title: Severe hypercholesterolemia and phytosterolemia with extensive xanthomas in primary biliary cirrhosis: role of biliary excretion on sterol homeostasis.
abstract: Primary biliary cirrhosis (PBC) is an autoimmune, chronic, cholestatic liver disease that affects primarily women. PBC is commonly associated with hypercholesterolemia that has been associated with cholestasis. We report an exceptionally high blood cholesterol and phytosterols with just mild cholestasis indicating a selective defect in sterol biliary secretion in a patient with PBC.
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Servicios de Medicina Interna and Endocrinología, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Lucia
authors.initials: L
authors.lastname: Baila-Rueda
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Servicios de Medicina Interna and Endocrinología, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Rocio
authors.initials: R
authors.lastname: Mateo-Gallego
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Servicios de Medicina Interna and Endocrinología, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Itziar
authors.initials: I
authors.lastname: Lamiquiz-Moneo
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Servicios de Medicina Interna and Endocrinología, Hospital Universitario Miguel Servet, Zaragoza, Spain.
authors.forename: Ana
authors.initials: A
authors.lastname: Cenarro
authors.affiliation: Unidad de Lípidos and Laboratorio de Investigación Molecular, Servicios de Medicina Interna and Endocrinología, Hospital Universitario Miguel Servet, Zaragoza, Spain. Electronic address: civeira@unizar.es.
authors.forename: Fernando
authors.initials: F
authors.lastname: Civeira
mesh_terms: D000328:Adult; D001659:Biliary Tract / Q000378:metabolism*; D005260:Female; D006706:Homeostasis; D006801:Humans; D006937:Hypercholesterolemia / Q000150:complications* / Q000378:metabolism*; D007410:Intestinal Diseases / Q000150:complications* / Q000378:metabolism*; D008052:Lipid Metabolism, Inborn Errors / Q000150:complications* / Q000378:metabolism*; D008105:Liver Cirrhosis, Biliary / Q000150:complications*; D010840:Phytosterols / Q000009:adverse effects* / Q000378:metabolism; D013261:Sterols / Q000378:metabolism*; D014973:Xanthomatosis / Q000150:complications*
chemical_list: D010840:Phytosterols; D013261:Sterols
keywords: Hypercholesterolemia; Phytosterolemia; Primary biliary cirrhosis; Xanthomas