Management of lipoprotein X and its complications in a patient with primary sclerosing cholangitis

• DOI: 10.2217/clp.15.23
• Publication Date: 2015-08-19T13:34:55Z
• Authors: Eric J Brandt; Shane M Regnier; Edward Ky Leung; Sharon H Chou; Beverly W Baron; Helen S Te; Michael H Davidson; Robert M Sargis
• Keywords: Primary Sclerosing Cholangitis; Primary Biliary Cirrhosis; Hyperlipidemia; Sequela

AbstractLipoprotein X (LpX) is an abnormal lipoprotein found in conditions such as lecithin:cholesterol acyltransferase deficiency and cholestatic states (e.g., primary biliary cirrhosis sclerosing cholangitis). Management of severe hypercholesterolemia due to LpX with drugs physical removal methods not well established the literature. A case discussed a 51-year-old woman who presented multiple electrolyte abnormalities, xanthomas neuropathy be secondary setting cholangitis. This highlights that oral medications, including statins, may insufficient normalize lipid levels or improve clinical symptoms presents therapeutic plasma exchange safe effective option treat morbid sequela hyperlipidemia.Keywords:: CholestasisHypercholesterolemiaLipoprotein XNeuropathyPlasma ExchangePrimary Sclerosing CholangitisStatinXanthoma