Plasma Plant Sterol Levels Do Not Reflect Cholesterol Absorption in Children with Smith-Lemli-Opitz Syndrome

Louise S. Merkens, Julia M. Jordan, Jennifer A. Penfield, Dieter Lütjohann, William E. Connor, Robert D. Steiner

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

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. 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.

Original languageEnglish (US)
Pages (from-to)557-561.e1
JournalJournal of Pediatrics
Volume154
Issue number4
DOIs
StatePublished - Apr 2009

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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