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罗伦佐的油治疗89例无症状性肾上腺脑白质营养不良患者的随访研究 被引量:5
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作者 moser h.w. Raymond G.V. 郭俊 《世界核心医学期刊文摘(神经病学分册)》 2005年第12期7-8,共2页
Objectives: To identify asymptomatic boys with X-linked adrenoleukodystrophy who have a normal magnetic resonance image (MRI), and to assess the effect of 4:1 glyceryl trioleate-glyceryl trierucate (Lorenzo’s oil) on... Objectives: To identify asymptomatic boys with X-linked adrenoleukodystrophy who have a normal magnetic resonance image (MRI), and to assess the effect of 4:1 glyceryl trioleate-glyceryl trierucate (Lorenzo’s oil) on disease progression. Method: Eighty-nine boys (mean±SD baseline age, 4.7±4.1 years; range, 0.2-15 years) were identified by a plasma very long-chain fatty acids assay used to screen at-risk boys. All were treated with Lorenzo’s oil and moderate fat restriction. Plasma fatty acids and clinical status were followed for 6.9±2.7 years. Changes in plasma hexacosanoic acid levels were assessed by measuring the length-adjusted area under the curve, and a proportional hazards model was used to evaluate association with the development of abnormal MRI results and neurological abnormalities. Results: Of the 89 boys, 24%developed MRI abnormalities and 11%developed both neurological and MRI abnormalities. Abnormalities occurred only in the 64 patients who were aged 7 years or younger at the time therapy was started. There was significant association between the development of MRI abnormalities and a plasma hexacosanoic acid increase. (For a 0.1-μg/mL increase in the length-adjusted area under the curve for the hexacosanoic acid level, the hazard ratio for incident MRI abnormalities in the whole group was 1.36; P=.01; 95%confidence interval, 1.07-1.72.) Results for patients aged 7 years or younger were similar (P=.04). Conclusions: In this single-arm study, hexacosanoic acidreductionbyLorenzo’s oil was associated with reduced risk of developing MRI abnormalities. We recommend Lorenzo’s oil therapy in asymptomatic boys with X-linked adrenoleukodystophy who have normal brain MRI results. 展开更多
关键词 无症状性 随访研究 十六酸 基线年龄 神经系统异常 长链脂肪酸 临床状况 遗传型 模型评价 测量曲线
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