Arthritis and rheumatism
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To determine factors associated with, and the predictors of, widespread body pain in children. ⋯ Children who report behavioral problems or other somatic symptoms are at increased risk, at least in the short term, of developing chronic widespread pain. It remains to be determined whether these are long-term risk factors for the onset of such symptoms, and whether they could be predictors of the development of other functional syndromes.
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Arthritis and rheumatism · Sep 2003
Case ReportsFavorable preliminary experience with etanercept in two patients with the hyperimmunoglobulinemia D and periodic fever syndrome.
The hyperimmunoglobulinemia D and periodic fever syndrome (HIDS; MIM 260920) is caused by recessive mutations in the mevalonate kinase gene (MVK), which encodes an enzyme involved in cholesterol and nonsterol isoprenoid biosynthesis. HIDS is characterized by persistently elevated polyclonal IgD and recurrent febrile episodes. Although abnormalities in tumor necrosis factor alpha (TNF alpha) are not the primary cause of HIDS, plasma TNF alpha levels are elevated in HIDS patients during attacks and thus may be a therapeutic target. This study assessed the effects of etanercept, a soluble p75 TNF alpha receptor-Fc fusion protein, in 2 patients with HIDS. ⋯ Our favorable experience with etanercept for the treatment of HIDS suggests that further investigation of this therapy is warranted.
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Arthritis and rheumatism · Sep 2003
Heterogeneity among patients with tumor necrosis factor receptor-associated periodic syndrome phenotypes.
To investigate the prevalence of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) among outpatients presenting with recurrent fevers and clinical features consistent with TRAPS. ⋯ The presence of TNFRSF1A shedding defects and low sTNFRSF1A levels in 3 families without a TNFRSF1A mutation indicates that the genetic basis among patients with "TRAPS-like" features is heterogeneous. TNFRSF1A mutations are not commonly associated with nonfamilial recurrent fevers of unknown etiology.