Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The nature of Type V hyperlipoproteinemia including mode of presentation, prominent clinical and biochemical features, and genetics, was examined in 29 adults presenting with the Type V lipoprotein phenotype. Initially 23 of the 29 patients had various metabolic stimuli (diabetes out of control, estrogenic agents, pancreatitis, ethanolism) superposed on their acute hypertriglyceridemia. After metabolic stabilization, 17 of the 29 subjects were shown to have familial hypertriglyceridemia. In the 17 kindreds with familial hypertriglyceridemia, the lack of a specific, distinctive genetic marker for the Type V genotype and for the Type IV genotype restricts the conclusion that the pattern of inheritance was consistent with an autosomal dominant trait.
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PMID:Familial and acquired type V hyperlipoproteinemia. 107 94

Women with hypertriglyceridemia are prone to gestational pancreatitis, a condition carrying substantial maternal and fetal risk. We describe a 33-year-old woman with familial hypertriglyceridemia who had recurrent hyperlipidemic abdominal crises during previous pregnancies despite dietary fat restrictions. A fifth pregnancy was carried to term without complications after aggressive dietary therapy and intermittent intravenous feeding, administered whenever her triglyceride levels exceeded an arbitrarily selected threshold concentration of 28 mmol/L. The efficacy of this approach may be explained by the published observation that low-fat (high-carbohydrate) isocaloric diets elevate serum triglyceride levels by the oral, but not the intravenous, route in normal individuals. Reduction of this patient's serum triglyceride concentrations by interrupting oral intake and administering intravenous glucose appeared to prevent late-pregnancy pancreatitis and culminated in uncomplicated full-term delivery.
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PMID:Successful hyperlipemic pregnancy. 200 35

The hyperlipoproteinemias are disturbances in the metabolism of lipoproteins. Elevated levels of total and low density lipoprotein-cholesterol, and low levels of high density lipoprotein-cholesterol are proven risk factors for atherosclerosis. The significance of hypertriglyceridemia as an independent risk factor for atherosclerosis is controversial, however, at high levels triglycerides are a major risk factor for pancreatitis. Lipoprotein abnormalities can be divided into dietary, primary (genetic), and secondary disorders. The major causes of moderate and severe hypercholesterolemia are familial hypercholesterolemia, familial combined hyperlipidemia, severe primary (polygenic) hypercholesterolemia, and familial dysbetalipoproteinemia. Causes of hypertriglyceridemia include familial hypertriglyceridemia, familial lipoprotein lipase deficiency, sporadic hypertriglyceridemia, and secondary causes.
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PMID:Hyperlipoproteinemias: Part I. Lipoprotein classification and abnormalities. 194 97

A retrospective review of 20 adult patients with splanchnic venous obstruction secondary to chronic pancreatitis was performed to identify factors important in the preoperative diagnosis and operative management of this disorder. Fifteen patients with isolated splenic vein obstruction, four with portal occlusion and one with isolated inferior mesenteric vein occlusion were studied. Chronic alcoholism was the etiology of the pancreatitis in 18 patients; two patients had a Type IV hyperlipidemia. Four patients with massive hemorrhage from gastric varices were treated with splenectomy. Most patients presented with complaints of chronic pancreatitis, requiring pancreaticojejunostomy to treat the underlying pancreatic disorder. Splenic artery inflow control was obtained preoperatively in six patients and offers a technique that is beneficial in decreasing operative blood loss. The dynamic computerized tomography (CT) scan was a valuable diagnostic tool in identifying splanchnic venous obstruction although selective angiography is required for confirmation of the diagnosis.
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PMID:Splanchnic venous obstruction. A complication of chronic pancreatitis. 291 45

