Document Detail

Plasma plant sterol levels do not reflect cholesterol absorption in children with Smith-Lemli-Opitz syndrome.
MedLine Citation:
PMID:  19101685     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: To test the hypothesis that there is a correlation between the ratio of plant sterols to cholesterol in plasma and dietary cholesterol absorption in children with Smith-Lemli-Opitz syndrome (SLOS), a cholesterol synthesis disorder.
STUDY DESIGN: We obtained measurements of cholesterol absorption with a direct radioisotope cholesterol absorption method during 9 visits of children with SLOS. We measured plasma sterols in 22 children with SLOS and 16 control children, and we measured dietary intake of cholesterol and sitosterol (n=11 SLOS).
RESULTS: The correlations of 2 plasma plant sterol ratios (sitosterol/cholesterol and campesterol/cholesterol) with direct cholesterol absorption measurement were poor (R= -0.33 and R= -0.25, respectively), significantly lower than the published correlation in adults (R=0.73; P< .02).
CONCLUSIONS: Although the ratios of plant sterols to cholesterol in plasma has been used as a surrogate for cholesterol absorption in adults and children, these ratios may not accurately reflect cholesterol absorption in children with SLOS. These ratios should not be used as a surrogate for cholesterol absorption in children without further validation.
Louise S Merkens; Julia M Jordan; Jennifer A Penfield; Dieter Lütjohann; William E Connor; Robert D Steiner
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Publication Detail:
Type:  Evaluation Studies; Journal Article; Research Support, N.I.H., Extramural     Date:  2008-12-21
Journal Detail:
Title:  The Journal of pediatrics     Volume:  154     ISSN:  1097-6833     ISO Abbreviation:  J. Pediatr.     Publication Date:  2009 Apr 
Date Detail:
Created Date:  2009-03-27     Completed Date:  2009-04-14     Revised Date:  2014-09-12    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  557-561.e1     Citation Subset:  AIM; IM    
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MeSH Terms
Biological Markers / blood
Case-Control Studies
Child, Preschool
Cholesterol, Dietary / blood,  metabolism*
Intestinal Absorption*
Phytosterols / blood*
Sensitivity and Specificity
Sitosterols / blood
Smith-Lemli-Opitz Syndrome / blood*,  diet therapy*
Grant Support
M01 RR-000334/RR/NCRR NIH HHS; R01 HL-073980/HL/NHLBI NIH HHS; R01 HL073980/HL/NHLBI NIH HHS; R01 HL073980-01/HL/NHLBI NIH HHS; R01 HL073980-08/HL/NHLBI NIH HHS; UL1 RR024140/RR/NCRR NIH HHS
Reg. No./Substance:
0/Biological Markers; 0/Cholesterol, Dietary; 0/Phytosterols; 0/Sitosterols; 5LI01C78DD/gamma-sitosterol

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