hyperimmunoglobulinemia D syndrome


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hyperimmunoglobulinemia D syndrome (HIDS)

a periodic fever inherited as an autosomal-recessive trait, caused by mutations in the gene for mevalonate kinase and having onset usually before 1 year of age. It is characterized by attacks of high fever preceded by chills, occurring at intervals of approximately 4 to 8 weeks and lasting 4 to 6 days, often accompanied by headache, arthritis and arthralgia, erythematous lesions, and hepatosplenomegaly. Serum IgD levels are continuously high. Also called Dutch type periodic fever.