Hyperimmunoglobulinemia D with periodic fever


Engelsk navn: Hyperimmunoglobulinemia D with periodic fever
Engelske synonym: HIDS,Hyper-IgD syndrome,Hyperimmunoglobinemia D with recurrent fever,Hyperimmunoglobulinemia D syndrome,Partial mevalonate kinase deficiency


Definisjon

A rare autoinflammatory disease, and form of mevalonate kinase deficiency (MKD), characterized by periodic attacks of fever and a systemic inflammatory reaction (cervical lymphadenopathy, abdominal pain, vomiting, diarrhea, arthralgia and skin manifestations.

Fra Orphanet

ORPHA: 343
Klassifiseringsnivå: Subtype of disorder
ICD-10: E85.0

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