Merkens Louise S, Jordan Julia M, Penfield Jennifer A, Lütjohann Dieter, Connor William E, Steiner Robert D
Department of Pediatrics, Oregon Health & Science University, Portland, OR 97239-2998, USA.
J Pediatr. 2009 Apr;154(4):557-561.e1. doi: 10.1016/j.jpeds.2008.10.045. Epub 2008 Dec 21.
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.
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).
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).
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.
检验如下假设，即在患有胆固醇合成障碍的史密斯-勒米-奥皮茨综合征（SLOS）儿童中，血浆中植物甾醇与胆固醇的比值和膳食胆固醇吸收之间存在相关性。
我们在SLOS患儿的9次就诊期间，采用直接放射性同位素胆固醇吸收法对胆固醇吸收情况进行了测量。我们测量了22名SLOS患儿和16名对照儿童的血浆甾醇，并测量了11名SLOS患儿的膳食胆固醇和谷甾醇摄入量。
两种血浆植物甾醇比值（谷甾醇/胆固醇和菜油甾醇/胆固醇）与直接胆固醇吸收测量值之间的相关性较差（分别为R = -0.33和R = -0.25），显著低于已发表的成人相关性（R = 0.73；P <.02）。
尽管血浆中植物甾醇与胆固醇的比值已被用作成人和儿童胆固醇吸收的替代指标，但这些比值可能无法准确反映SLOS患儿的胆固醇吸收情况。在未进一步验证之前，这些比值不应作为儿童胆固醇吸收的替代指标。
