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Peds rheum

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Werner Garavello, Marco Romagnoli, Renato Maria Gaini
OBJECTIVE: To evaluate whether adenotonsillectomy leads to complete resolution in children with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome. STUDY DESIGN: Thirty-nine children with PFAPA syndrome were randomized to either adenotonsillectomy (surgery group; n = 19) or expectant management (control group; n = 20). All patients were then invited prospectively to record all PFAPA episodes, and were evaluated clinically every 3 months for 18 months after randomization...
August 2009: Journal of Pediatrics
Giulia Vigo, Giorgia Martini, Silvia Zoppi, Fabio Vittadello, Francesco Zulian
OBJECTIVES: Tonsillectomy has recently been suggested as an effective treatment for PFAPA syndrome but little is known about its long-term efficacy. We compared the clinical features and the long-term outcome of a large cohort of patients with PFAPA syndrome treated with tonsillectomy or with standard medical treatment. METHODS: We conducted a retrospective study on patients with PFAPA syndrome followed at a tertiary care centre from January 1993 to August 2010...
July 2014: Clinical and Experimental Rheumatology
Stefano Stagi, Federico Bertini, Donato Rigante, Fernanda Falcini
BACKGROUND: The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease characterized by regularly recurrent fever episodes due to seemingly unprovoked inflammation. OBJECTIVE: To assess serum 25-hydroxyvitamin D [25(OH)D] concentrations in children with PFAPA syndrome and evaluate longitudinally the effect of wintertime vitamin D supplementation on the disease course. STUDY DESIGN: We have evaluated 25 Italian patients (19 males, 6 females, aged 2...
June 2014: International Journal of Pediatric Otorhinolaryngology
Marie Cochard, Johanna Clet, Lan Le, Pascal Pillet, Xavier Onrubia, Thierry Guéron, Mohammed Faouzi, Michaël Hofer
OBJECTIVES: To determine whether PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) patients have a positive family history (FH) for recurrent fever syndromes. METHOD: For all patients with PFAPA seen in two paediatric rheumatology centres (Romandy, Switzerland and Bordeaux, France), parents were interviewed to record the FH for periodic fever. As controls, we interviewed a group of children without history of recurrent fever. RESULTS: We recruited 84 patients with PFAPA and 47 healthy children...
October 2010: Rheumatology
Jelena Vojinovic, Rolando Cimaz
Vitamin D, upon its discovery one century ago, was classified as a vitamin. This classification still greatly affects our perception about its biological role. 1,25(OH)2D (now known as the D hormone) is a pleiotropic steroid hormone that has multiple biologic effects. It is integral to the regulation of calcium homeostasis and bone turnover as well as having anti-proliferative, pro-differentiation, anti-bacterial, immunomodulatory and anti-inflammatory properties within the body in various cells and tissues...
May 29, 2015: Pediatric Rheumatology Online Journal
A Katharina Simon, Georg A Hollander, Andrew McMichael
This article reviews the development of the immune response through neonatal, infant and adult life, including pregnancy, ending with the decline in old age. A picture emerges of a child born with an immature, innate and adaptive immune system, which matures and acquires memory as he or she grows. It then goes into decline in old age. These changes are considered alongside the risks of different types of infection, autoimmune disease and malignancy.
December 22, 2015: Proceedings. Biological Sciences
Isabelle Koné-Paut
BD is a systemic inflammatory disease with a variable vasculitis. Paediatric onset is very rare and carries a strong genetic component. Oral ulcers and fever of unknown origin are frequent at onset and difficult to distinguish from other inflammatory disorders; therefore, expert opinion is still mandatory to recognize the disease early. An international expert consensus has recently proposed new classification criteria for children with BD. The clinical spectrum of BD is heterogeneous and influenced by gender, ethnicity and country of residence...
February 18, 2016: Pediatric Rheumatology Online Journal
Federica Vanoni, Katerina Theodoropoulou, Michaël Hofer
The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) is the most common cause of periodic fever in childhood. The current pharmacological treatment includes corticosteroids, which usually are efficacious in the management of fever episodes, colchicine, for the prophylaxis of febrile episodes, and other medication for which efficacy has not been proven so far. Tonsillectomy is an option for selected patients. Usually PFAPA syndrome resolves during adolescence, but there is increasing evidence that this condition may persist into adulthood...
June 27, 2016: Pediatric Rheumatology Online Journal
Youngmin Youn, Jisoo Kim, Young Mi Hong, Sejung Sohn
Forty-three patients with Kawasaki disease who were resistant to initial intravenous immunoglobulin (IVIG) were randomized to receive either a second dose of IVIG (n = 32) or an infliximab (n = 11). With IVIG retreatment 21 patients (65.6%) responded, and with infliximab 10 patients (90.9%) responded. The infliximab group had shorter duration of fever and fewer days of hospitalization. Coronary artery outcomes and adverse events were similar.
April 2016: Pediatric Infectious Disease Journal
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