1/13. liver transplantation in a child with severe hypercholesterolaemia in alagille syndrome.INTRODUCTION: liver transplantation is a curative treatment modality in children with end stage liver disease in alagille syndrome. CLINICAL PICTURE: We report a 3-year-old child with this condition who had severe hypercholesterolaemia, pruritus and extensive xanthomatosis. TREATMENT: liver transplantation was performed in this patient. OUTCOME: He recovered well with normalisation of his lipid profile. This procedure also resulted in resolution of the disfiguring xanthomatosis.- - - - - - - - - - ranking = 1keywords = plant (Clic here for more details about this article) |
2/13. gout, bradycardia, and hypercholesterolemia after renal transplantation.Approximately 17,000 solid organ transplantations are done annually in the united states. Increasingly, care of these patients will be provided by primary care physicians. In this report, we illustrate the complexity of common medical problems in a patient who had cellulitis and who had had a cadaveric renal transplantation 10 years earlier. Immunosuppressive therapy was cyclosporine (100 mg twice a day) and prednisone (10 mg once a day). The patient's hospital course was complicated by acute gout and symptomatic bradycardia. In both instances, usual treatment--full-dose indomethacin for gout and withholding verapamil for bradycardia--could have had significant interaction with the cyclosporine. At the time of discharge, a therapeutic plan for long-term management of hypercholesterolemia included possible drug interactions with cyclosporine. The potential for drug toxicity in the transplant patient necessitates careful monitoring of immunosuppressive drug levels. Ongoing communication with the transplant center is also needed.- - - - - - - - - - ranking = 1.3333333333333keywords = plant (Clic here for more details about this article) |
3/13. Central neurotoxicity of cyclosporine in two children with nephrotic syndrome.The central neurotoxicity of cyclosporin A (CsA) has been abundantly documented in pediatric and adult recipients of bone marrow or organ transplants, with variations in the rate of occurrence from 0.5% to 35%. We report two cases of central neurotoxicity ascribable to CsA in children with nephrotic syndrome due to lipoid nephrosis. The manifestations of CsA-related central neurotoxicity include confusion, aphasia, dystonias, akinetic mutism, parkinsonism, palsies, seizures, catatonia, coma, brain hemorrhage, and cortical blindness. Decreased density of the cerebral white matter is visible by computed tomography (CT) in 50% of cases, with the most commonly involved sites being the occipital cortex, the cerebellum, the periventricular substance, and the brainstem. magnetic resonance imaging is more sensitive and more specific than CT for investigating the white matter. High-signal lesions are seen on T2-weighted sequences in the areas that are abnormal by CT. Many risk factors have been reported, including hypomagnesemia, hypocholesterolemia, high-dose glucocorticoid therapy, arterial hypertension, and infections. We present two patients with central neurotoxicity both of whom have elevated cholesterol levels.- - - - - - - - - - ranking = 0.16666666666667keywords = plant (Clic here for more details about this article) |
4/13. Homozygous hypercholesterolaemia and ezetimibe: a case report.A girl of Indian origin presented with unusual nodules on her hands, and total cholesterol was found to be > 25 mmol/L. The girl had "mild" P664L mutation and total cholesterol levels fell by 38% when she was on a diet and statin therapy. A further reduction of 26% in total cholesterol and 37% in low-density lipoprotein (LDL) was achieved by adding ezetimibe to the treatment. CONCLUSION: A case of homozygous hypercholesterolaemia is reported in order to highlight treatment options such as liver transplantation, LDL-aphaeresis and treatment with ezetimibe.- - - - - - - - - - ranking = 0.16666666666667keywords = plant (Clic here for more details about this article) |
5/13. Severe hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of the liver.We describe a series of cases of extreme hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of the liver after an allogeneic bone marrow transplant. All of the patients presented with a total cholesterol in excess of 1000 mg/dl (25.9 mmol/l). At the time they were also noted to have pseudohyponatremia. Cholesterol appeared to be predominantly carried by lipoprotein X. Intrahepatic cholestasis leading to reflux of bile lipoproteins into the bloodstream and subsequent formation of protein X appears to be the mechanism underlying this phenomenon. Complications, including retinal cholesterol thromboembolism and cholesteroloma of the lung have been seen in the patient with the highest cholesterol levels. Severe hypercholesterolemia is an important, and likely more common than previously reported, long-term complication of allogeneic hematopoietic stem cell transplantation. It is important for clinicians to familiarize themselves with the diagnostic and therapeutic challenges this condition presents.- - - - - - - - - - ranking = 0.33333333333333keywords = plant (Clic here for more details about this article) |
