PGM3-CDG

Orpha code: 443811OMIM code: 615816

Definition

PGM3-CDG is a rare congenital disorder of glycosylation caused by mutations in the <i>PGM3</i> gene and characterized by neonatal to childhood onset of recurrent bacterial and viral infections, inflammatory skin diseases, atopic dermatitis and atopic diatheses, and marked serum IgE elevation. Early neurologic impairment is evident including developmental delay, intellectual disability, ataxia, dysarthria, sensorineural hearing loss, myoclonus and seizures.

Disease data
Classification

Disease

Synonyms
CID due to PGM3 deficiency
CID spowodowany niedoborem PGM3
Wrodzone zaburzenie glikozylazji zależne od PGM3
Złożony zespół niedoboru odporności spowodowany deficytem PGM3
Combined immunodeficiency due to PGM3 deficiency
PGM3-related congenital disorder of glycosylation
ORPHA code
443811
OMIM code
615816
ICD10 code
E77.8
ICD11 code
-

No additional description.

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