Periodic fever syndrome in children☆,☆☆
Section snippets
METHODS
Patients were included in this study if they were evaluated by 1 of the authors or if there was telephone consultation with either parents or physicians of a child with periodic fevers. A PFAPA Registry form requested demographic information, birth and family history, characteristics of the febrile pattern, and associated symptoms. Medical records on each child were reviewed for clinical and laboratory data.
To determine the status of the febrile episodes and associated findings in these
Initial PFAPA Registry Patients
A total of 176 PFAPA Registry forms were returned to the authors. Of this number, 94 patients were identified with well-documented, regularly recurring periodic febrile episodes; 77 patients were excluded because the registry information did not clearly document the periodicity of the fever with asymptomatic intervening intervals. Five patients were excluded because they had diagnoses of other causes for periodic fevers including cyclic neutropenia (3 patients), Behcet syndrome (1 patient), and
DISCUSSION
The earliest reports of periodic fevers were presented in the 1940s by Reimann7 and Reimann and deBarardini.8 Using the term periodic to mean episodes recurring at regular intervals, Reimann described a group of patients with periodic fever beginning in infancy, persisting for years to decades, having cycles of similar duration, and a generally benign course.9, 10, 11 In 1974 he described an otherwise healthy man with high fever of 5 days’ duration, recurring at intervals of 20 to 23 days for 8
Acknowledgements
We are grateful for the assistance received from the multiple referring physicians and parents of the patients described.
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