6/13. Severe hypercholesterolemia associated with decreased hepatic triglyceride lipase activity and pseudohyponatremia in patients after allogeneic stem cell transplantation.A 55-year-old woman with Ph-negative acute lymphoblastic leukemia in primary induction failure received allogeneic peripheral blood stem cell transplantation from her HLA-compatible sister. Pseudohyponatremia developed due to extreme hypercholesterolemia of 4091 mg/dL accompanied by lipoprotein X and lipoprotein Y.The hypercholesterolemia was caused by cholestasis due to chronic GVHD and ischemic cholangiopathy. In addition, we found that hepatic triglyceride lipase (HTGL) activity was severely decreased, which could be another novel factor causing extreme hypercholesterolemia after allogeneic transplantation. The total cholesterol has been gradually decreasing followed by the improvement of cholestasis with bezafibrate, ursodeoxycholic acid and prednisone treatments, and by a slight increase in HTGL-protein. To our knowledge, this is the first report to describe the association of decreased HTGL with extreme hypercholesterolemia after allogeneic transplantation.- - - - - - - - - - ranking = 1.1666666666667keywords = plant (Clic here for more details about this article) |
7/13. How to choose a statin after kidney transplantation: case analyses.Statins are effective to reduce total cholesterol and low-density lipoprotein fractions in a dose-dependent manner. Moreover, they have an excellent safety profile. Besides cholesterol-lowering properties, statins have additional actions described in the present case report. The diverse effects of these drugs means that many patients with different disease entities, such as cardiovascular diseases, can benefit from their effects. However, the problem of so called "class effect" remains controversial. The question whether each drug within the group is equivalent appears important, as long as statins must be taken long-term and as the therapy affects patient quality of life and survival. To address this question, we present two renal transplant recipients treated with statins. The induction of pharmacokinetic tolerance and damage to hepatic cells during administration of statins is discussed. Presented data suggest that the choice of statin should be based on individual patient requirements and adapted to the individual treatment response.- - - - - - - - - - ranking = 0.83333333333333keywords = plant (Clic here for more details about this article) |
8/13. Aneurysmal dilatation in saphenous vein bypass grafts.Three patients are reported who developed aneurysmal degeneration of a saphenous vein arterial bypass graft. All three had hypercholesterolemia and two had marked elevation of triglycerides. One bypass was for occlusive disease and two for popliteal aneurysm. There was a long lag, 3 to 7 years between graft implantation and aneurysmal degeneration. The pathology was similar for all three cases with lipid laden macrophages, loss of elastic lamina, and other atherosclerotic changes in the vein wall. While atheromatous changes and aneurysm formation in saphenous vein bypass grafts are rare, this may occur especially in patients with hypercholesterolemia and hypertriglyceridemia. Careful and prolonged follow-up plus vigorous management of the hyperlipidemic state is mandatory in these patients.- - - - - - - - - - ranking = 0.16666666666667keywords = plant (Clic here for more details about this article) |
9/13. rhabdomyolysis and renal injury with lovastatin use. Report of two cases in cardiac transplant recipients.Hyperlipidemia, particularly hypercholesterolemia, occurs in cardiac transplant recipients both as a preexisting condition and as a consequence of immunosuppressive therapy. lovastatin (Mevacor) has emerged as an agent that may effectively manage this condition. Few serious side effects of this drug have been observed. We describe two cardiac transplant recipients treated with lovastatin in conjunction with their other medications, including cyclosporine, who developed acute renal failure and rhabdomyolysis. Resolution of muscle damage followed discontinuation of cyclosporine and lovastatin therapy. We postulate that hepatic dysfunction secondary to cyclosporine predisposed these patients to lovastatin-induced muscle damage. Use of this drug in cardiac and other organ transplant recipients should be accompanied by close surveillance of creatine kinase, hepatic transaminases, and cyclosporine levels.- - - - - - - - - - ranking = 1.1666666666667keywords = plant (Clic here for more details about this article) |
10/13. Phytosterolaemia, xanthomatosis and premature atherosclerotic arterial disease: a case with high plant sterol absorption, impaired sterol elimination and low cholesterol synthesis.A fourth case is described in which phytosterolaemia, earlier diagnosed as familial hypercholesterolaemia, was associated with normocholesterolaemia, hypersplenism and premature atherosclerotic arterial disease requiring a three-vessel coronary bypass at the age of 29 years. During a follow-up of 5 years 22-26% and 27-30% of serum and bile sterols were plant sterols, respectively. In addition to campesterol and beta-sitosterol, stigmasterol and a fourth major plant sterol, tentatively identified as avenasterol, were found in bile, and in free and esterified forms in all serum lipoproteins. Analysis of faecal steroids and measurement of biliary lipid secretion indicated that in addition to enhanced absorption of plant sterols their decreased biliary secretion contributed to the development of phytosterolaemia. Impaired biliary cholesterol secretion was compensated for by a markedly reduced cholesterol but normal bile acid synthesis and resulted in bile undersaturated with respect to cholesterol, in a reduced intestinal cholesterol pool and in a very low faecal excretion of cholesterol as neutral sterols. Cholestyramine brought about a modest increase in cholesterol elimination as bile acids, increased cholesterol synthesis as evidenced by the sterol balance value and the increased cholesterol precursors squalene and methyl sterols in plasma and bile, and reduced the plasma cholesterol by 21% and plant sterols by 16%, but had no effect on the biliary composition of main sterols.- - - - - - - - - - ranking = 1.3333333333333keywords = plant (Clic here for more details about this article) |
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