Document Detail


Changes in lipoprotein profile and urinary albumin excretion in familial LCAT deficiency with lipid lowering therapy.
MedLine Citation:
PMID:  19230892     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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 1.5g nocte and fenofibrate M/R 160mg 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 6.61mmol/L; HDL cholesterol 0.57mmol/L; Lp(X) cholesterol 3.24mmol/L; triglyceride 4.13mmol/L; apolipoprotein A1 46mg/dL; and apolipoprotein B 53mg/dL. After six weeks of treatment his total cholesterol was 4.16; HDL cholesterol 0.52; Lp(X) cholesterol 1.73mmol/L; triglyceride 1.80mmol/L; apolipoprotein A1 36mg/dL; and apolipoprotein B 50mg/dL. Baseline serum creatinine was 106micromol/L and urine albumin/creatinine ratio was 127.3mg/mmol and after 12 months was 101micromol/L and 31.5mg/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.
Authors:
Michael S Yee; Darrell V Pavitt; William Richmond; H Terence Cook; Adam G McLean; Jonathan Valabhji; Robert S Elkeles
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2009-01-29
Journal Detail:
Title:  Atherosclerosis     Volume:  205     ISSN:  1879-1484     ISO Abbreviation:  Atherosclerosis     Publication Date:  2009 Aug 
Date Detail:
Created Date:  2009-07-28     Completed Date:  2009-12-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0242543     Medline TA:  Atherosclerosis     Country:  Ireland    
Other Details:
Languages:  eng     Pagination:  528-32     Citation Subset:  IM    
Affiliation:
Imperial College London, St Mary's Hospital, London, UK. m.yee@imperial.ac.uk
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MeSH Terms
Descriptor/Qualifier:
Adult
Albumins / analysis*
Antilipemic Agents / therapeutic use
Calcium / metabolism
Cholesterol / metabolism
Coronary Vessels / pathology
Humans
Lecithin Acyltransferase Deficiency / blood*,  genetics*,  urine*
Lipoprotein-X / metabolism
Lipoproteins / blood*
Male
Niacin / pharmacology
Procetofen / pharmacology
Tomography, X-Ray Computed / methods
Triglycerides / metabolism
Chemical
Reg. No./Substance:
0/Albumins; 0/Antilipemic Agents; 0/Lipoprotein-X; 0/Lipoproteins; 0/Triglycerides; 49562-28-9/Procetofen; 57-88-5/Cholesterol; 59-67-6/Niacin; 7440-70-2/Calcium

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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