Hyperlipidemic Pancreatitis Research Articles

Association of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutation/Variant/Haplotype and Tumor Necrosis Factor (TNF) Promoter Polymorphism in Hyperlipidemic Pancreatitis

The mechanism by which hypertriglyceridemia (HTG) leads to pancreatitis is not clear. We sought to determine whether the genes involved in pancreatic ductal or acinar cell injury, including the cationic trypsinogen gene [protease, serine, 1 (trypsin 1) (PRSS1)], the pancreatic secretory trypsin inhibitor gene [serine peptidase inhibitor, Kazal type 1 (SPINK1)], the cystic fibrosis transmembrane conductance regulator gene [cystic fibrosis transmembrane conductance regulator (ATP-binding cassette subfamily C, member 7) (CFTR)], and inflammation genes such as tumor necrosis factor [tumor necrosis factor, TNF superfamily, member 2 (TNF)] are associated with hyperlipidemic pancreatitis (HLP) in patients with HTG. We performed genetic analysis of 126 HTG patients in Taiwan (46 with HLP and 80 without HLP). The entire coding and intronic regions of the PRSS1, SPINK1, and CFTR genes were identified by heteroduplex analysis techniques and were confirmed by sequencing analysis. The presence of 125G/C, 1001 + 11C>T, 1540A>G (Met470Val), 2694T>G, and 4521G>A in CFTR, the presence of 272C>T in SPINK1, and TNF promoter polymorphisms (nucleotide positions 1031, 863, 857, 308, and 308) were measured by direct sequencing. Of the 126 HTG patients, 13 (10.3%) carried a CFTR mutation. No PRSS1 or SPINK1 mutations were detected in our patients or in HTG controls. The CFTR gene mutation rates in HTG with and without HLP were 26.1% (12 of 46) and 1.3% (1 of 80), respectively (P <0.0001). The CFTR gene mutations were all Ile556Val. A multivariate analysis of HTG patients indicated that triglycerides, CFTR 470Val, and TNF promoter 863A were independent risk markers for HLP. This genetic study is the first one to address the association of HLP with the CFTR mutation/variant/haplotype and TNF promoter polymorphism in a Chinese HTG population. The results suggest that the occurrence of HLP is multifactorial and polygenic.

Advances in pathogenesis of hyperlipidemic acute pancreatitis and its diagnosis and treatment

Advances in pathogenesis of hyperlipidemic acute pancreatitis and its diagnosis and treatment

Double filtration plasmapheresis in treatment of hyperlipidemic acute pancreatitis

To investigate the therapeutic effects of double filtration plasmapheresis(DFPP) in treatment of hyperlipidemic acute pancreatitis. Nine patients with acute hyperlipidemic pancreatitis were treated with DFPP in addition to the conventional therapeutic measures. The clinical symptoms,serum levels of triglyceride (TG) and APACHE II scores were observed before and after DFPP. After DFPP the clinical symptoms of patients were improved greatly; serum levels of TG decreased from (83.48 +/-2.54)mmol/L to (4.09 +/-0.65)mmol/L(P<0.01) and APACHE II scores decreased from 12.2 +/- 2.3 to 6.2 +/- 1.3(P <0.05). There were no significant side effects during and after DFPP. DFPP can be effectively and safely applied in patients with acute hyperlipidemic pancreatitis.

Hemorheological abnormalities in lipoprotein lipase deficient mice with severe hypertriglyceridemia

Severe hypertriglyceridemia (HTG) is a metabolic disturbance often seen in clinical practice. It is known to induce life-threatening acute pancreatitis, but its role in atherogenesis remains elusive. Hemorheological abnormality was thought to play an important role in pathogenesis of both pancreatitis and atherosclerosis. However, hemorheology in severe HTG was not well investigated. Recently, we established a severe HTG mouse model deficient in lipoprotein lipase (LPL) in which severe HTG was observed to cause a significant increase in plasma viscosity. Disturbances of erythrocytes were also documented, including decreased deformability, electrophoresis rate, and membrane fluidity, and increased osmotic fragility. Scanning electron microscopy demonstrated that most erythrocytes of LPL deficient mice deformed with protrusions, irregular appearances or indistinct concaves. Analysis of erythrocyte membrane lipids showed decreased cholesterol (Ch) and phospholipid (PL) contents but unaltered Ch/PL ratio. The changes of membrane lipids may be partially responsible for the hemorheological and morphologic abnormalities of erythrocytes. This study indicated that severe HTG could lead to significant impairment of hemorheology and this model may be useful in delineating the role of severe HTG in the pathogenesis of hyperlipidemic pancreatitis and atherosclerosis.

