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Syndrome of periodic fever, pharyngitis, and aphthous stomatitis
Insights
A rare periodic fever syndrome in children presents with recurrent fevers and flu-like symptoms every 4-6 weeks. Attacks resolve spontaneously, and prednisone may shorten episodes.
Area of Science:
- Pediatrics
- Immunology
- Genetics
Background:
- A periodic fever syndrome with clinical similarities to cyclic neutropenia has been identified in pediatric patients.
- This syndrome is characterized by recurrent episodes of fever and associated symptoms.
Purpose of the Study:
- To describe the clinical presentation, laboratory findings, and treatment response of a periodic fever syndrome in children.
- To differentiate this syndrome from cyclic neutropenia and assess its prevalence.
Main Methods:
- Retrospective case series analysis of 12 children diagnosed with the syndrome.
- Clinical observation and review of laboratory data during febrile episodes.
- Evaluation of treatment response to prednisone and nonsteroidal anti-inflammatory agents.
Main Results:
- The syndrome presents with recurrent fevers, malaise, chills, aphthous stomatitis, pharyngitis, headache, and cervical adenopathy every 4-6 weeks.
- Episodes resolve spontaneously within 4-5 days, with mild leukocytosis and elevated ESR during attacks.
- Children experience normal growth, no increased susceptibility to infection, and no long-term sequelae. Attacks may be aborted by prednisone but not NSAIDs.
Conclusions:
- This periodic fever syndrome is a distinct clinical entity in children, presenting sporadically.
- The syndrome is characterized by recurrent, self-limiting febrile episodes with specific associated symptoms.
- Prednisone shows efficacy in aborting attacks, suggesting an inflammatory or immune basis.
Abstract:
A syndrome of periodic fever that resembles human cyclic neutropenia in its clinical presentation has been identified in 12 children observed at two major referral centers. Attacks characterized by abrupt onset of fever, malaise, chills, aphthous stomatitis, pharyngitis, headache, and tender cervical adenopathy occur at 4- to 6-week intervals over periods of years. These episodes of illness resolve spontaneously in 4 to 5 days. Mild leukocytosis and elevation of the erythrocyte sedimentation rate during attacks are the only laboratory abnormalities. Affected children grow normally, are not unusually susceptible to infection, and exhibit no long-term sequelae. Attacks may be aborted by short courses of prednisone but do not respond to nonsteroidal anti-inflammatory agents. This syndrome is sporadic and appears to be much more common than cyclic neutropenia.