Hyperimmunoglobulinemia D and periodic fever syndrome; treatment with etanercept and follow-up
✍ Scribed by Rezan Topaloğlu; Nuray Aktay Ayaz; Hans R. Waterham; Aysel Yüce; Fatma Gumruk; Özden Sanal
- Publisher
- Springer
- Year
- 2008
- Tongue
- English
- Weight
- 83 KB
- Volume
- 27
- Category
- Article
- ISSN
- 0770-3198
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## Abstract We describe for the first time a case of macrophage activation syndrome in a child with hyperimmunoglobulinemia D with periodic fever syndrome who required intensive care support. Up‐regulated monokine production, high serum levels of triglycerides and ferritin, clotting abnormalities w
The hyperimmunoglobulinemia D and periodic fever (hyper-IgD) syndrome is typified by recurrent febrile attacks with abdominal distress, joint involvement (arthralgias/arthritis), headache, skin lesions, and an elevated serum IgD level (> 100 U/ml). This familial disorder has been diagnosed in 56 sub