کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
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5985902 | 1178787 | 2014 | 5 صفحه PDF | دانلود رایگان |
âºAlthough total cholesterol is often elevated in PBC, the severe hypercholesterolemia (>1,400 mg/dL) of this patient is extremely rare.âºThe presence of interdigital xanthomas, xanthelasmas and palmar xanthomas illustrates that these lipid deposits can be present both in primary and secondary hypercholesterolemias.âºPlasma phytosterols were extremely high, suggesting a selective defect in the biliary excretion of phytosterols in PBC.âºThe patient did not show any functional mutation in ABCG5/G8 genes.âºThe severe hypercholesterolemia, with only mild cholestasis, indicates a selective defect in sterol biliary secretion, probably independent of ABCG5/ABCG8 excretion mechanism.
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.
Journal: Journal of Clinical Lipidology - Volume 8, Issue 5, SeptemberâOctober 2014, Pages 520-524