The clinical characteristics of PFAPA syndrome
نویسنده
چکیده
Results Individual episodes of fever usually resolved with a single intramuscular 1 mg/kg dose of methyl prednisolone. The mean fever resolve duration during the febril episode was 1.64 hours. FMF gene was investigated in 109 and we determined heterozygot MEFV gene mutations in 19 of our patients (17%). At the onset of febril episodes the mean CRP value was 6.09±8.48 mg/dl (1-38), the mean Serum Amiloid A value was 240 mg/L (2,7-1900), Prokalsitonin values were negative. Our testing included at least a CBC at the onset of febril episodes (the mean WBC: 13150/mm3, range 4500-26.400 cells/mm3) with a preponderance of neutrophils. IgM levels were low in the 5 patients, IgA levels were low in the 6 patients and IgG levels were low 11 patients according to their healthy age matched controls. PFAPA patients had normal CRP and Procalsitonin levels between febril episodes. A total of 89 patients were treated with colchicine. The mean interval between episodes was statistically prolonged in patients who were on prophylactic colchicine therapy.
منابع مشابه
Periodic Fever accompanied by Aphthous stomatitis, Pharyngitis, and cervical Adenitis syndrome (PFAPA syndrome) in adults
BACKGROUND The new syndrome, known as PFAPA, of periodic fever characterized by abrupt onset of fever, malaise, aphthous stomatitis, tonsillitis, pharyngitis and cervical adenopathy has been described only in pediatric patients. It usually begins before the age of 5 years and in most cases resolves spontaneously before age 10. OBJECTIVES To describe a series of adults with PFAPA syndrome. M...
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