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Neuropediatrics 2016; 47(02): 132-136
DOI: 10.1055/s-0036-1572413
Short Communication
Georg Thieme Verlag KG Stuttgart · New York

Severe Hypertriglyceridemia in Glut1D on Ketogenic Diet

Joerg Klepper
1   Children's Hospital Aschaffenburg Alzenau, Aschaffenburg, Germany
,
Baerbel Leiendecker
2   Department of Paediatric Neurology, University of Essen, Essen, Germany
,
Nicole Heussinger
1   Children's Hospital Aschaffenburg Alzenau, Aschaffenburg, Germany
,
Ekkehart Lausch
3   Department of Paediatrics, University of Freiburg, Freiburg, Germany
,
Friedrich Bosch
4   Children‘s Hospital Klinikum Fürth, Fürth, Germany
› Author Affiliations
Further Information

Publication History

11 November 2015

27 December 2015

Publication Date:
22 February 2016 (online)

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Abstract

High-fat ketogenic diets are the only treatment available for Glut1 deficiency (Glut1D). Here, we describe an 8-year-old girl with classical Glut1D responsive to a 3:1 ketogenic diet and ethosuximide. After 3 years on the diet a gradual increase of blood lipids was followed by rapid, severe asymptomatic hypertriglyceridemia (1,910 mg/dL). Serum lipid apheresis was required to determine liver, renal, and pancreatic function. A combination of medium chain triglyceride-oil and a reduction of the ketogenic diet to 1:1 ratio normalized triglyceride levels within days but triggered severe myoclonic seizures requiring comedication with sultiam. Severe hypertriglyceridemia in children with Glut1D on ketogenic diets may be underdiagnosed and harmful. In contrast to congenital hypertriglyceridemias, children with Glut1D may be treated effectively by dietary adjustments alone.

Keywords

glucose transporter - Glut1 - Glut1 deficiency - ketogenic diet - hypertriglyceridemia - side effects - blood lipids
 

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