autosomaal recessieve hereditaire aandoening
gecombineerde immunodeficiëntie
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primaire immunodeficiëntie met afwijkende lymfoïde immuniteit door meerdere factoren (aandoening)
primaire immunodeficiëntie met afwijkende lymfoïde immuniteit door meerdere factoren
NIK-deficiëntie
Primary immunodeficiency with multifaceted aberrant lymphoid immunity
NF-kappa-B-inducing kinase deficiency
NIK deficiency
A rare, genetic, primary combined T and B cell immunodeficiency characterized by recurrent, severe viral and bacterial infections. Immunologic findings include decreased immunoglobulin levels, decreased numbers of B and NK cells, reduced relative CD19+ B cells in peripheral blood, impaired memory responses to viral infections and defective antigen-specific T-cell proliferation.
Id1197478005
StatusPrimitive
Pathological processafwijkend immuunproces
SNOMED CT to Orphanet simple map447731
SNOMED CT to ICD-10 extended map
TargetD81.8
RuleTRUE
AdviceALWAYS D81.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified