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MOGS-CDG

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Disease definition

MOGS-CDG is a form of congenital disorders of N-linked glycosylation characterized by generalized hypotonia, craniofacial dysmorphism (prominent occiput, short palpebral fissures, long eyelashes, broad nose, high arched palate , retrognathia), hypoplastic genitalia, seizures, feeding difficulties, hypoventilation, severe hypogammaglobulinemia with generalized edema, and increased resistance to particular viral infections (particularly to enveloped viruses). The disease is caused by loss-of-function mutations in the gene MOGS (2p13.1).

ORPHA:79330

Classification level: Disorder

Synonym(s):
  • CDG syndrome type IIb
  • CDG-IIb
  • CDG2B
  • Carbohydrate deficient glycoprotein syndrome type IIb
  • Congenital disorder of glycosylation type 2b
  • Congenital disorder of glycosylation type IIb
  • Glucosidase 1 deficiency

Source: PubMed ID 10788335 24716661 35790351

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Infancy, Neonatal

ICD-10: E77.8

ICD-11: 5C54.0

OMIM: 606056

UMLS: C1853736

MeSH: C565264

GARD: 10767

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
Disease review articles
Clinical genetics review
English (2017) - GeneReviews
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