Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome - PFAPA syndrome.
Presse Med
; 48(1 Pt 2): e77-e87, 2019 Feb.
Article
in En
| MEDLINE
| ID: mdl-30683466
ABSTRACT
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a complex autoinflammatory disease with a clinical phenotype characterised by recurrent episodes of fever, systemic inflammation and symptoms and signs depicted in disease acronym. Although PFAPA is the most common autoinflammatory disease among children in many parts of the world, the condition is still an enigma, which include the regular episodes, the prompt responses to corticosteroids, the genetic bases for the familial clustering and therapeutic effects of tonsillectomy. This review explores PFAPA syndrome with the aim of describing the current clinical and scientific understanding of the condition.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Hereditary Autoinflammatory Diseases
Type of study:
Diagnostic_studies
/
Etiology_studies
/
Prognostic_studies
Limits:
Humans
Language:
En
Journal:
Presse Med
Year:
2019
Document type:
Article