Cases reported "Hyperlipoproteinemias"

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1/84. The use of antioxidants in retarding atherosclerosis: fact or fiction?

    The proposal that antioxidants may retard the progression of atherosclerosis is not new. Published studies examining the effect of antioxidants on experimental antioxidants extend back to 1940. The results have all been inconsistent. However, the data regarding the beneficial effects of retarding atherosclerotic progression are strong enough to warrant continued research on the lipoprotein oxidation theory or atherosclerosis. However, caution is needed to avoid embracing a concept without proof. It should be noted that the National Cholesterol education Program does not recommend the use of antioxidant vitamin supplements to reduce CAD. Atherogenesis is produced by multiple factors. To believe that all such factors are mediated by uncontrolled oxidative events is, to say the least, naive. Finally, should antioxidants prove to be effective in retarding coronary atherosclerosis, their place on the therapeutic ladder of CAD prevention would be low. The overwhelmingly proven evidence favors the following factors that have been proven to lower morbidity and mortality due to atherosclerosis: (a) treatment of hypertension, (b) cessation of tobacco use, (c) treatment of dyslipidemia, (d) achieving a normal weight, (e) regular exercise, (f) treatment of homocystinuria, especially in cases with renal disease, and (g) antioxidants.
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ranking = 1
keywords = lipoprotein
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2/84. Lipoprotein (a) immunapheresis in the treatment of familial lipoprotein (a) hyperlipoproteinemia in a patient with coronary heart disease.

    This paper reports 2 years' experience with lipoprotein (a) (Lp[a]) immunapheresis which was successfully handled on a now 40-year-old patient with familial Lp(a) hyperlipoproteinemia inducing severe coronary heart disease with 2 myocardial infarctions and diffuse coronary sclerosis. Continued treatment by Lp(a) immunabsorption with specific sheep antibodies reduced stenosis in coronary vessels more than 50% and stopped the progression of coronary heart disease. A special apheresis technique and the results of continued absorption effects are described.
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ranking = 10
keywords = lipoprotein
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3/84. DALI LDL-apheresis: anticoagulation with r-hirudin in a patient with heparin-induced thrombocytopenia (HIT II).

    A 50-year old male patient with familial hypercholesterolemia and hyperlipoproteinemia (a), who underwent low density lipoprotein-apheresis treatment developed heparin-induced thrombocytopenia type II (HIT II). Because heparin is contraindicated in patients with HIT, an alternative LDL-apheresis system and modified anticoagulation regimen was necessary. Treatment was changed to a new system called DALI (direct adsorption of lipids). After confirmation of the diagnosis HIT II, DALI LDL-apheresis was carried out with recombinant-hirudin (lepirudin) and citrate in order to prevent hypercoagulability. Efficient LDL-apheresis therapy with minimum therapeutic blood levels of lepirudin (1.4 mg/dl) was achieved with an initial intravenous bolus of 0.114 mg/kg of lepirudin followed by continuous lepirudin infusion of 0.350 mg/h. Thrombin-antithrombin iii complex production was well controlled and other hemostatic markers showed no abnormalities. LDL-cholesterol and lipoprotein(a) concentrations were effectively reduced. R-hirudin offers a novel anticoagulation strategy and is, at present, the only alternative for patients with HIT II requiring LDL-apheresis on a regular basis.
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ranking = 3
keywords = lipoprotein
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4/84. A newly identified lipoprotein lipase (LPL) gene mutation (F270L) in a Japanese patient with familial LPL deficiency.

