Combined immunodeficiency due to GINS1 deficiency

Orpha code: 505227OMIM code: 617827

Definition

A rare syndrome with combined immunodeficiency characterized by intrauterine and postnatal growth retardation, chronic neutropenia, and natural killer (NK) cell deficiency due a defect in DNA replication leading to blockade of immune cell differentiation in the bone marrow, particularly affecting NK cells. Other clinical features include recurrent viral and bacterial infections and eczema, as well as mild facial dysmorphism.

Disease data
Classification

Disease

Synonyms
CID due to GINS1 deficiency
Combined immunodeficiency with intrauterine growth retardation-NK cell deficiency-neutropenia
Combined immunodeficiency with intrauterine growth retardation-natural killer cell deficiency-neutropenia
CID due to GINS1 deficiency
Combined immunodeficiency with intrauterine growth retardation-NK cell deficiency-neutropenia
Combined immunodeficiency with intrauterine growth retardation-natural killer cell deficiency-neutropenia
ORPHA code
505227
OMIM code
617827
ICD10 code
D81.8
ICD11 code
-

No additional description.

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