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Nutrients 2017, 9(11), 1222; doi:10.3390/nu9111222

Nutritional Therapies in Congenital Disorders of Glycosylation (CDG)

1
Metabolic Center, University Hospitals Leuven, B-3000 Leuven, Belgium
2
Department of Development and Regeneration, Faculty of Medicine, KU Leuven, B-3000 Leuven, Belgium
3
Department of Gastroenterology-Hepatology and Metabolic Center, University Hospitals Leuven, B-3000 Leuven, Belgium
4
Hayward Genetics Center, Tulane University School of Medicine, New Orleans, LA 70112, USA
*
Author to whom correspondence should be addressed.
Received: 16 October 2017 / Revised: 1 November 2017 / Accepted: 1 November 2017 / Published: 7 November 2017
(This article belongs to the Special Issue Nutrition and Liver Disease)
View Full-Text   |   Download PDF [225 KB, uploaded 7 November 2017]

Abstract

Congenital disorders of glycosylation (CDG) are a group of more than 130 inborn errors of metabolism affecting N-linked, O-linked protein and lipid-linked glycosylation. The phenotype in CDG patients includes frequent liver involvement, especially the disorders belonging to the N-linked protein glycosylation group. There are only a few treatable CDG. Mannose-Phosphate Isomerase (MPI)-CDG was the first treatable CDG by high dose mannose supplements. Recently, with the successful use of d-galactose in Phosphoglucomutase 1 (PGM1)-CDG, other CDG types have been trialed on galactose and with an increasing number of potential nutritional therapies. Current mini review focuses on therapies in glycosylation disorders affecting liver function and dietary intervention in general in N-linked glycosylation disorders. We also emphasize now the importance of early screening for CDG in patients with mild hepatopathy but also in cholestasis. View Full-Text
Keywords: galactose; mannose; congenital disorders of glycosylation (CDG); treatment; glycosylation; diet galactose; mannose; congenital disorders of glycosylation (CDG); treatment; glycosylation; diet
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Witters, P.; Cassiman, D.; Morava, E. Nutritional Therapies in Congenital Disorders of Glycosylation (CDG). Nutrients 2017, 9, 1222.

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