    We have systematically investigated the molecular defects resulting in a primary lipoprotein lipase (LPL) deficiency in a Japanese male infant (proband SH) with fasting hyperchylomicronemia. Neither LPL activity nor immunoreactive LPL mass was detected in pre- or postheparin plasma from proband SH. dna sequence analysis of the LPL gene of proband SH revealed homozygosity for a novel missense mutation of F270L (Phe(270)-->Leu/TTT(1065)-->TTG) in exon 6. The function of the mutant F270L LPL was determined by both biochemical and immunocytochemical studies. in vitro expression experiments on the mutant F270L LPL cDNA in COS-1 cells demonstrated that the mutant LPL protein was synthesized as a catalytically inactive form and its total amount was almost equal to that of the normal LPL. Moreover, the synthesized mutant LPL was non-releasable by heparin because the intracellular transport of the mutant LPL to the cell surface - by which normal LPL becomes heparin-releasable - was impaired due to the abnormal structure of the mutant LPL protein. These findings explain the failure to detect LPL activities and masses in pre- and postheparin plasma of the proband. The mutant F270L allele generated an XcmI restriction enzyme site in exon 6 of the LPL gene. The carrier status of F270L in the proband's family members was examined by digestion with XcmI. The proband was ascertained to be homozygous for the F270L mutation and his parents and sister were all heterozygous. The LPL activities and masses of the parents and the sister (carriers) were half or less than half of the control values. Regarding the phenotype of the carriers, the mother with a sign of hyperinsulinemia manifested hypertriglyceridemia (type IV hyperlipoproteinemia), whereas the healthy father and the sister were normolipidemic. Hyperinsulinemia may be a strong determinant of hypertriglyceridemia in subjects with heterozygous LPL deficiency.
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ranking = 6.006929407889
keywords = lipoprotein, deficiency
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5/84. Novel glomerular lipoprotein deposits associated with apolipoprotein e2 homozygosity.

    BACKGROUND: Hyperlipoproteinemia is occasionally associated with severe glomerular injury caused by abnormal accumulation of lipid in glomeruli, which occurs in conditions such as lipoprotein glomerulopathy (LPG). This study investigates the cases of two siblings with homozygous apolipoprotein (apo) E2 who show unique histologic features, massive proteinuria, and dysbetalipoproteinemia. methods: Histologic studies were performed using renal biopsy specimens. plasma lipoproteins were extensively characterized. The exons of the apo E genes were sequenced to avoid missing any mutations. RESULTS: Histologically, the siblings' condition resembled LPG by light microscopy studies. Electron microscopy studies revealed large lipoid deposits in the paramesangium, subendothelium, and subepithelium of the glomeruli, which were different from LPG in terms of not forming the layered structure resembling a fingerprint even in large lipoprotein thrombi, and mesangial foam cells. Immunohistochemically, the lipoid deposits contained apo E and apo B. These patients did not have either diabetic nephropathy or other known forms of glomerulonephritis. The sequence of exons of the apo E genes revealed homozygosity for apo E2 in both cases. CONCLUSION: The extensive lipoprotein deposition in glomeruli, which resembles LPG, can also occur in apo E2 homozygous individuals, but in a distinct fashion. Because the two cases were siblings, they may have other shared alleles, in addition to the apo E2 allele, that negatively affect processing of lipoproteins and lead to abnormal accumulation of lipoprotein deposits in glomeruli.
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ranking = 17
keywords = lipoprotein
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6/84. A case of anorexia nervosa with severe hyperlipoproteinemia.

    OBJECTIVE: The complication of severe hyperlipoproteinemia with anorexia nervosa is very rare. We investigated the mechanisms of severe hyperlipoproteinemia in a patient with anorexia nervosa. methods: The measurement of plasma levels of lipids, apolipoproteins (Apo), lipoprotein subfractions, free T3, and estrogen, apo (lipoprotein) E phenotyping, and the assay of lymphocyte low-density lipoprotein (LDL)-receptor activity were accomplished in a 40-year-old female patient with anorexia nervosa. RESULTS: Her body mass index was 10.3 kg/m2. Her plasma levels of total cholesterol (C), triglyceride (TG), apoB, apoE, very-low-density lipoprotein (VLDL)-C, and intermediate-density lipoprotein (IDL)-C were 757 mg/dl, 526 mg/dl, 288 mg/dl, 13.6 mg/dl, 133 mg/dl, and 99 mg/dl, respectively. VLDL was cholesterol rich (C/TG ratio = 0.68; normal value = 0.2). The plasma LDL was high and skewed to less dense fractions. Her apoE phenotype was E 3/2. Her lymphocyte LDL-receptor activity was 79% of normal subjects. The plasma level of estradiol was low and that of free T3 was subnormal. DISCUSSION: We concluded that the plasma lipoprotein abnormality of this anorexia nervosa patient was induced by the impaired removal of TG-rich lipoprotein remnants and less dense LDL due to apoE phenotype E 3/2, subnormal LDL-receptor activity, subnormal plasma level of free T3, and diminished secretion of estrogen.
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ranking = 14
keywords = lipoprotein
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7/84. Isolated internal cerebral venous thrombosis in a neonate with increased lipoprotein (a) level: diagnostic and therapeutic considerations.

