1/321. Primary carcinoma of the colon and hyperlipemia: a paraneoplastic syndrome. The human counterpart of the association of hyperlipidemia with cancer is rare, as compared with the relative frequency of the syndrome in experimental animals. A case is presented of adenocarcinoma of the colon with a non casual relationship between the presence and the progression of the tumor and hyperlipemia. Surgical resection and antiblastic chemotherapy moreover seemed to influence the metabolic anomaly. ( info) |
2/321. Myocardial infarction in patients with systemic lupus erythematosus with normal findings from coronary arteriography and without coronary vasculitis--case reports. The authors present the cases of two young patients, a man and a woman, who presented with myocardial infarction, in the absence of ischemic heart disease or stenosis of the coronary arteries. The woman was known to have systemic lupus erythematosus (SLE) for the past 3 years (the immunoglobulin m [IgM] anticardiolipins antibodies were positive), without a history of coronary risk factors. Suddenly she presented with acute chest pain on rest that lasted 4 hours and culminated in anterior wall myocardial infarction. She was admitted to the coronary care unit, where no thrombolysis was given. She did not have echocardiographic evidence of Libman-Sacks endocarditis, but myocardial infarction was evident at the electrocardiogram (ECG). The young man had SLE (the IgM anticardiolipins were absent, but he was positive for lupus anticoagulant antibodies), he was hyperlipidemic, was a moderate smoker and moderately obese, and had no history of ischemic heart disease. He suddenly presented with an acute myocardial infarction documented by ECG, enzymes, and gammagraphy. In both patients, coronary angiography findings were normal and myocardial biopsy did not show evidence of arteritis. The relevance of these cases is the rare association of ischemic heart disease in SLE, with normal coronary arteries and without evidence of arteritis or verrucous endocarditis. ( info) |
3/321. The evaluation of lipoprotein changes during gemfibrozil treatment. During treatment with gemfibrozil, 1200 mg daily, total serum triglyceride levels were reduced in 7 of the 11 patients, owing chiefly to a fall in VLDL triglyceride levels. Total serum cholesterol responded variably, falling in a majority of patients with hypercholesterolaemia. High density lipoprotein cholesterol rose during treatment in patients who had either hypertriglyceridaemia or hypercholesterolaemia. ( info) |
4/321. Treatment of protein-losing gastropathy with atropine. Protein loss from the gastric mucosa with hypertrophic gastric folds and hypoalbuminemia has been associated with low, normal and elevated gastric acid output. A case of protein-losing gastropathy with slightly elevated gastric acid output is described. Associated findings were hypertrophic gastric folds, hypoalbuminemia, hyperlipidemia, lymphadenopathy, edema, ascites and venous thrombosis. Oral administration of atropine resulted in a cessation of gastrointestinal protein loss and correction of hypoalbuminemia. ( info) |
5/321. Xanthoma disseminatum: a case with hepatic involvement, diabetes insipidus and type IIb hyperlipidaemia. Xanthoma disseminatum (XD) is a rare benign non-X-histiocytic disorder of unknown aetiology. We report a 37-year-old man who presented with XD preceded by a decade of cranial diabetes insipidus, with associated type IIb hyperlipidaemia and computed tomographic evidence of hepatic involvement. A review of the literature is also included. ( info) |
6/321. Intervention for hyperlipidemia associated with protease inhibitors. In the past 3 years, treatment for hiv infection has significantly improved the prognosis for hiv-infected persons. The administration of protease inhibitors for the treatment of hiv infection has had a significant role in the reduction of AIDS-related complications. Recent findings have indicated that protease inhibitors may significantly increase lipids to levels that pose a health risk that may be greater than the illness itself. This article reviews the initial findings of a study that investigated the impact of interventions for the treatment of protease inhibitor-related hyperlipidemia. The purpose of the study was to determine if initiation of interventions based on the National Cholesterol education Program Guidelines would be effective in lowering protease inhibitor-related hyperlipidemia without disrupting the effectiveness of the hiv therapy. A total of 45 hiv-infected individuals who were taking a protease inhibitor and had abnormally elevated lipids were enrolled into this study. Mean serum cholesterol level prior to initiation of a protease inhibitor regimen was 170 mg/dl as compared to a mean cholesterol at time of enrollment of 289 mg/dl and triglycerides of 879 mg/dl. Interventions included diet and exercise and the prescription of gemfibrozil alone or in combination with atorvatstatin. During the course of the study, overall intervention significantly reduced serum cholesterol level to 201 mg/dl (p. 01) over a study period of ten months. Case studies of five medical events related to hyperlipidemia are included. Currently, 26 participants continue in the study. Sixteen participants discontinued protease inhibitor therapy during the course of the study and thus ended their participation. ( info) |
7/321. Gestational hyperlipidemic pancreatitis without non-gestational hyperlipidemia. A 27 year-old pregnant woman was referred to our department with nausea, abdominal pain, and hypertriglyceridemia (5500 mg/dl). A diagnosis of acute gestational hyperlipidemic pancreatitis was made. She had no history of nongestational hyperlipidemia. Subsequently, she underwent pancreatic drainage and Caesarean section. Our experience suggests that gestational hyperlipidemic pancreatitis may occur in pregnant women without nongestational hyperlipidemia. Intensive monitoring of serum lipid levels is mandatory when managing pregnant women who develop or show gestational worsening of hypertriglyceridemia. ( info) |
8/321. Hyperlipidemia associated with protease inhibitor therapy. OBJECTIVE: To report a case of extreme hyperlipidemia associated with protease inhibitor-based antiretroviral therapy and review the relevant literature concerning lipid abnormalities with hiv infection and antiretroviral therapy. CASE SUMMARY: A 35-year-old hiv-infected man developed a serum cholesterol of 1472 mg/dL and fasting serum triglycerides of 8660 mg/dL after initiation of antiretroviral therapy consisting of ritonavir, saquinavir, nevirapine, and didanosine. All other medications had been stable during this time period and the abnormality resolved after discontinuation of antiretroviral therapy and initiation of lipid-lowering therapy. The elevated cholesterol and triglyceride concentrations did not recur when therapy was reinstituted with nelfinavir, saquinavir, nevirapine, and didanosine. The hyperlipidemia then was attributed to ritonavir. DISCUSSION: Lipid abnormalities are common in patients with hiv infection and usually consist of hypocholesterolemia and moderate hypertriglyceridemia. hypercholesterolemia and hypertriglyceridemia have been reported with ritonavir and, less commonly, with other currently available protease inhibitors. Some cases of ritonavir-associated hyperlipidemia have been extreme. Although an association between hyperlipidemia and clinical consequences such as pancreatitis and atherosclerotic disease has not been well described with protease inhibitor therapy, pancreatitis is common in hiv-infected patients. It is possible that in some cases, protease inhibitor-induced hypertriglyceridemia may contribute to the development of pancreatitis. CONCLUSIONS: Optimal management of lipid abnormalities in hiv-infected patients is controversial. The potential benefit of reducing the incidence of pancreatitis and atherosclerotic events must be weighed against the risk of intolerance, toxicity, and drug interactions. ( info) |
9/321. Identification of compound heterozygous mutations (G188E/W382X) of lipoprotein lipase gene in a Japanese infant with hyperchylomicronemia: the G188E mutation was newly identified in Japanese. We herein report a case of a 5-month-old Japanese female (patient AN) with fasting hyperchylomicronemia due to a primary lipoprotein lipase (LPL) deficiency. Patient AN was compound heterozygous for a missense mutation (GG818G-->GAG/Gly188-->Glu; G188E) in exon 5 and a nonsense mutation (TGG1401-->TGA/Trp382-->Stop; W382X) in exon 8 of the LPL gene. This resulted in less than 10% of the control levels for both the LPL activity and immunoreactive LPL mass in the postheparin plasma. A G188E mutation was thus identified for the first time in a Japanese, and the haplotype of this G188E allele was different from that of the G188E alleles identified in other ethnic groups. This additional mutation might be useful for early diagnosis of LPL gene aberrations in Japanese patients with fasting hyperchylomicronemia. ( info) |
10/321. A novel frameshift mutation in exon 6 (the site of Asn 291) of the lipoprotein lipase gene in type I hyperlipidemia. A new heterozygous lipoprotein lipase gene defect has been identified in a type I hyperlipidemic patient at the position of notable amino acid Asn 291. The patient is a 33-year-old male. His body mass index (BMI) was 18.5 kg/m2. The total cholesterol (TC), triglycerides (TG) and high density lipoprotein-cholesterol (HDL-C) concentration from his fasting plasma were 4.8, 11.9 and 0.4 mmol/l, respectively. The lipoprotein lipase (LPL) activity and mass in the postheparin plasma (PHP) from the patient were 0.58 mmol/ml/h (normal range: 7.7 /-2.6) and 244 ng/ml (normal range: 192 /-30), respectively. The hepatic lipase activity of the PHP from the patient was 10.6 mmol/ml/h (normal range: 9.9 /-3.6). dna analysis of the LPL gene revealed that this patient had a heterozygous one nucleotide deletion of A coding Asn 291, resulting in a premature termination of the LPL protein at amino acid residue 303. The other abnormality in the LPL gene of the proband was an amino acid residue 194 defect (Ile194-->Thr), which is known to cause a defective enzyme. A medium-chain triglyceride (MCT) loading test was conducted to find how this triglyceride affects plasma lipoprotein metabolism in this patient in a short term (Fig. 3). The plasma total cholesterol (TC) or high density lipoprotein (HDL)-C levels did not change significantly after oral administration of a fatty meal containing long chain triglycerides (LCT) or MCT. The plasma TG level, on the other hand, increased from 11.9 to 19.2 mmol/l ( 61%) at 6 h after loading a fatty meal containing LCT, whereas the plasma TG levels tended to even decrease at 6 h after oral administration of an MCT, tricaprin (from 11.6 to 10.5 mmol/l (-9.4%)). These results suggest that MCT, as opposed to LCT, is useful for treatment of type I hyperlipidemia with a novel mutation at the notable amino acid Asn 291 of the LPL gene. ( info) |