Polyunsaturated fatty acid deficiency during dietary treatment of very long-chain acyl-CoA dehydrogenase deficiency. Rescue with soybean oil
Polyunsaturated fatty acid deficiency during dietary treatment of very long-chain acyl-CoA dehydrogenase deficiency. Rescue with soybean oil
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极长链酰基辅酶A脱氢酶缺乏症饮食治疗期间多不饱和脂肪酸缺乏。
DOI：
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发表时间：
2001
影响因子：
4.2
通讯作者：
P. Sanjurjo
中科院分区：
文献类型：
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作者：
J. Ruiz;L. Aldámiz;J. Arrizabalaga;L. Aquino;P. Jimeno;G. Pérez‐Nanclares;P. Sanjurjo
超长链酰辅酶A脱氢酶(VLCAD)缺乏症的营养管理是基于避免禁食和用中链甘油三酯替代长链和超长链甘油三酯。我们报告了两例这种疾病，在营养治疗开始后的三个月和五个月后发展为ω-6必需脂肪酸缺乏症(自闭症产品：Monogen)。这种改变在这些患者中可能特别危险，因为他们可能对色素性视网膜病变的发展敏感。将亚油酸作为总卡路里摄入量的3-4%作为大豆油的补充可以改善这一缺陷。我们想就VLCAD缺乏症营养管理中的早期并发症以及在饮食中加入大豆油的可能性进行评论。
Nutritional management of very long-chain acyl-CoA dehydro"genase (VLCAD) deficiency is based on the avoidance of fasting and substitution of medium-chain triglycerides for long- and very long-chain triglycerides. We report two cases of this disease, which developed ω-6 essential fatty acid deficiency after three and five months from the beginning of nutritional therapy (SHS product: Monogen). This alteration could be especially dangerous in these patients owing to their possible susceptibility to the development of pigmentary retinopathy. The incorporation of linoleic acid as 3–4% of total caloric intake supported as soybean oil ameliorates this deficiency. We wish to remark on this early complication in the nutritional management of VLCAD deficiency and the possibility of rescue by the incorporation of soybean oil into the diet.
DOI：
10.1016/s0021-9258(18)54882-1
发表时间：
1991-10
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
A. Voss;M. Reinhart;S. Sankarappa;H. Sprecher
通讯作者：
A. Voss;M. Reinhart;S. Sankarappa;H. Sprecher