型(P=0.032).结论 HLP患者LPL Hind Ⅲ H2等位基因频率显著高于正常人群,主要与高三酰甘油血症(HTG)相关,而与胰腺炎无关;且H2H2基因型的HLP患者血清TG、ApoE值高于其他基因型者.HTG可引起LPL基因和蛋白表达活性增高,加速TG大量分解代谢和FFA等分解产物蓄积,是诱发和加重HLP的中心环节和重要病理生理机制.     

Coagulopathy and the prognostic potential of D-dimer in hyperlipidemia-induced acute pancreatitis

BACKGROUND: Coagulopathy and its association with disease severity in hyperlipidemia(HL)- and non-hyperlipidemia(NHL)-induced acute pancreatitis(AP) are not clear. The present study was to evaluate the relationship between coagulation homeostasis and AP.METHODS: This study included 106 AP patients admitted to our hospital between October 2011 and January 2013. Stratified by disease severity,the patients were divided into two groups: a mild AP(MAP) group(n=69); and a moderately severe AP(MSAP) group(n=37). Based on disease etiology,there were 31 HL-induced AP(HLP) cases and 75 NHL-induced AP(NHLP) cases. The HLP and NHLP groups were compared for parameters of coagulation homeostasis,lipid metabolism,and disease severity. Correlations between disease severity and levels of D-dimer and protein C were investigated,and the prognostic potential of D-dimer was evaluated.RESULTS: Compared with MAP patients,MSAP patients showed higher levels of D-dimer and lower levels of protein C. HLP patients had higher protein C levels than NHLP patients. Both D-dimer and protein C levels were significantly associated with the disease severity,not the disease etiology. D-dimer levels correlated positively with low density lipoprotein cholesterol levels and performed well as a sensitive and specific predictor of disease severity in AP patients,especially in HLP patients.CONCLUSIONS: The coagulation homeostasis is different between HLP and NHLP patients,and HL may be a contributingfactor for thrombosis and fibrinolysis in HLP. D-dimer may be a robust marker of disease severity in HLP.     

Hyperlipidemia in acute pancreatitis. Relationship with etiology, onset, and severity of the disease.

Serum lipid (triglycerides and cholesterol) concentrations were studied in 49 patients with acute pancreatitis (AP). The aims of the study were to investigate the prevalence of hyperlipidemia (HL) in patients with AP according to etiology and to evaluate whether HL precedes or is a consequence of AP. Moreover, we analyzed the relationship between HL and the development of pancreatic necrosis. At admission, 23 patients (47%) had HL: 9 of 19 patients with alcoholic pancreatitis, 5 of 18 patients with biliary pancreatitis, and 9 of 12 patients with AP of miscellaneous etiologies (p less than 0.05). Severe HL (serum triglycerides greater than 20 mmol/L) was observed in five patients. Serum lipid levels in patients with AP and HL decreased markedly during the first 72 h of evolution, but remained slightly above the upper normal limit in most of them after 15 d. The prevalence of HL was similar in edematous and necrotizing pancreatitis. Necrotizing pancreatitis was significantly associated with the presence of hypertriglyceridemia in conjunction with hypercholesterolemia (p less than 0.05). The observations that a) hyperlipidemia is an early event in acute pancreatitis, (b) serum lipid values decrease during the acute phase of the disease, (c) hyperlipidemia has a different prevalence in different etiologies, and (d) high serum lipid levels are not always associated to pancreatic necrosis suggest that HL is a preexistent metabolic abnormality with respect to AP. On the other hand, HL may play a role in aggravating AP.     

Long-term follow-up of patients with acute hypertriglyceridemia-induced pancreatitis

