Effectiveness of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: a case series

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic high fever (febrile attacks) lasting for 3-6 days and recurring regularly. In this syndrome, fever is associated with aphthous stomatitis, pharyngitis, and cervical adenitis.1-4 Almo...

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Bibliographic Details
Main Authors: Kazuto Taniguchi (Author), Sakumo Kii (Author), Kazuyasu Uemichi (Author)
Format: Book
Published: Indonesian Pediatric Society Publishing House, 2021-04-01T00:00:00Z.
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Summary:Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic high fever (febrile attacks) lasting for 3-6 days and recurring regularly. In this syndrome, fever is associated with aphthous stomatitis, pharyngitis, and cervical adenitis.1-4 Almost all patients with PFAPA syndrome spontaneously resolve approximately 8 years after the onset of symptoms. However, the cause and etiology of PFAPA syndrome remain unclear.4 Oral prednisolone reduces fever during febrile attacks.5-7 Tonsillectomy has also been reported to induce remission of PFAPA syndrome.8-12 However, tonsillectomy is not a popular treatment for PFAPA syndrome because its effectiveness is still unclear. Furthermore, tonsillectomy is generally recommended for patients aged 3 years and older to avoid post-operative complications.13 We report here on four patients aged 3 years and older with PFAPA syndrome. Tonsillectomy completely eliminated febrile attacks and was safely performed without post-operative complications in all of our patients.
Item Description:0030-9311
2338-476X
10.14238/pi61.3.2021.171-4