Changes in lipoprotein profile and urinary albumin excretion in familial LCAT deficiency with lipid lowering therapy

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• 作者：Michael S. Yee ; Darrell V. Pavitt ; William Richmond ; H. Terence Cook ; Adam G. McLean ; Jonathan Valabhji ; Robert S. Elkeles
• 关键词：Familial LCAT deficiency ; Lipoprotein (X) ; Iodixanol density gradient ultracentrifugation ; HDL cholesterol ; Coronary calcification score
• 刊名：Atherosclerosis
• 出版年：2009
• 出版时间：August 2009
• 年：2009
• 卷：205
• 期：2
• 页码：528-532
• 全文大小：668 K

文摘

Familial lecithin:cholesterol acyltransferase deficiency (FLD) is a monogenic autosomal recessive condition, affecting cholesterol esterification and leads to progressive renal impairment and end-stage renal failure, probably due to the abnormal lipoprotein (X) (Lp(X)).

We report a case of FLD, whom we treated with a combination of nicotinic acid 1.5 g nocte and fenofibrate M/R 160 mg od and report changes in lipid profile and Lp(X), after six weeks and serum creatinine and urine albumin/creatinine ratio after 12 months. We assessed the cardiovascular risk using electron beam computed tomography.

At baseline total cholesterol was 6.61 mmol/L; HDL cholesterol 0.57 mmol/L; Lp(X) cholesterol 3.24 mmol/L; triglyceride 4.13 mmol/L; apolipoprotein A1 46 mg/dL; and apolipoprotein B 53 mg/dL. After six weeks of treatment his total cholesterol was 4.16; HDL cholesterol 0.52; Lp(X) cholesterol 1.73 mmol/L; triglyceride 1.80 mmol/L; apolipoprotein A1 36 mg/dL; and apolipoprotein B 50 mg/dL. Baseline serum creatinine was 106 μmol/L and urine albumin/creatinine ratio was 127.3 mg/mmol and after 12 months was 101 μmol/L and 31.5 mg/mmol respectively. His coronary artery calcification score was zero.

We have shown, we believe for the first time, that combination lipid modifying therapy in FLD leads to a reduction in Lp(X) concentration and an associated reduction in urine albumin excretion at 12 months.