BACKGROUND: An acute and potentially life-threatening complication of hypertriglyceridemia (HTG) is acute pancreatitis (AP). Hypertriglyceridemia, usually severe, may be primary in origin or secondary to alcohol abuse, diabetes mellitus, pregnancy, and use of drugs. STUDY: The efficacy of treatment to prevent relapses in 17 patients with AP attributed to HTG was investigated in the current prospective study. The mean follow-up period of patients was 42 months. Hypertriglyceridemia-induced AP comprised 6.9% of all patients with AP (n = 246) hospitalized in our clinic during the study (6 years). RESULTS: Causative conditions of HTG-induced AP were familial HTG in eight patients, HTG caused by uncontrolled diabetes mellitus in five, HTG aggravated by drugs in two (one by tamoxifen and one by fluvastatin), familial hyperchylomicronemia (HCM) in one, and lipemia of pregnancy in one. During the acute phase of pancreatitis, patients underwent standard treatment. Thereafter, HTG was efficiently controlled with high dosages of fibrates or a fibrate plus acipimox, except for the patient with HCM, who was on a specific diet (the only source of fat was a special oil consisting of medium chain triglyceride) and taking a high dosage of acipimox. One of the patients died during the acute phase of pancreatitis with acute respiratory distress syndrome. During follow-up, maintenance treatment was successful and only one patient relapsed, because he discontinued diet and drug treatment. CONCLUSION: Appropriate diet and drug treatment, including dose titration, of severe HTG is very effective in preventing relapses of HTG-induced AP.     

Hyperlipidemia intensifies cerulein-induced acute pancreatitis associated with activation of protein kinase C in rats

AIM: To investigate the effects of hyperlipidemia on acute pancreatitis (AP) and the possible mechanisms. METHODS: Rat models of hyperlipidemia and AP were established by Triton WR1339 and cerulein respectively. Human albumin was used to treat AP complicated by hyperlipidemia. In each group, we compared the histological score, volume of ascites, ratio of pancreatic wet/dry weight, serum amylase (AMY) and pancreatic acinar cell apoptosis. The level of protein kinase C (PKC) membrane translocation in pancreatic tissue was detected by Western blot. RESULTS: In the hyperlipidemia model established by Triton WR1339, triglyceride (TG) increased remarkably and reached its peak 6 h after injection, and most rats developed mild acute pancreatitis. Histological score, volume of ascites, ratio of wet/dry weight and serum AMY in AP animals with hyperlipidemia were obviously higher than those in AP animals (P < 0.05) and decreased after albumin therapy but not significantly (P > 0.05). Apoptotic cells detected by terminal deoxynucleotidyl transferase-mediated dUTPbiotin nick end labeling (TUNEL) increased in AP animals with hyperlipidemia and did not change distinctly after albumin therapy. PKC membrane translocation level increased in AP animals with hyperlipidemia and decreased remarkably after albumin therapy (P < 0.05). CONCLUSION: Hyperlipidemia may induce AP or intensify pancreatic injury. Albumin therapy can not alleviate pancreatic lesion effectively. PKC activation may be one mechanism by which AP is intensified by hyperlipidemia.     

高甘油三酯血症对大鼠急性胰腺炎模型中胰腺导管上皮细胞间紧密连接的影响研究

目的研究高甘油三酯血症(hypertriglyceridemia,HTG)对大鼠急性胰腺炎(acute pancreatitis,AP)模型胰腺组织细胞间紧密连接(tight junctions,TJs)的影响。方法48只4周龄雄性SD大鼠随机分成普通饲养组(24只)和高脂饲养组(24只),4周后普通饲养组随机分为C组和AP组,高脂饲养组随机分为HTG组和高甘油三酯血症急性胰腺炎(HTGP)组。AP组和HTGP组经腹腔注射雨蛙肽建立AP模型,C组和HTG组经腹腔注射同等剂量的生理盐水,分别于造模24 h、48 h处置大鼠,采用HE染色法观察胰腺病理改变,免疫组织化学法检测胰腺组织中TJs蛋白脂解刺激脂蛋白受体(LSR)、Tricellulin蛋白(TRIC)、ZO-1、occludin、claudin-7定位及表达,透射电镜观察胰腺导管上皮细胞间TJs变化情况。结果高脂饲养组的大鼠血清甘油三酯(triglyceride,TG)较普通饲养组明显升高(P<0.05);HTG组胰腺组织病理评分高于C组(P<0.05),相同时点HTGP组胰腺组织病理评分高于AP组,但差异无统计学意义(P>0.05);HTG组LSR、TRIC表达显著低于C组(P<0.05),AP组和HTGP组的24 h时点LSR、TRIC、occludin、claudin-7表达均低于相应对照组(P<0.05),HTGP组24 h时点LSR和TRIC的表达显著低于AP组的对应时点(P<0.05);透射电镜观察AP组和HTGP组的24 h时点,发现主胰管上皮细胞TJs较C组和HTG组减少、细胞间隙增宽,且HTGP组比AP组的细胞间隙增宽更明显。结论TJs蛋白LSR、TRIC、ZO-1、occludin、claudin-7在HTG及HTGP大鼠胰腺组织中表达较非高脂模型下降,其中以三细胞间紧密连接(tricellular tight junctions,tTJs)蛋白LSR及TRIC下降明显,提示HTG可能减弱胰腺组织的tTJs,进而加重胰腺组织损伤。     