    Background. Internal cerebral venous thrombosis is a life-threatening condition, which requires immediate therapy. Being infrequent in childhood, internal venous thrombosis is very rare in the neonate and has never been observed without concomitant occlusion of further dural sinuses. Case Description. We report a neonate born at term, who developed seizures on the third day of life after normal pregnancy and uneventful delivery. Ultrasound and CT disclosed bilateral intraventricular and intracerebral hemorrhage with an unusual distribution for germinal matrix hemorrhage. MRI disclosed thrombotic occlusion of the straight sinus and the internal cerebral veins with concomitant bleeding into the ventricles, the basal ganglia, thalamus and the periventricular hemispheres. The other sinuses were not affected. The clinical condition of the child improved after initiation of a low-dose heparin treatment with subsequent partial recanalization of the occluded vessels. The screening for risk factors disclosed an elevated lipoprotein (a) level, also present in both parents. Conclusion. Internal cerebral venous thrombosis may be encountered in neonates and must be included in the list of differential diagnosis of perinatal intraventricular and intracerebral bleeding. MRI allows the diagnosis even in the absence of widespread dural sinus occlusion. Low dose heparin may be a therapeutic option in these cases. This is the first report of neonatal internal venous thrombosis due to hereditary lipoprotein (a) level elevation, which must be included in the list of possible predisposing conditions.
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ranking = 6
keywords = lipoprotein
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8/84. Simultaneous occurrence of selective ACTH insensitivity, achalasia and alacrimia accompanied by hyperlipoproteinaemia.

    An extremely rare clinical syndrome on a 7-year-old-girl is presented. Besides isolated glucocorticoid insufficiency, achalasia and alacrima disturbance of the lipid metabolism was also detected--being a special feature of this case. The details of the endocrine workup is discussed, providing clues for the possible pathomechanism. The correct diagnosis and specific therapy is of utmost importance in the everyday life of the patient.
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ranking = 4
keywords = lipoprotein
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9/84. A case of marked hyperlipoprotein(a)emia associated with nephrotic syndrome and advanced atherosclerosis.

    In 1989, we encountered a 68-year-old male patient with marked hyperlipoprotein(a)emia (hyperLp(a)emia), who was being treated for hypertension and arteriosclerotic obliterans (ASO) at an outpatient clinic of our hospital. He began to develop leg edema in 2002 and was referred to the Department of internal medicine. It was determined that he had severe hyperlipidemia (total cholesterol, 362 mg/dl), proteinuria, and hypoalbuminemia, suggesting the presence of nephrotic syndrome. On lipoprotein analysis, he was found to have very high levels of Lp(a) in the plasma (329 mg/dl). Severe atherosclerosis was also found: that is, abdominal aortic aneurysm (AAA) and coronary artery disease (CAD) were detected, in addition to ASO. After remission of the nephrotic syndrome, the plasma Lp(a) level decreased to 204 mg/dl and the total cholesterol concentration decreased to 179 mg/dl, while very high levels of Lp(a) persisted. We estimate that the markedly elevated Lp(a) plasma levels in this patient may have played some role in the progression of atherosclerosis.
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ranking = 6
keywords = lipoprotein
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10/84. A patient with protein-losing enteropathy associated with systemic lupus erythematosus.

    A 46-year-old woman previously diagnosed as having systemic lupus erythematosus presented with severe hypoalbuminemia and anasarca. She was demonstrated to have protein-losing enteropathy without any other active symptoms of SLE. Her bowel habit was normal and endoscopic examination revealed non-specific colitis and a small ulcer in the duodenum. serum biochemistry showed an abnormal profile of the serum protein, including severe hyperlipoproteinemia and hyperfibrinogenemia. The process of protein-losing was not selective in terms of the molecular size. All of these symptoms and the abnormalities in laboratory data were improved by corticosteroid therapy.
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ranking = 1
keywords = lipoprotein
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