Safety and tolerance of the ketogenic diet in patients with Zellweger Syndrome

被引：1

作者：

Stephanie, Borst ^{[1
]}

Michael, Ciliberto ^{[2
]}

Sreenath, Thati Ganganna ^{[2
]}

机构：

[1] Univ Iowa, 225 S Grand Ave, Iowa City, IA 52242 USA

[2] Univ Iowa, Stead Family Childrens Hosp, 200 Hawkins Dr, Iowa City, IA 52242 USA

来源：

EPILEPSY & BEHAVIOR REPORTS | 2024年 / 26卷

关键词：

Epilepsy; Ketogenic Diet; Zellweger Syndrome; Peroxisomal Disorder; CHAIN FATTY-ACIDS; PHYTANIC ACID;

D O I：

10.1016/j.ebr.2024.100655

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Zellweger Syndrome is a peroxisomal disorder that can lead to elevation of long chain fatty acids and epilepsy, which can be drug resistant. The treatment of drug resistant epilepsy can include the ketogenic diet in appropriately chosen patients. Typically, the ketogenic diet is contraindicated in individuals with defects in fatty acid metabolism because of the diet's reliance on medium and long chain fatty acids. To our knowledge this is the first publication outlining the use of the ketogenic diet in patients with defects in beta oxidation of very long chain fatty acids. We present two patients with Zellweger Syndrome who were placed on a ketogenic diet for drug resistant epilepsy. Safety and tolerance of the ketogenic diet in patients with Zellweger Syndrome.

引用

页数：3

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