Nutritional therapies in congenital disorders of glycosylation (CDG)

Peter Witters, David Cassiman, Eva Morava-Kozicz

Research output: Contribution to journalReview article

14 Scopus citations

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.

Original languageEnglish (US)
Article number1222
JournalNutrients
Volume9
Issue number11
DOIs
StatePublished - Nov 1 2017
Externally publishedYes

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Keywords

  • Congenital disorders of glycosylation (CDG)
  • Diet
  • Galactose
  • Glycosylation
  • Mannose
  • Treatment

ASJC Scopus subject areas

  • Food Science
  • Nutrition and Dietetics

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