Management of fatty acid oxidation disorders: A survey of current treatment strategies
Management of fatty acid oxidation disorders: A survey of current treatment strategies
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DOI：
10.1016/s0002-8223(02)90386-x
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发表时间：
2002-12-01
影响因子：
--
通讯作者：
Singh, RH
中科院分区：
文献类型：
--
作者：
Solis, JO;Singh, RH
脂肪酸氧化障碍患者营养护理的规范化。缺乏一项文献综述和全国代谢营养师调查描述了美国目前用于治疗脂肪酸氧化障碍患者的治疗策略范围。由代谢紊乱专业营养师提供的问卷调查评价了用于治疗脂肪酸氧化紊乱、中链酰基辅酶A脱氢酶缺乏症（MCAD）、极长链酰基辅酶A脱氢酶缺乏症（VLCAD）、长链3-羟酰基辅酶A脱氢酶缺乏症（LCHAD）、长链酰基辅酶A脱氢酶缺乏症（LCAD）和三功能蛋白缺乏症（TFP）的做法。这项调查显示，支持使用中的协议的证据严重缺乏。串联质谱技术的最新进展有望增加已确定的脂肪酸氧化障碍患者的数量，这加强了对全面的临床研究的需求，以确定对这些遗传性疾病患者的最佳护理。
Standardization of the nutritional care for patients with fatty-acid oxidation disorders is. lacking. A literature review and national survey of metabolic dietitians describes the range of therapeutic strategies currently employed in the U.S. to treat patients with fatty-acid oxidation disorders. Questionnaire responses provided by dietitians specializing in metabolic disorders evaluated practices used for treatment of fatty acid oxidation disorders, medium-chain acyl-CoA dehydrogenase deficiency (MCAD), very-long-chain acyl-CoA dehydrogenase deficiency (VLCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD), long-chain acyl-CoA dehydrogenase deficiency (LCAD), and Trifunctional Protein deficiency (TFP). This survey reveals a significant lack of evidence supporting the protocols in use. Recent advances in tandem mass spectrometry technology promises an increase in the number of identified patients with fatty-acid oxidation disorders, which reinforces the need for comprehensive, clinical research studies to determine optimal care for patients with these genetic disorders.