Plasma plant sterol levels do not reflect cholesterol absorption in children with Smith-Lemli-Opitz syndrome.
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.


METHODS
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.
Merkens
Louise S
LS
Department of Pediatrics, Oregon Health & Science University, Portland, OR 97239-2998, USA. merkensl@ohsu.edu.
Jordan
Julia M
JM
Penfield
Jennifer A
JA
Lütjohann
Dieter
D
Connor
William E
WE
Steiner
Robert D
RD
D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002791:Cholesterol, Dietary / Q000097:blood / Q000378:metabolism*; D005260:Female; D006801:Humans; D007223:Infant; D007408:Intestinal Absorption; D008297:Male; D010840:Phytosterols / Q000097:blood*; D012680:Sensitivity and Specificity; D012855:Sitosterols / Q000097:blood; D019082:Smith-Lemli-Opitz Syndrome / Q000097:blood* / Q000178:diet therapy*
D015415:Biomarkers; D002791:Cholesterol, Dietary; D010840:Phytosterols; D012855:Sitosterols; C025473:gamma-sitosterol