Literature DB >> 32239314

Long-term management of patients with PFAPA syndrome.

Erkan Yıldız1, Selçuk Kuzu2, Orhan Kemal Kahveci2, Şahin Ulu2, Abdulkadir Bucak2.   

Abstract

OBJECTIVE: To evaluate the long-term treatment results of patients with PFAPA syndrome and to determine their need for tonsillectomy.
MATERIALS AND METHODS: The clinical characteristics, treatments and long-term results of 16 patients admitted to the Pediatric and Otorhinolaryngology Clinic between 2015 and 2019 were retrospectively analyzed.
RESULTS: Twelve male and four female patients were examined between 1.5 and 8 years (mean age 4.8 ± 1.1) (75% male, 25% female). The mean duration of attacks was 4.4 ± 1.4 weeks. Twelve patients recovered completely with single-dose steroid therapy. In the 4-year follow-up of 12 patients who were given a single dose of steroid therapy, there were no relapses. Surgical decisions were made for four patients whose attacks did not pass with steroid treatment. Two patients (75%) underwent adenotonsillectomy, and one patient underwent tonsillectomy While three of these patients did not have an attack again, one patient continued to have an attack every 8 weeks. At the age of 9, his attacks were completely resolved spontaneously. All patients had fever. The most common concomitant symptoms were pharyngitis (94%), cervical adenitis (82%) and aphthous stomatitis (77%). Exudative tonsillitis was present in 25% of the patients.
CONCLUSION: Medical treatment should be the first treatment option in patients with PFAPA syndrome. Although single-dose steroid treatment is effective in these patients, tonsillectomy is an important treatment option in refractory cases.

Entities:  

Keywords:  PFAPA syndrome; Periodic fever; Steroid; Tonsillectomy

Mesh:

Year:  2020        PMID: 32239314     DOI: 10.1007/s00405-020-05943-7

Source DB:  PubMed          Journal:  Eur Arch Otorhinolaryngol        ISSN: 0937-4477            Impact factor:   2.503


  2 in total

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Journal:  Front Pediatr       Date:  2021-11-25       Impact factor: 3.418

Review 2.  Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries-A decade of experience from North India.

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Journal:  Front Immunol       Date:  2022-09-20       Impact factor: 8.786

  2 in total

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