PFAPA syndrome

PFAPA syndrome is an autoinflammatory disease of the childhood period presenting with recurrent fever, aphthous stomatitis, pharyngitis and cervical lymphadenitis. The disease is often seen in children under the age of five. Fever attacks recur with irregular time intervals and are unresponsive to treatment with antibiotics and antipyretics. All of the clinical findings resolve immediately with a single dose of corticosteroid. The ultimate treatment method is tonsilloadenoidectomy. Main aspects of PFAPA syndrome are mentioned in this review. (Turk Arch Ped 2009, 44: 80-3

Retrospective analysis of children with uveitis treated with infliximab.

Tumor necrosis factor-alpha (TNF-alpha) antagonists have been successfully used in refractory cases of pediatric uveitis.(1-7) We report our experience with the monoclonal anti-TNF-alpha antibody infliximab in the treatment of uveitis in 20 children. Twenty children with noninfectious uveitis were treated with infliximab as an initial line of immunomodulatory therapy (4 children) or for uncontrolled intraocular inflammation despite aggressive immunosuppressive therapy (16 children). Intraocular inflammation was controlled in the short term in all children; resistance developed in 4 children after long-term therapy

Prevalence of the MEFV gene mutations in childhood polyarteritis nodosa

Objectives To test the hypothesis that alterations in the Mediterranean fever (MEFV) gene area susceptibility factor for the development of polyarteritis nodosa (PAN) we investigated the prevalence of MEFV mutations in patients with PAN without tiny symptoms of familial Mediterranean fever (FMF)

MEFV Mutations Modify the Clinical Presentation of Henoch-Schonlein Purpura

Objective. To investigate the prevalence of MEFV gene mutations in Turkish patients with Henoch-Schonlein purpura (HSP) but with no symptoms of familial Mediterranean fever (FMF). In addition, we assessed the clinical and laboratory characteristics of HSP patients with and without MEFV mutations