Hypertriglyceridemia is a recognized complication of pregnancy. In patients with familial hypertriglyceridemia, the biochemical changes are greatly enhanced during pregnancy and may be associated with acute pancreatitis, a potentially fatal triad. Three patients were studied, in one of whom previously undiagnosed hyperlipidemia resulted in a fatal attack of fulminant acute pancreatitis. In the other two patients, this complication was avoided by close monitoring and restriction of dietary facts. A history of episodic abdominal cramps, often beginning in early childhood, or the presence of lipemic fasting plasma should alert the clinician to the presence of severe familial hypertriglyceridemia. Early diagnosis allows for the institution of relatively simple management strategies, which diminish the risk of pancreatitis.
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PMID:Hyperlipidemia, pregnancy and pancreatitis. 318 71

Interrelationships between pregnancy, hypertriglyceridemia, and pancreatitis were assessed in three women with familial hypertriglyceridemia. One subject had known familial hypertriglyceridemia, familial type V hyperlipoproteinemia, prior to conception. In this woman a progressive increase in triglyceride levels to more than 3,000 mg/dl during the first two trimesters required dietary intervention and hospitalization at 28 weeks' gestation. Use of an isocaloric National Institutes of Health type V diet reduced triglyceride levels to less than 900 mg/dl; the pregnancy was uneventful with term delivery of a healthy neonate. The familial hypertriglyceridemia was covert in the other two women until term. In one subject, subsequently shown to have familial type V, acute hemorrhagic pancreatitis with a pancreatic pseudocyst, shock, and hypocalcemia developed at 39 weeks' gestation; the neonate was safely delivered, and the mother survived. In the second, entirely asymptomatic subject, triglyceride levels greater than 5,000 mg/dl were discovered incidentally at term cesarean section during delivery of a healthy neonate. With a fat restricted diet, plasma triglyceride levels abruptly fell post partum to less than 500 mg/dl, and subsequent studies revealed familial type III hyperlipoproteinemia. Routine quantitation of plasma cholesterol and triglyceride levels or simple visual examination of fasting plasma for triglyceride-induced opacity or "milky" appearance should be done during early pregnancy. This would allow the obstetrician to identify women with severe familial hypertriglyceridemia prior to the superimposition of the physiologic hyperlipidemia of pregnancy upon familial hypertriglyceridemia with resultant, and often catastrophic, acute pancreatitis.
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PMID:Pancreatitis, familial hypertriglyceridemia, and pregnancy. 735 61

Our specific aim was to assess severe hypertriglyceridemia and pancreatitis that occurred when postmenopausal estrogen replacement therapy (ERT) or tamoxifen had been given by their physicians to women with preexisting, usually covert, primary familial hypertriglyceridemia. We retrospectively studied 31 women referred for diagnosis and therapy of hypertriglyceridemia over 2.75 years whose initial visit fasting plasma triglyceride levels were > 750 mg/dl. Of the 31 women with hypertriglyceridemia, 12 (39%) had been given exogenous estrogen by their physicians (11 ERT, one tamoxifen). Ten of the 12 women, while undergoing ERT, had triglyceride levels > 1200 mg/dl. In triglyceride referral categories 750 to 1000, 1000-1500, and > 1500 mg/dl, 17% (2 of 12), 33% (3 of 9), and 70% (7 of 10), respectively, of the 31 women with hypertriglyceridemia were receiving ERT. The higher the triglycerides were at referral, the greater was the likelihood that women were taking ERT (x2 = 6.6, p = 0.035). Four of the seven women with triglyceride levels > 1500 mg/dl while undergoing ERT were hospitalized with severe acute pancreatitis; another two had severe abdominal pain thought to be pancreatic in origin. To quickly lower dangerously high triglyceride levels, ERT was stopped in all 12 women. Lopid (1.2 to 1.5 gm/day) was given to the seven women not already taking it, and four were also given omega-3 fatty acids (4 to 15 gm/day). Median plasma triglyceride level at the initial visit in the 12 women undergoing ERT was 1665 mg/dl.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Severe hypertriglyceridemia and pancreatitis when estrogen replacement therapy is given to hypertriglyceridemic women. 828 56

