Lipoprotein-X in a Patient with Lymphoplasmacytic Sclerosing Cholangitis: An Unusual Cause of Secondary Hypercholesterolemia
ABSTRACT: Objective: To describe the presence of lipoprotein-X in a patient with obstructive liver disease and review the available literature on secondary hypercholesteremia occurring in the presence of lipoprotein-X.Methods: Reported is the case of a 40-year-old man who presented with severe hyper...
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| Format: | Article |
| Language: | English |
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Elsevier
2016-01-01
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| Series: | AACE Clinical Case Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2376060520303722 |
| _version_ | 1818725084688285696 |
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| author | Amy Chow, MD Vincent A. Rifici, PhD Stephen H. Schneider, MD |
| author_facet | Amy Chow, MD Vincent A. Rifici, PhD Stephen H. Schneider, MD |
| author_sort | Amy Chow, MD |
| collection | DOAJ |
| description | ABSTRACT: Objective: To describe the presence of lipoprotein-X in a patient with obstructive liver disease and review the available literature on secondary hypercholesteremia occurring in the presence of lipoprotein-X.Methods: Reported is the case of a 40-year-old man who presented with severe hypercholesterolemia due to obstructive cholestasis secondary to lymphoplasmacytic sclerosing cholangitis. A literature search was conducted through PubMed for references from 1976 to 2013 regarding the appearance of lipoprotein-X in plasma secondary to cholestasis. Twenty-eight articles were found that addressed the physiology, pathogenesis, complications, or treatments of lipoprotein-X in the context of cholestasis.Results: Severe hypercholesterolemia was found in a patient with lymphoplasmacytic sclerosing cholangitis. His elevated plasma cholesterol level was due to the presence of lipoprotein-X, which is a particle with a density similar to low-density lipoprotein (LDL) that does not contain apolipoprotein B. The pathogenesis of lipoprotein-X is poorly understood. Unlike LDL, lipoprotein-X may not be atherogenic. Neither statins nor other lipid-lowering agents have been shown to be useful in treating the hypercholesterolemia that results from production of lipoprotein-X.Conclusion: Lipoprotein-X may occur in obstructive liver disease, and the therapy is focused on treating the underlying disease.Abbreviations: ALP = alkaline phosphatase ALT = alanine aminotransferase apo = apolipoprotein AST = aspartate aminotransferase ERCP = endoscopic retrograde cholangiopancreatography HDL = highdensity lipoprotein LCAT = lecithin:cholesterol acyltransferase LDL = low-density lipoprotein Lp-X = lipoprotein-X PBC = primary biliary cirrhosis |
| first_indexed | 2024-12-17T21:36:41Z |
| format | Article |
| id | doaj.art-8dd05ceefb9e41d08333bdba01bdd1cd |
| institution | Directory Open Access Journal |
| issn | 2376-0605 |
| language | English |
| last_indexed | 2024-12-17T21:36:41Z |
| publishDate | 2016-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | AACE Clinical Case Reports |
| spelling | doaj.art-8dd05ceefb9e41d08333bdba01bdd1cd2022-12-21T21:31:43ZengElsevierAACE Clinical Case Reports2376-06052016-01-0121e20e24Lipoprotein-X in a Patient with Lymphoplasmacytic Sclerosing Cholangitis: An Unusual Cause of Secondary HypercholesterolemiaAmy Chow, MD0Vincent A. Rifici, PhD1Stephen H. Schneider, MD2From the Division of Endocrinology, Metabolism and Nutrition, Department of Medicine, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey.From the Division of Endocrinology, Metabolism and Nutrition, Department of Medicine, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey.From the Division of Endocrinology, Metabolism and Nutrition, Department of Medicine, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey.; Address correspondence to Dr. Stephen H. Schneider, Division of Endocrinology, Metabolism and Nutrition, Rutgers Robert Wood Johnson Medical School, 1 RWJ Place Room 386 MEB, New Brunswick, NJ 08903-0019.ABSTRACT: Objective: To describe the presence of lipoprotein-X in a patient with obstructive liver disease and review the available literature on secondary hypercholesteremia occurring in the presence of lipoprotein-X.Methods: Reported is the case of a 40-year-old man who presented with severe hypercholesterolemia due to obstructive cholestasis secondary to lymphoplasmacytic sclerosing cholangitis. A literature search was conducted through PubMed for references from 1976 to 2013 regarding the appearance of lipoprotein-X in plasma secondary to cholestasis. Twenty-eight articles were found that addressed the physiology, pathogenesis, complications, or treatments of lipoprotein-X in the context of cholestasis.Results: Severe hypercholesterolemia was found in a patient with lymphoplasmacytic sclerosing cholangitis. His elevated plasma cholesterol level was due to the presence of lipoprotein-X, which is a particle with a density similar to low-density lipoprotein (LDL) that does not contain apolipoprotein B. The pathogenesis of lipoprotein-X is poorly understood. Unlike LDL, lipoprotein-X may not be atherogenic. Neither statins nor other lipid-lowering agents have been shown to be useful in treating the hypercholesterolemia that results from production of lipoprotein-X.Conclusion: Lipoprotein-X may occur in obstructive liver disease, and the therapy is focused on treating the underlying disease.Abbreviations: ALP = alkaline phosphatase ALT = alanine aminotransferase apo = apolipoprotein AST = aspartate aminotransferase ERCP = endoscopic retrograde cholangiopancreatography HDL = highdensity lipoprotein LCAT = lecithin:cholesterol acyltransferase LDL = low-density lipoprotein Lp-X = lipoprotein-X PBC = primary biliary cirrhosishttp://www.sciencedirect.com/science/article/pii/S2376060520303722 |
| spellingShingle | Amy Chow, MD Vincent A. Rifici, PhD Stephen H. Schneider, MD Lipoprotein-X in a Patient with Lymphoplasmacytic Sclerosing Cholangitis: An Unusual Cause of Secondary Hypercholesterolemia AACE Clinical Case Reports |
| title | Lipoprotein-X in a Patient with Lymphoplasmacytic Sclerosing Cholangitis: An Unusual Cause of Secondary Hypercholesterolemia |
| title_full | Lipoprotein-X in a Patient with Lymphoplasmacytic Sclerosing Cholangitis: An Unusual Cause of Secondary Hypercholesterolemia |
| title_fullStr | Lipoprotein-X in a Patient with Lymphoplasmacytic Sclerosing Cholangitis: An Unusual Cause of Secondary Hypercholesterolemia |
| title_full_unstemmed | Lipoprotein-X in a Patient with Lymphoplasmacytic Sclerosing Cholangitis: An Unusual Cause of Secondary Hypercholesterolemia |
| title_short | Lipoprotein-X in a Patient with Lymphoplasmacytic Sclerosing Cholangitis: An Unusual Cause of Secondary Hypercholesterolemia |
| title_sort | lipoprotein x in a patient with lymphoplasmacytic sclerosing cholangitis an unusual cause of secondary hypercholesterolemia |
| url | http://www.sciencedirect.com/science/article/pii/S2376060520303722 |
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