title: Plasma plant sterol levels do not reflect cholesterol absorption in children with Smith-Lemli-Opitz syndrome.
abstract: 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.
authors.affiliation: Department of Pediatrics, Oregon Health & Science University, Portland, OR 97239-2998, USA. merkensl@ohsu.edu.
authors.forename: Louise S
authors.initials: LS
authors.lastname: Merkens
authors.forename: Julia M
authors.initials: JM
authors.lastname: Jordan
authors.forename: Jennifer A
authors.initials: JA
authors.lastname: Penfield
authors.forename: Dieter
authors.initials: D
authors.lastname: Lütjohann
authors.forename: William E
authors.initials: WE
authors.lastname: Connor
authors.forename: Robert D
authors.initials: RD
authors.lastname: Steiner
mesh_terms: 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*
chemical_list: D015415:Biomarkers; D002791:Cholesterol, Dietary; D010840:Phytosterols; D012855:Sitosterols; C025473:gamma-sitosterol