Recurrent Acute Pancreatitis in a Patient with Type IIb hyperlipoproteinemia: A Case Report and Review of the Literature in Korea

Hyperlipidemia is a rare cause of pancreatitis. It has been believed that free fatty acids released from hydrolyzed serum chylomicrons or triglycerides and chylomicrons induce hyperlipidemic pancreatitis by damaging acinar cells and capillaries. Type I, IV or V hyperlipidemic (Fredrickson's classification) pancreatitides have distinctive features of increased and heightened serum chylomicron and triglyceride levels. In contrast, type IIb hyperlipidemia usually doesn't have increased chylomicrons. It is a dominant inherited genetic disorder and doesn't manifest the subjective symptom before combining vascular complications such as coronary artery disease. Only a few cases of type IIb hyperlipidemic pancreatitis have been reported. We experienced a male patient with recurrent hyperlipidemic pancreatitis combined with type IIb hyperlipidemia. We present the case report and a review of the literature of hyperlipidemic pancreatitis, especially cases in Korea.

Recurrent acute pancreatitis and its relative factors

To evaluate the causes and the relative factors of recurrent acute pancreatitis. From 1997 to 2000, acute pancreatitis relapsed in 77 of 245 acute pancreatitis patients. By reviewing the clinical treatment results and the follow-up data, we analyzed the recurrent factors of acute pancreatitis using univariate analysis and multivariate analysis. Of the 245 acute pancreatitis patients, 77 were patients with recurrent acute pancreatitis. Of them, 56 patients relapsed two times, 19 relapsed three times, each patient relapsed three and four times. Forty-seven patients relapsed in hospital and the other 30 patients relapsed after discharge. Eighteen patients relapsed in 1 year, eight relapsed in 1-3 years, and four relapsed after 3 years. There were 48 cases of biliary pancreatitis, 3 of alcohol pancreatitis, 5 of hyperlipidemia pancreatitis, 21 of idiopathic pancreatitis. Univariate analysis showed that the patients with local complications of pancreas, obstructive jaundice and hepatic function injury were easy to recur during the treatment period of acute pancreatitis (P=0.022<0.05, P=0.012<0.05 and P=0.002<0.05, respectively). Multivariate analysis showed that there was no single factor related to recurrence. Of the 47 patients who had recurrence in hospital, 16 had recurrence in a fast period, 31 after refeeding. Acute pancreatitis is easy to recur even during treatment. The factors such as changes of pancreas structure and uncontrolled systemic inflammatory reaction are responsible for the recurrence of acute pancreatitis. Early refeeding increases the recurrence of acute pancreatitis. Defining the etiology is essential for reducing the recurrence of acute pancreatitis.

Acute pancreatitis in HIV-infected patients: are etiologies changing since the introduction of protease inhibitor therapy?

Hypertriglyceridemia is a well-established cause of acute pancreatitis in the general population. Protease inhibitor (PI) therapy, introduced in 1996 for HIV infection, is associated with moderate to severe hypertriglyceridemia. To determine whether the prevalence of hyperlipidemic pancreatitis in HIV-infected patients has increased since the introduction of PIs. This was a retrospective study of patients with acute pancreatitis and HIV infection admitted to three local hospitals between 1990 and 2001. Before PIs became available (1990-1995), 30 index cases of acute pancreatitis in the setting of HIV infection were identified, and one of these cases (3.3%) was attributed to hypertriglyceridemia. After the introduction of PIs (1996-2001), 54 cases of acute pancreatitis in HIV-infected patients were identified, and two of these cases were attributed to hypertriglyceridemia (3.7%; p = 0.6). In both time periods, medication-induced pancreatitis was the most common cause of pancreatitis in HIV-infected patients. Despite the well-established association between PIs and hypertriglyceridemia, there was no significant increase in the prevalence of hyperlipidemic pancreatitis in this HIV-infected population after the introduction of PIs. Medication-associated pancreatitis remains the most common cause of acute pancreatitis in the era of potent antiretroviral therapy.

Hypertriglyceridemia and Hypercholesterolemia Induced by L-Asparaginase

Hypertriglyceridemia and Hypercholesterolemia Induced by L-Asparaginase

Issues in hyperlipidemic pancreatitis.

Hypertriglyceridemia (HTG) is a rare cause of pancreatitis. Pancreatitis secondary to HTG, presents typically as an episode of acute pancreatitis (AP) or recurrent AP, rarely as chronic pancreatitis. 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 an identifiable risk factor. The typical clinical profile of hyperlipidemic pancreatitis (HLP) is a patient with a preexisting lipid abnormality along with the presence of a secondary factor (e.g., poorly controlled diabetes, alcohol use, or a medication) that can induce HTG. Less commonly, a patient with isolated hyperlipidemia (type V or I) without a precipitating factor presents with pancreatitis. Interestingly, serum pancreatic enzymes may be normal or only minimally elevated, even in the presence of severe pancreatitis diagnosed by imaging studies. The clinical course in HLP is not different from that of pancreatitis of other causes. Routine management of AP caused by hyperlipidemia should be similar to that of other causes. A thorough family history of lipid abnormalities should be obtained, and an attempt to identify secondary causes should be made. Reduction of TG levels to well below 1,000 mg/dL effectively prevents further episodes of pancreatitis. The mainstay of treatment includes dietary restriction of fat and lipid-lowering medications (mainly fibric acid derivatives). Experiences with plasmapheresis, lipid pheresis, and extracorporeal lipid elimination are limited.

