[Tonsillectomy in Cases with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome].
Mariko HaraKeisuke YoshihamaManabu KomoriKae FujiiNoriko MorimotoPublished in: Nihon Jibiinkoka Gakkai kaiho (2018)
The periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory
disease, characterized, as its name suggests, by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis.
This syndrome is the most common cause of recurrent fever in children, however the rate of recognition of this syndrome
is still low. Tonsillectomy has been suggested as an effective treatment, even though the precise, pathophysiology
underlying this syndrome remains unknown. In this study, we investigated the outcomes in patients who underwent tonsillectomy.
In particular, we examined the surgical outcomes and clinical features of the patients who underwent tonsillectomy.
A total of 19 patients with PFAPA syndrome underwent tonsillectomy at our hospital from July 2013 to May
2016. Before the surgery, while all the patients had received medications, none showed complete resolution of the syndromes.
However, of the 19 patients, 15 showed complete resolution of the syndrome immediately after the surgery.
Four patients had fever even after the surgery. Three patients showed partial remission, with the frequency and duration
of the episodes decreasing after the surgery. However, in one patient, the fever persisted as before the surgery. There
were no significant differences in the clinical characteristics, such as the age at onset, fever episodes, associated symptoms,
or age at surgery among the three groups. However, we observed a trend towards a higher frequency of a family
history in patients with persistent symptoms after surgery. Tonsillectomy was highly effective against PFAPA syndrome,
however, some patients failed to respond to the procedure. Therefore, it is important to carefully evaluate the risks and
benefits in each case. The indications for tonsillectomy have not yet been clearly established. It is essential to continue
further investigations to establish effective therapeutic strategies for this syndrome.
Keyphrases
- end stage renal disease
- ejection fraction
- newly diagnosed
- minimally invasive
- chronic kidney disease
- prognostic factors
- case report
- emergency department
- depressive symptoms
- coronary artery disease
- physical activity
- metabolic syndrome
- atrial fibrillation
- insulin resistance
- weight loss
- patient reported outcomes
- sleep quality
- high resolution
- single molecule
- glycemic control