Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0242339 (dyslipidemia)
 13,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Low-density lipoprotein apheresis (LDL-apheresis) was done with either cascade filtration (DF) or dextran sulfate cellulose adsorption (DSC) in a patient with primary biliary cirrhosis who developed severe dyslipidemia associated with cholestasis and accumulation of lipoprotein-X (LP-X). The extracorporeal treatment was initially performed weekly, and resulted in a sharp drop in total cholesterol from 1038 to 430 mg/dl. During the next four months the patient was treated every 10-15 days, and pre-apheresis cholesterol levels were maintained between 438 and 505 mg/dl, until an orthotopic liver transplantation was successfully performed. With semi-selective DF a mean 47.1% of total cholesterol was removed per procedure compared to 30.0% with DSC, although the volume of treated plasma was 38.0 vs 49.9 ml/kg body weight. The changes in plasma cholesterol levels during DSC and DF showed that the kinetics of cholesterol removal were similar with both techniques, but the efficacy differed; DF removed both LDL and LP-X from plasma, whereas DSC selectively lowered the LDL content. Cascade filtration may therefore be considered as a first-choice treatment for patients with LP-X accumulation due to cholestasis.
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PMID:Impaired efficacy of selective LDL-apheresis in primary biliary cirrhosis. 206 Sep 91

A 75 year old woman presented with long-lasting pruritus and elevation of serum levels of alkaline phosphatase and gamma-glutamyl transpeptidase (GGT). Potentially hepatotoxic drugs were stopped; an abdominal ultra-sonography was normal. Antimitochondrial antibodies were elevated and are the serologic hallmark of primary biliary cirrhosis. The disease can progress from an asymptomatic inflammation to a cirrhosis. The disease-modifying treatment consists in ursodeoxycholic acid 13-15 mg/kg per day. Concomitant to the primary biliary cirrhosis hypothyroidism, osteoporosis, malabsorption and dyslipidemia can occur.
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PMID:[No complaints, but constantly elevated cholestatic serum liver tests]. 2173 1

Primary biliary cirrhosis (PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies (AMA), and characteristic liver biopsy findings of nonsuppurative destructive cholangitis and interlobular bile duct destruction. About 10% of PBC patients, however, lack AMA. A variant, called PBC-autoimmune hepatitis (AIH) overlap, is characterized by the above findings of PBC together with findings of elevated serum alanine aminotransferase, elevated serum immunoglobulin G, and circulating anti-smooth muscle antibodies, with liver biopsy demonstrating periportal or periseptal, lymphocytic, piecemeal necrosis. PBC is hypothesized to be related to environmental exposure in genetically vulnerable individuals. It typically occurs in middle-aged females. Prominent clinical features include fatigue, pruritis, jaundice, xanthomas, osteoporosis, and dyslipidemia. The Mayo Risk score is the most widely used and best prognostic system. Ursodeoxycholic acid is the primary therapy. It works partly by reducing the concentration and injury from relatively toxic bile acids. PBC-AIH overlap syndrome is treated with ursodeoxycholic acid and corticosteroids, especially budesonide. Obeticholic acid and fibrate are promising new, but incompletely tested, therapies. Liver transplantation is the definitive therapy for advanced disease, with about 70% 10-year survival after transplantation. Management of pruritis includes local skin care, dermatologist referral, avoiding potential pruritogens, cholestyramine, and possibly opioid antagonists, sertraline, or rifaximin. Management of osteoporosis includes life-style modifications, administration of calcium and vitamin D, and alendronate. Statins are relatively safe to treat the osteopenia associated with PBC. Associated Sjogren's syndrome is treated by artificial tears, cyclosporine ophthalmic emulsion to stimulate tear production; and saliva substitutes, cholinergic agents, and scrupulous oral and dental care. Complications of cirrhosis from advanced PBC include esophageal varices, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, and hepatoma formation.
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PMID:Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy. 2595 76

The liver plays a crucial role in the metabolism of lipids and lipoproteins through the biosynthesis of cho- lesterol, fatty acids, apolipoproteins, and proteins involved in lipoprotein homeostasis. Therefore, changes in the hepatic contents of cholesterol or fatty acids or the impairment of the protein synthesis by liver disorders, such as primary biliary cirrhosis, cirrhosis, and fatty liver, can directly or indirectly influence the lipo- protein profile in the circulation. Although dyslipidemia secondary to liver diseases might not have clinical importance from the aspect of diagnosis(i.e., dyslipidemia rarely gives a clue to diagnose liver diseases), it might possess clinical significance in the near future after a paradigm shift in the treatment of liver diseases; since the prognosis of pa- tients with liver diseases has been improving as a result of emerging novel drugs, such as anti-HCV reagents, long-term complications, such as atherosclerosis, might be important in the management of liver diseases. [Review].
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PMID:[Dyslipidemia Secondary to Liver Diseases]. 3069 61