Hyperlipidemic pancreatitis during pregnancy

Hyperlipidemic pancreatitis during pregnancy

Hyperlipidemic pancreatitis: the qatar experience

Abstract Hyperlipidemic pancreatitis represents one of the most acute and dramatic consequences of the chylomicronemia syndrome which, if misdiagnosed, can lead to pancreatic destruction and death. A retrospective analysis of 16 cases admitted to Hamad General Hospital since 1990 investigated the relationship between hyperlipidemia and pancreatitis. All the patients had discrete, recurrent episodes of acute pancreatitis with typical pain, hyperamylasemia and hypertriglyceridemia. Acute pancreatitis usually occurred when the serum triglyceride levels were above 10 mmol/L. Chylomicronemia, with the serum being visibly creamy, was a constant feature of the lipoprotein pattern of the patients during these acute episodes. It seems that chylomicronemia is the most consistent feature of hyperlipidemic pancreatitis. Most patients respond well to a low fat diet but lipid lowering agents are generally required to prevent recurrence.

F299. Rheological changes in hypertensive patients treated with ramipril

Severe hypertriglyceridemia (HTG) is a metabolic disturbance often seen in clinical practice. It is known to induce life-threatening acute pancreatitis, but its role in atherogenesis remains elusive. Hemorheological abnormality was thought to play an important role in pathogenesis of both pancreatitis and atherosclerosis. However, hemorheology in severe HTG was not well investigated. Recently, we established a severe HTG mouse model deficient in lipoprotein lipase (LPL) in which severe HTG was observed to cause a significant increase in plasma viscosity. Disturbances of erythrocytes were also documented, including decreased deformability, electrophoresis rate, and membrane fluidity, and increased osmotic fragility. Scanning electron microscopy demonstrated that most erythrocytes of LPL deficient mice deformed with protrusions, irregular appearances or indistinct concaves. Analysis of erythrocyte membrane lipids showed decreased cholesterol (Ch) and phospholipid (PL) contents but unaltered Ch/PL ratio. The changes of membrane lipids may be partially responsible for the hemorheological and morphologic abnormalities of erythrocytes. This study indicated that severe HTG could lead to significant impairment of hemorheology and this model may be useful in delineating the role of severe HTG in the pathogenesis of hyperlipidemic pancreatitis and atherosclerosis.

Extracorporeal lipid elimination for treatment of gestational hyperlipidemic pancreatitis

Gestational hyperlipidemia complicated by pancreatitis during the 24th week of gestation has been successfully managed by long-term extracorporeal elimination of triglyceride-rich lipoproteins. Three modes of treatment (plasma exchange, immunospecific apheresis, and a combination of both treatments) were compared for efficacy as therapy for metabolic derangements, altered blood rheology, and the loss of immunoglobulins. Treatments were performed by means of a peripheral venovenous approach. A combination plasma exchange/apheresis technique was highly effective; the loss of immunoglobulins remained acceptable. Clinical symptoms of pancreatitis subsided within 24 hours of the first treatment. A relapse during the 32nd week of gestation necessitated treatments more frequently than once a week. At the 36th week of gestation, after confirming lung maturity as indicated by a lecithin-sphingomyelin ratio of > 2.0, a cesarian section was performed. A healthy boy was delivered (2470 g; Apgar score, 9/10). This is the first report to show that long-term extracorporeal elimination of lipoproteins is a highly effective treatment of hyperlipidemic gestational pancreatitis.

Hyperlipidemic Pancreatitis in Pregnancy: A Case Report and Review of the Literature

A case of pancreatitis associated with type I primary hyperlipoproteinemia in pregnancy is reported, with a review of the literature. There have been 11 cases of hyperlipidemic pancreatitis during pregnancy reported in the English literature since 1956. This case is the first report of adult respiratory distress syndrome complicating hyperlipidemic pancreatitis in pregnancy. Guidelines for prevention and management of this rare disorder are presented.

Treatment of Hyperlipidemic Pancreatitis in Pregnancy With Total Parenteral Nutrition

A 29-yr-old pregnant woman presented in her third trimester with severe pancreatitis, hyperlipidemia, and small-for-dates fetus. Studies suggested that her pancreatitis was caused by profound hypertriglyceridemia, which was the result of an underlying lipoprotein disorder exacerbated by pregnancy. Throughout the first 7 wk of hospitalization, attempts to refeed the patient with solid food and various elemental diets resulted in the induction of hypertriglyceridemia and relapses of pancreatitis. Concern for the nutritional status of the mother and the possibility of further growth retardation of the fetus prompted the use of total parenteral nutrition for the last 2 wk of gestation. This treatment was well tolerated by the mother, promptly reversed maternal weight loss, caused a rapid resolution of her pancreatitis, and did not induce further hypertriglyceridemia. This report documents that total parenteral nutrition may be safely and effectively used in the management of hyperlipidemic pancreatitis in pregnancy.