[PFAPA syndrome: study of 10 cases].
OBJECTIVE
«PFAPA syndrome» is an autoinflammatory entity consisting of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Its etiology is unknown although a dysregulation in the control of the autoinflammatory response seems to play a role. Although a genetic origin is suspected, no specific mutation has been determined yet. Corticosteroids are the mainstay of the treatment during the acute attacks. However, in long-term follow-up the role of tonsillectomy is controversial.


METHODS
A retrospective study of the pediatric cases diagnosed with the PFAPA syndrome was performed in our center during the last 4 years.


RESULTS
Ten patients were diagnosed with the syndrome who received corticosteroids as the only treatment with improvement and favourable prognosis.


CONCLUSIONS
PFAPA syndrome is the most common periodic fever disorder described in childhood whose genetic background has not been yet clarified. Our contribution with 10 patients further supports the common existence of this entity and the need to keep it in mind when having recurrent fevers.
Unidad de Infectología Pediátrica, Departamento de Pediatría, Hospital Universitario de Getafe, Madrid, España. pilarantonmartin@hotmail.com
Pilar
P
Antón-Martín
Roberto
R
Ortiz Movilla
Ana
A
Álvarez García
Sara
S
Guillén Martín
Marta
M
Ruiz Jiménez
José Tomás
JT
Ramos Amador
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D005334:Fever / Q000188:drug therapy / Q000209:etiology*; D056660:Hereditary Autoinflammatory Diseases / Q000188:drug therapy* / Q000453:epidemiology; D006801:Humans; D007223:Infant; D008199:Lymphadenitis / Q000209:etiology*; D008297:Male; D009333:Neck; D010507:Periodicity; D010612:Pharyngitis / Q000209:etiology*; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology; D013281:Stomatitis, Aphthous / Q000188:drug therapy / Q000209:etiology*; D013577:Syndrome
D000305:Adrenal Cortex Hormones