Current knowledge of hypertriglyceridemic pancreatitis

Severe hypertriglyceridemia (HTG) is a well established and the most common cause of acute pancreatitis (AP) after alcohol and gall stone disease. It is alleged to account for up to 10% of all pancreatitis episodes. Studies suggest that in patients with triglyceride (TG) levels > 1000 mg/dL (> 11.3 mmol/L), hypertriglyceridemia-induced acute pancreatitis (HTGP-AP) occurs in approximately 15–20% of all subjects referred to Lipid Clinics. Until now, there is no clear evidence which patients with severe HTG will develop pancreatitis and which will not. Underlying pathophysiological concepts include hydrolysis of TG by pancreatic lipase and excessive formation of free fatty acids with inflammatory changes and capillary injury. Additionally hyperviscosity and ischemia may play a decisive role. The clinical features of HTG-AP patients are supposed to be no different from patients with AP of other etiologies. Yet, there are well-conducted studies suggesting that HTG-AP is associated with a higher severity and complication rate. Therapeutic measurements in HTG-AP include dietary modifications, different antihyperlipidemic agents, insulin and/or heparin treatment. The beneficial use of plasmapheresis is repeatedly reported and suggested in many studies. Yet, due to the lack of randomized and controlled trials, it is currently unknown if plasmapheresis may improve morbidity and mortality in the clinical setting of HTG-AP. Since there are no commonly accepted clinical guidelines in the management of HTG-AP, there is a definite need for an international, multicenter approach to this important subject.     

The role of diet and drugs in lowering serum cholesterol in the postmyocardial infarction patient

Summary Lipid disorders are the most important factors in the development of coronary heart disease (CHD). They need to be treated in primary as well as in secondary prevention. U.S. and European Consensus Conferences a greed that desirable serum cholesterol levels should not exceed 200 mg/dL. When baseline cholesterol averages above 250 mg/dL, the minimum requirement for characterizing the lipoprotein disorder is measurement of cholesterol, triglycerides, and high-density lipoprotein (HDL) in the fasting state. In selecting targets for serum lipid values, it may be taken into account that CHD incidence is lowest in persons with serum cholesterol below 180 mg/dL. Any kind of lipid-lowering therapy should be commenced with dietary treatment. If this is ineffective, drugs may be applied additionally. Possible causes of secondary hyperlipidemia should be excluded. There is no strict age limit for treatment but the subject's cardiovascular status should be examined carefully, especially in secondary prevention. The patient in whom extensive myocardial damage is the main arbiter of prognosis is unlikely to gain from strenuous efforts aimed at retarding progression of atheromata, the major causes of CHD. A simple classification distinguishes drugs with a predominant effect on hypercholesterolemia from those effective in endogenous hypertriglyceridemia but with a somewhat weaker cholesterol-lowering action. Using lipid-lowering drugs, their indications and side effects should be considered.     

Hyperlipidemia in acute pancreatitis

Whether hyperlipidemia is a pre-existing metabolic disorder or a consequence of acute pancreatitis is still debated. Mild to moderate elevation of serum triglyceride levels are likely to be an epiphenomenon of the pancreatic disease. A marked hyperchylomicronemia and hypertrygliceridemia would be needed to trigger acute pancreatitis; a relevant defect in the lipid catabolism and clearance should therefore pre-exist. The aim of the present study was to investigate whether patients with acute pancreatitis and marked hyperlipidemia have an impaired clearance capacity of exogenous lipids, which would define the hyperlipidemia as a preexistent abnormality and therefore a potential cause of the pancreatic disease. With this aim, the kinetics of the removal of exogenous triglycerides from the circulation have been analyzed. Twenty patients with acute pancreatitis have been studied. Ten of them suffered from an episode of acute pancreatitis with marked hyperlipidemia (serum triglyceride levels>20mmol/L). Four to six months after recovery from the pancreatitis, a two-stage infusion of Intralipid 20% was carried out and the fractional removal rate (K₂) and the maximal clearance capacity (K₁) of exogenous triglycerides were calculated. At low infusion rates a first order kinetics for removal was observed, whereas at high infusion rates a zero order kinetics was operating. All patients with a previous attack of normolipidemic acute pancreatitis had normal K₂ and K₁ values. Five patients with previous hyperlipidemic acute pancreatitis had an abnormally low clearance capacity of exogenous triglycerides, whereas the remaining five had normal removal values. The present study provides new information in the association between hyperlipidemia and acute pancreatitis by showing that even a marked elevation of serum lipid levels should not be invariably considered as the etiological factor of the pancreatic disease, even if other potential causes are not evident.     

