Early dietary treatments with Lorenzo's oil and docosahexaenoic acid for neurological development in a case with Zellweger syndrome

被引：66

作者：

Tanaka, Kyoko ^{[1
]}

Shimizu, Toshiaki ^{[1
]}

Ohtsuka, Yoshikazu ^{[1
]}

Yamashiro, Yuichiro ^{[1
]}

Oshida, Kyoichi ^{[1
]}

机构：

[1] Juntendo Univ, Sch Med, Dept Pediat, Bunkyo Ku, Tokyo 1138421, Japan

来源：

BRAIN & DEVELOPMENT | 2007年 / 29卷 / 09期

关键词：

Zellweger syndrome; docosahexaenoic acid; Lorenzo's oil; Lunaria oil;

D O I：

10.1016/j.braindev.2007.02.005

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

We treated a girl with Zellweger syndrome using a special infant formula supplemented with middle chain triglyceride (MCT) milk, docosahexaenoic acid (DHA), Lorenzo's oil, and Lunaria oil, which is rich in nervonic acid (C24: 1). We examined the fatty acid contents of the plasma and red blood cell (RBC) membrane. Neurological development was evaluated using Denver developmental screening test and auditory brainstem response (ABR). Her delayed neurological development, liver dysfunction, and cholestasis were all improved 2 weeks after starting the dietary treatment. DHA level in RBC membranes was increased and very long chain fatty acid (VLCFA,C26:0) levels were decreased. Our findings suggest that the dietary treatment with combination of MCT milk, DHA, Lorenzo's oil, and Lunaria oil in the patients with Zellweger syndrome bring some benefits for neurological development. (c) 2007 Elsevier B.V. All rights reserved.

引用

页码：586 / 589

页数：4

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