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Vol. 56, No. 3, 2010
Issue release date: April 2010
Section title: Original Paper
Ann Nutr Metab 2010;56:225–232
(DOI:10.1159/000283242)

Glycogen Storage Disease Type 1: Impact of Medium-Chain Triglycerides on Metabolic Control and Growth

Das A.M. · Lücke T. · Meyer U. · Hartmann H. · Illsinger S.
Department of Paediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: 4/1/2009
Accepted: 2/3/2010
Published online: 3/30/2010

Number of Print Pages: 8
Number of Figures: 5
Number of Tables: 2

ISSN: 0250-6807 (Print)
eISSN: 1421-9697 (Online)

For additional information: http://www.karger.com/ANM

Abstract

Background/Objective: Hypoketotic hypoglycaemia and hypertriglyceridaemia are biochemical hallmarks of glycogen storage disease (GSD) 1. Increased malonyl coenzyme A production which compromises oxidation of long-chain fatty acids via carnitine palmitoyltransferase (CPT) 1 inhibition plays a crucial role in the pathogenesis of these complications. Therapy consists primarily of nutritional support including frequent carbohydrate-rich meals. We studied the effect of a diet enriched in medium-chain triglycerides (MCT) on metabolic control/growth in GSD 1 as medium-chain fatty acids can be oxidised independently of CPT 1. Methods: An adult female, a 1.6-year-old boy with GSD 1a and a 6.5-year-old girl with GSD 1b treated with a classical GSD diet were enrolled; their ‘classical GSD diet’ was supplemented with MCT fats. Concentrations of glucose, lactate, ketone bodies triglycerides, uric acid, acylcarnitines in blood and organic acids in urine were determined. Results: No clinical or biochemical side-effects were observed. The MCT diet led to a decrease in uric acid concentrations in all patients. Triglyceride levels were reduced only in the youngest patient, while lactate concentrations did not significantly decrease. The MCT diet allowed for a reduction in carbohydrate and caloric intake required to maintain euglycaemia and led to improvement in growth in the two prepubertal patients. Conclusions: MCT supplementation had a positive effect on metabolic control and growth in our patients suffering from GSD 1.


Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: 4/1/2009
Accepted: 2/3/2010
Published online: 3/30/2010

Number of Print Pages: 8
Number of Figures: 5
Number of Tables: 2

ISSN: 0250-6807 (Print)
eISSN: 1421-9697 (Online)

For additional information: http://www.karger.com/ANM


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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