Bile lipid composition and haemostatic variables in a case of high density lipoprotein deficiency (Tangier disease).
A 62-year-old man with clinical and biochemical findings consistent with homozygous Tangier disease is presented. Widespread atherosclerosis was present. Bile lipid analysis showed a low molar percentage of cholesterol with a low saturation index. The data suggest that high density lipoprotein cholesterol may act as a preferential precursor of biliary cholesterol. Coagulation and platelet studies indicated that the patient's platelets were hyper-responsive to aggregating agents and produced an increased amount of thromboxane B2. A platelet storage pool deficiency was also found.
D000368:Aged; D016632:Apolipoprotein A-I; D001053:Apolipoproteins / Q000097:blood; D001646:Bile / Q000032:analysis*; D001647:Bile Acids and Salts / Q000032:analysis; D001778:Blood Coagulation Disorders / Q000097:blood* / Q000150:complications; D001791:Blood Platelet Disorders / Q000097:blood* / Q000150:complications; D002784:Cholesterol / Q000032:analysis / Q000097:blood; D008076:Cholesterol, HDL; D006801:Humans; D007009:Hypolipoproteinemias / Q000378:metabolism*; D008055:Lipids / Q000032:analysis*; D008075:Lipoproteins, HDL / Q000032:analysis / Q000097:blood; D008297:Male; D008875:Middle Aged; D012701:Serotonin / Q000097:blood; D013631:Tangier Disease / Q000097:blood / Q000150:complications / Q000378:metabolism*; D013929:Thromboxane B2 / Q000097:blood
D016632:Apolipoprotein A-I; D001053:Apolipoproteins; D001647:Bile Acids and Salts; D008076:Cholesterol, HDL; D008055:Lipids; D008075:Lipoproteins, HDL; D012701:Serotonin; D013929:Thromboxane B2; D002784:Cholesterol