Format

Send to

Choose Destination
Nutrients. 2017 Nov 7;9(11). pii: E1222. doi: 10.3390/nu9111222.

Nutritional Therapies in Congenital Disorders of Glycosylation (CDG).

Author information

1
Metabolic Center, University Hospitals Leuven, B-3000 Leuven, Belgium. peter.witters@uzleuven.be.
2
Department of Development and Regeneration, Faculty of Medicine, KU Leuven, B-3000 Leuven, Belgium. peter.witters@uzleuven.be.
3
Department of Gastroenterology-Hepatology and Metabolic Center, University Hospitals Leuven, B-3000 Leuven, Belgium. david.cassiman@kuleuven.be.
4
Metabolic Center, University Hospitals Leuven, B-3000 Leuven, Belgium. emoravakozicz@tulane.edu.
5
Department of Development and Regeneration, Faculty of Medicine, KU Leuven, B-3000 Leuven, Belgium. emoravakozicz@tulane.edu.
6
Hayward Genetics Center, Tulane University School of Medicine, New Orleans, LA 70112, USA. emoravakozicz@tulane.edu.

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.

KEYWORDS:

congenital disorders of glycosylation (CDG); diet; galactose; glycosylation; mannose; treatment

PMID:
29112118
PMCID:
PMC5707694
DOI:
10.3390/nu9111222
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Multidisciplinary Digital Publishing Institute (MDPI) Icon for PubMed Central
Loading ...
Support Center