Outcome of Tonsillectomy in Selected Patients With PFAPA Syndrome

Abstract

Otolaryngologists should be trained to recognize PFAPA syndrome, for which management consists of a regular and prolonged second-level pediatric and otolaryngological follow-up, with surgery only after the failure of traditional medical therapy.

Polyclonal, newly derived T cells with low expression of inhibitory molecule PD-1 in tonsils define the phenotype of lymphocytes in children with Periodic Fever, Aphtous Stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome

“…Our case series is similar to others in the literature regarding most frequent clinical symptoms (Feder and Salazar, 2010 Feb;Marshall et al, 1987;Tasher et al, 2006). A recurrence of aphthous stomatitis in PFAPA patients after surgery has been previously described in some studies (Valenzuela et al, 2013;Pignataro et al, 2009;Vigo et al, 2014), but this was not a finding of our study. The median age at surgery in PFAPA patients was also similar to that reported in other series (Feder and Salazar, 2010;Valenzuela et al, 2013).…”

“…The median age at surgery in PFAPA patients was also similar to that reported in other series (Feder and Salazar, 2010;Valenzuela et al, 2013). In line with previously published findings, the histology results of all of the PFAPA tonsils corresponded with the signs of chronic tonsillar inflammation, and the PFAPA tonsils cultivation examinations were negative for bacteria in all patients (Peridis et al, 2010b;Pignataro et al, 2009). The optimal treatment of PFAPA syndrome remains a matter of a debate.…”

Microbes of the tonsils in PFAPA (Periodic Fever, Aphtous stomatitis, Pharyngitis and Adenitis) syndrome - a possible trigger of febrile episodes

“…In one previous case series of nine PFAPA patients who underwent TE, atypical bacteria and respiratory viruses were searched by molecular analyses from the removed tonsil tissue. Only one sample there showed positive result yielding adenovirus . One limitation of our study was that the controls were slightly older than the PFAPA cases.…”

Measles Lymphadenopathy in a Child With PFAPA Syndrome

“…24,25 Molecular studies assessing adenovirus, human herpesvirus 6, and enterovirus have shown no significant difference in viral presence between PFAPA and non-PFAPA controls. 1,13,16,18 The lack of an identifiable trigger may reflect that the mild immune defect likely underlying PFAPA may be triggered by a multiple entities (infection, stress, and immunizations), similar to what has been proposed for Kawasaki disease. 26…”