Hypertriglyceridemia-induced recurrent acute pancreatitis: A case-based review

Hypertriglyceridemia is a rare, but well-known cause of acute pancreatitis. A serum triglyceride level of more than 1000 to 2000 mg / dl is the identifiable risk factor. It typically presents as an episode of acute pancreatitis or recurrent acute pancreatitis. The clinical course and routine management of Hypertriglyceridemia-induced pancreatitis is similar to other causes. A thorough family history is important, as is the identification of secondary causes of hypertriglyceridemia. The mainstay of therapy includes dietary restriction of fatty meal and fibric acid derivatives. We hereby report the case of a 37-year-old lady with a family history of dyslipidemia presenting with recurrent episodes of acute pancreatitis. We also review the literature for pathogenesis and management of hyperlipidemia.     

脑梗死并高脂血症患者血浆P-选择素水平的变化及降脂干预

为研究动脉粥样硬化性脑梗死患者动脉粥样硬化病变的炎症反应以及降脂干预对其产生的影响 ,以酶标多克隆抗体夹心法及硝酸还原酶比色法测定 36例健康对照组、30例急性脑梗死并高胆固醇血症组及 2 8例急性脑梗死并高甘油三酯血症组患者应用辛伐他汀治疗前后血浆P 选择素、氧化型低密度脂蛋白和血清一氧化氮的水平。结果发现 ,两组脑梗死患者血浆P 选择素和氧化型低密度脂蛋白水平较对照组明显增高 ,血清一氧化氮水平明显减低。急性脑梗死并高胆固醇血症组患者的血浆P 选择素水平与血浆氧化型低密度脂蛋白和低密度脂蛋白水平呈显著正相关 ,与血清一氧化氮水平呈显著负相关 ;急性脑梗死并高甘油三酯血症组患者的血浆P 选择素水平与氧化型低密度脂蛋白和甘油三酯水平呈显著正相关。辛伐他汀治疗 6周后 ,两组脑梗死患者的血浆P 选择素和氧化型低密度脂蛋白水平明显减低 ,血清一氧化氮水平明显回升。以上提示 ,急性脑梗死并高胆固醇血症和高甘油三酯血症患者均存在着动脉粥样硬化病变的炎症反应 ,其炎症反应的发展与血浆P 选择素水平的变化及血浆氧化型低密度脂蛋白、低密度脂蛋白、甘油三酯和血清一氧化氮水平的变化相一致 ,降脂干预可阻止动脉粥样硬化病变炎症反应的发展     

Hypertriglyceridaemia-associated acute pancreatitis: diagnosis and impact on severity

BackgroundThe level of hypertriglyceridaemia (HTG) at which the risk of acute pancreatitis (AP) increases and the impact of HTG on AP attributable to other aetiologies remains unclear.MethodsWe compared clinical outcomes of patients admitted within 48 h of the onset of abdominal pain from a first episode of AP and admission serum triglyceride levels of either <5.65 mmol/l (<500 mg/dl) or ≥5.65 to <11.3 mmol/l (moderate HTG) or ≥11.3 mmol/l (≥1000 mg/dl, severe HTG).ResultsAmong a cohort of 1,233 patients with AP there were significant progressive increases in all major deleterious clinical outcomes including mortality (all P_trend < 0.05) that were directly dependent on admission triglyceride levels. Outcomes were improved by earlier presentation (<24 h compared to 24–48 h from abdominal pain onset). Patients with severe HTG and a concomitant aetiology (n = 68) had significantly more persistent organ failure, pancreatic necrosis and longer hospital stays (P < 0.05) than those with severe HTG alone (n = 206).ConclusionsThere appears to be an association between HTG grade and the severity of AP. Severe HTG significantly increased the severity of AP, over AP attributable to other aetiologies. Moderate as well as severe HTG can be used as a criterion for the diagnosis of HTG-associated AP.     

