Article
A patient with hyper-IgD syndrome responding to anti-TNF treatment.
Clinical rheumatology - 1 Oct 2007
Demirkaya Erkan, Caglar M Kazim, Waterham Hans R, Topaloglu Rezan, Ozen Seza
Abstract excerpt
The hyperimmunoglobulinemia D periodic fever syndrome (HIDS) is caused by recessive mutations in the mevalonate kinase gene, which encodes an enzyme involved in cholesterol and nonsterol isoprenoid biosynthesis. The pathogenesis and treatment remains unclear. We describe a 6-year-old Turkish girl with severe disease. Her clinical features were accompanied with very high acute-phase reactants including a very high...
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