The clinical characteristics of PFAPA syndrome
نویسندگان
چکیده
منابع مشابه
The clinical characteristics of PFAPA syndrome
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 Seru...
متن کاملPW03-009 – Genetics of PFAPA syndrome
Introduction Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an autoinflammatory disorder of childhood and little is known about the underlying etiology. While mutations involving the IL-1 pathway have been identified in other recurrent fever disorders, including TNF-receptor associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS)...
متن کاملEffect of adenotonsillectomy in PFAPA syndrome.
OBJECTIVE To assess the benefits of adenotonsillectomy in the treatment of pediatric patients with PFAPA (periodic fever, aphthous ulcers, pharyngitis, and adenitis) syndrome. DESIGN Prospective case series. SETTING Tertiary care pediatric hospital. PATIENTS Pediatric patients meeting criteria for PFAPA syndrome. INTERVENTION Tonsillectomy with or without adenoidectomy. MAIN OUTCOME M...
متن کاملPFAPA syndrome: is it a family history?
Patients and methods In 2 of the participating centers (Lausanne-Geneva, Switzerland; Bordeaux, France), we questioned all parents during a phone call interview to complete the family history. We used the same questionnaire for a control group from a general pediatric consultation. We asked for positive family history of recurrent fevers, PFAPA and rheumatologic diseases. Patients and controls ...
متن کاملPFAPA syndrome as an hereditary autoinflamatory disorder
Introduction PFAPA syndrome (periodic fever, aphtous stomatitis, pharyngitis, adenitis) is an autoinflammatory disease, for which no genetic marker has been identified yet, and its etiology remains unknown. However, the clinical and biochemical similarities to other autoinflammatory conditions, including Familial Mediterranean Fever (FMF), suggest that a genetic impairment might constitute the ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Pediatric Rheumatology
سال: 2014
ISSN: 1546-0096
DOI: 10.1186/1546-0096-12-s1-p250