A rare case of diabetic ketoacidosis presenting with severe hypertriglyceridemia requiring plasmapheresis in an adult with type-2 diabetes mellitus: Case report

Introduction:Severe hypertriglyceridemia (HTG) is a rare complication of insulin resistance. Its presentation with diabetic ketoacidosis (DKA) has been reported in a few cases, where most patients have type-1 diabetes mellitus (DM). Our case represents a unique presentation of DKA associated with severe HTG above 10,000 mg/dL in an adult with type-2 DM.Patient concerns and diagnosis:Case Report: A 51-year-old man with no prior illnesses presented to the emergency department with abdominal pain and nausea. He was found to have DKA with a blood glucose level of 337 mg/dL, pH of 7.17, beta-hydroxybutyrate of 7.93 mmol/L, and anion gap of 20 mmol/L. His triglyceride levels were >10,000 mg/dL. His serum was found to be lipemic. Computerized tomography scan of the abdomen demonstrated mild acute pancreatitis. Negative GAD65 antibodies supported the diagnosis of type-2 DM.Interventions and outcomes:Endocrinology was consulted and one cycle of albumin-bound plasmapheresis was administered. This therapy significantly improved his HTG. DKA gradually resolved with insulin therapy as well. He was discharged home with endocrinology follow-up.Conclusion:This unique case highlights an uncommon but critical consequence of uncontrolled DM. It brings forth the possibility of severe HTG presenting as a complication of uncontrolled type-2 DM. Severe HTG commonly presents with acute pancreatitis, which can be debilitating if not managed promptly. Most patients with this presentation are managed with insulin infusion. The use of plasmapheresis for management of severe HTG has not been well studied. Our case supports the use of plasmapheresis as an effective and rapid treatment for severe HTG.     

Current Diagnosis and Management of Primary Chylomicronemia

Primary chylomicronemia (PCM) is a rare and intractable disease characterized by marked accumulation of chylomicrons in plasma. The levels of plasma triglycerides (TGs) typically range from 1,000 - 15,000 mg/dL or higher. PCM is caused by defects in the lipoprotein lipase (LPL) pathway due to genetic mutations, autoantibodies, or unidentified causes. The monogenic type is typically inherited as an autosomal recessive trait with loss-of-function mutations in LPL pathway genes ( LPL , LMF1 , GPIHBP1 , APOC2 , and APOA5 ). Secondary/environmental factors (diabetes, alcohol intake, pregnancy, etc.) often exacerbate hypertriglyceridemia (HTG). The signs, symptoms, and complications of chylomicronemia include eruptive xanthomas, lipemia retinalis, hepatosplenomegaly, and acute pancreatitis with onset as early as in infancy. Acute pancreatitis can be fatal and recurrent episodes of abdominal pain may lead to dietary fat intolerance and failure to thrive.The main goal of treatment is to prevent acute pancreatitis by reducing plasma TG levels to at least less than 500-1,000 mg/dL. However, current TG-lowering medications are generally ineffective for PCM. The only other treatment options are modulation of secondary/environmental factors. Most patients need strict dietary fat restriction, which is often difficult to maintain and likely affects their quality of life.Timely diagnosis is critical for the best prognosis with currently available management, but PCM is often misdiagnosed and undertreated. The aim of this review is firstly to summarize the pathogenesis, signs, symptoms, diagnosis, and management of PCM, and secondly to propose simple diagnostic criteria that can be readily translated into general clinical practice to improve the diagnostic rate of PCM. In fact, these criteria are currently used to define eligibility to receive social support from the Japanese government for PCM as a rare and intractable disease.Nevertheless, further research to unravel the molecular pathogenesis and develop effective therapeutic modalities is warranted. Nationwide registry research on PCM is currently ongoing in Japan with the aim of better understanding the disease burden as well as the unmet needs of this life-threatening disease with poor therapeutic options.     

高三酰甘油血症急性胰腺炎的特点与处理

胆结石和高三酰甘油是中国急性胰腺炎最常见的两大病因,高三酰甘油血症急性胰腺炎(HTGP)患者较年轻、男性较多、更易合并2型糖尿病、肥胖等,且并发症发生率更高、病情更重.HTGP最初的支持治疗与其他病因导致的急性胰腺炎类似,另外还采用降低血清三酰甘油水平的特殊治疗,包括肝素、胰岛素的输注、血浆置换、血液滤过等,后期生活方...