Chylomicronemia syndrome (CMS) is a rare disorder characterized by the presence of chylomicrons in the fasting state causing a milky appearance of plasma, eruptive xanthomas, and hepatosplenomegaly; an acute and potentially life threatening complication is severe acute pancreatitis. The underlying defects are inborn errors of metabolism such as deficiencies of lipoprotein lipase (LPL) or apoprotein C-II (apo C-II) as well as familial hypertriglyceridemia. Moreover, CMS can be precipitated when mild hypertriglyceridemia is exacerbated by additional factors such diabetes mellitus, ethanol abuse, or pregnancy. The purpose of the present study was to retrospectively analyze the results of therapeutic plasma exchange (TPE) in 5 patients transferred to our hospital for severe acute pancreatitis due to chylomicronemia syndrome. In a total of 7 TPE sessions, on average 3,286 +/- 247 ml of plasma (i.e., about 1 patient plasma volume) were treated per session. Triglyceride (TG) levels were decreased from 4,972 +/- 2,469 mg/dl on admission to 1,614 +/- 1,276 mg/dl (-70%) after the TPE sessions, and a further decrease was achieved by conservative treatment. Part of the TG reducing effect of the treatment was probably due to heparin induced lipolysis. Acute pancreatitis was resolved in all cases, and 1 pregnant patient delivered without problems at term. In summary, 1 or 2 TPE sessions sufficed to substantially decrease the bulk of triglycerides in acutely exacerbated chylomicronemia syndrome causing a rapid resolution of acute severe pancreatitis.
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PMID:Therapeutic plasma exchange in patients with chylomicronemia syndrome complicated by acute pancreatitis. 1042 20

A 33-year-old secundipara with a history of gestational diabetes and familial hypertriglyceridemia exacerbated during her previous pregnancy was admitted in the 36th week of gestation with diffuse abdominal pain, vomiting, low-grade fever, and general malaise. A blood sample had a lipemic, milky-pink appearance and plasma concentrations were as follows: triglycerides 2173 mg/dL, cholesterol 320 mg/dL, amylase 801 U/L, lactate dehydrogenase 650 U/L, creatinine 1.5 mg/dL, glucose 380 mg/dL, and left-shifted white cells. Acute pancreatitis was diagnosed and owing to signs of fetal distress, a cesarean was performed under light general anesthesia with propofol, succinylcholine, and sevoflurane. After the umbilical cord was cut, rocoronium and fentanyl were administered. The neonate was healthy and the patient's condition evolved favorably with conservative treatment. The incidence of pancreatitis during pregnancy is low but related morbidity and mortality are high. The usual cause is biliary tract disease, although rare metabolic alterations such as hyperlipidemia may occasionally act as the trigger. Early diagnosis and treatment are the keys to successful surgery and postoperative recovery.
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PMID:[Hypertriglyceridemic pancreatitis and pregnancy]. 1475 42

Lipoprotein lipase is a key enzyme in hydrolysis of triglyceride and exchange of lipids between lipoproteins in circulation. It has also been found for lipoprotein lipase to play key roles in number of pathophysiological conditions. Over hundred different lipoprotein lipase gene variants have been described in the literature. Lipoprotein lipase gene has been observed as a key factor involved in the pathogenesis of hypertriglyceridemia, coronary heart disease and pancreatitis. In Croatian population the following gene variants have been described: -93T/G, D9N, V108V, N291S, S447X, Pvu II i Hind III. The most important finding was the first mutation W86R which caused familial hypertriglyceridemia. The investigation of mutations and polymorphisms may open the new directions for molecular diagnostics of hypertriglyceridemia and help us recognize the new mutations. Differential diagnosis of hypertriglyceridemia and medical practice involved in their prevention and treatment may be improved by knowing the frequency of lipoprotein lipase gene variants as well as their influence on lipid profile.
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PMID:[Lipoprotein lipase--physiological and pathophysiological roles of this gene variant in Croatian population]. 1748 16


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