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Congenital Disorders of Glycosylation

Woods, Amanda G. RN, MSN, NNP-BC; Woods, Christopher W. RN, MSN, NNP-BC; Snow, Timothy M. RN, MSN, NNP-BC

Section Editor(s): Zukowsky, Ksenia

doi: 10.1097/ANC.0b013e318241cb20
Beyond the Basics

Congenital disorders of glycosylation (CDG) are a group of disorders involving a defect in the synthesis of oligosaccharides. Oligosaccharides are fundamental for protein stability and cellular communication and are present in almost every cell in the human body. A defect in the synthesis of oligosaccharides can result in multisystemic effects. Congenital disorders of glycosylation are classified into type I and type II disorders, each with subgroup classifications. All CDGs are autosomal recessive disorders, with CDG type I being the most common. This article will explore both types of CDG, their clinical presentation, diagnosis, and management.

Women's Hospital, Greensboro, North Carolina (Mrs Woods); and Wake Forest University Baptist Medical Center, Winston Salem, North Carolina (Messrs Woods and Snow).

Correspondence: Amanda G. Woods, RN, MSN, NNP-BC, Women's Hospital of Greensboro, 801 Green Valley Rd, Greensboro, NC 27408 (

The authors declare no conflict of interest.

© 2012 National Association of Neonatal Nurses