137 research outputs found

    NOD2/CARD 15 gene mutations in patients with Familial Mediterranean Fever

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    Anesthesia for intra-articular corticosteroid injections in juvenile idiopathic arthritis: A survey of pediatric rheumatologists

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    <p>Abstract</p> <p>Objective</p> <p>To determine the methods of anesthesia currently being used by pediatric rheumatologists when performing intra-articular corticosteroid injections (IACI).</p> <p>Study design</p> <p>A questionnaire was emailed to all members of the Childhood Arthritis & Rheumatology Research Alliance, a pediatric rheumatology research network in North America. The questionnaire consisted of 11 questions ranging from procedure technique, treatments prescribed for topical anesthesia and oral analgesia, and factors that might affect procedural pain.</p> <p>Results</p> <p>Seventy-four of 161 physicians (46%) responded to the questionnaire. On average, each physician injected 33 children (median 25, range 1-160) and 43 joints (median 30, range 1-150) yearly. Local anesthesia was used in children on average ≥ 8 years (range 2-16 years), with general anesthesia being more frequently used for younger children. All respondents used local anesthesia. The most commonly used methods of local anesthesia were EMLA<sup>® </sup>cream plus subcutaneous lidocaine (58.8%), ethyl chloride spray only (39.7%), EMLA<sup>® </sup>cream only (33.8%), subcutaneous lidocaine only (25%), and lidocaine iontophoresis only (11.8%). Buffering of the lidocaine was routinely done only 7.4% of the time.</p> <p>Conclusion</p> <p>Although pediatric rheumatologists in North America perform IACI on a large number of patients each year, a wide variety of methods are used to deliver local anesthesia with no accepted standard of care. More studies are needed to determine the optimal method of local anesthesia delivery to minimize pain associated with IACI.</p

    P01-018 – An earliest diagnosis of FMF

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    Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome

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    <p>Abstract</p> <p>Background</p> <p>This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous <b>s</b>tomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease.</p> <p>Methods</p> <p>A cohort of patients with a median age of 4.9 years experiencing 'typical PFAPA' episodes participated in this study. Blood cells and serum cytokines were analyzed by CBC analysis and multiplex ELISA.</p> <p>Results</p> <p>Oscillations in the concentration of blood cells during the afebrile and febrile phases of typical PFAPA syndrome were observed; novel findings include increased monocytes and decreased eosinophils during a febrile episode and increased thrombocytes in the afebrile interval. Relatively modest levels of pro-inflammatory cytokines were present in sera. IFNγ-induced cytokine IP10/CXCL10 was increased after the onset of fever while T cell-associated cytokines IL7 and IL17 were suppressed during afebrile and febrile periods.</p> <p>Conclusions</p> <p>Identification of dysregulated blood cells and serum cytokines is an initial step towards the identification of biomarkers of PFAPA disease and/or players in disease pathogenesis. Future investigations are required to conclusively discern which mediators are associated specifically with PFAPA syndrome.</p

    Periodic fever syndromes in Eastern and Central European countries: results of a pediatric multinational survey

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    <p>Abstract</p> <p>Objective</p> <p>To analyze the prevalence of diagnosed and suspected autoinflammatory diseases in Eastern and Central European (ECE) countries, with a particular interest on the diagnostic facilities in these countries.</p> <p>Methods</p> <p>Two different strategies were used to collect data on patients with periodic fever syndromes from ECE countries- the Eurofever survey and collection of data with the structured questionnaire.</p> <p>Results</p> <p>Data from 35 centers in 14 ECE countries were collected. All together there were 11 patients reported with genetically confirmed familial Mediterranean fever (FMF), 14 with mevalonate-kinase deficiency (MKD), 11 with tumor necrosis factor receptor associated periodic syndrome (TRAPS) and 4 with chronic infantile neurological cutaneous and articular syndrome (CINCA). Significantly higher numbers were reported for suspected cases which were not genetically tested. All together there were 49 suspected FMF patients reported, 24 MKD, 16 TRAPS, 7 CINCA and 2 suspected Muckle-Wells syndrome (MWS) patients.</p> <p>Conclusions</p> <p>The number of genetically confirmed patients with periodic fever syndromes in ECE countries is very low. In order to identify more patients in the future, it is important to organize educational programs for increasing the knowledge on these diseases and to establish a network for genetic testing of periodic fever syndromes in ECE countries.</p
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