Article: Long-Term Follow-Up of Children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome
journal of pediatrics. 2011 Dec., v. 159, no. 6
2011
Abstract: OBJECTIVE: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. STUDY DESIGN: Patients enrolled in a PFAPA registry were contacted and surveyed. RESULTS: Patients in ... ...
| Abstract | OBJECTIVE: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. STUDY DESIGN: Patients enrolled in a PFAPA registry were contacted and surveyed. RESULTS: Patients in the registry (n = 59) were surveyed with a follow-up time ranging from 12 to 21 years. Fifty patients had complete symptom resolution, with mean symptom duration of 6.3 years (95% CI, 5.4-7.3), and no sequelae developed. Nine patients continued to have persistent symptoms for a mean duration of 18.1 years (95% CI, 17.4-18.8). There were no differences in initial presentation between subjects with resolved PFAPA and subjects with persistent PFAPA. In subjects with persistent PFAPA, the mean duration of fever >38.3°C decreased from 3.6 days at onset to 1.8 days at follow-up (P = .01), and the mean symptom-free interval between episodes increased from 29 to 159 days (P < .005). Thirty-seven of 44 patients treated with corticosteroids reported prompt symptom resolution. Twelve patients underwent tonsillectomy or adenotonsillectomy; 9 of these patients experienced markedly reduced symptoms, and 6 patients had resolution of symptoms. Two subjects received other diagnoses. CONCLUSIONS: In long-term follow-up, most patients with PFAPA experienced spontaneous symptom resolution without sequelae. Patients with persistent symptoms had episodes of shorter duration and reduced frequency. |
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| Keywords | adrenal cortex hormones ; children ; complications (disease) ; fever ; patients ; pharyngitis |
| Language | English |
| Dates of publication | 2011-12 |
| Size | p. 958-964. |
| Publishing place | Mosby, Inc. |
| Document type | Article |
| ZDB-ID | 3102-1 |
| ISSN | 1097-6833 ; 0022-3476 |
| ISSN (online) | 1097-6833 |
| ISSN | 0022-3476 |
| DOI | 10.1016/j.jpeds.2011.06.004 |
| Database | NAL-Catalogue (AGRICOLA) |
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