Fever is one of the leading symptom in childhood diseases. If there are recurrent febrile episodes, one have to distinguish between recurrent infections in the normal age range, periodic fever syndromes or even a primary immunodeficiency. Diagnostic tools to distinguish one from another are reported. The warning signs for primary immunodeficiency are listed and discussed. For periodic fever syndromes, the PFAPA syndrome, the most frequent in Swiss children, is discussed in some detail.
Fever is one of the main symptoms leading to medical evaluation. Not only infections cause fever but also inflammatory disorders. To distinguish one from another, a thorough medical history and clinical evaluation are needed. Sometimes, only the clinical course will reveal the diagnosis. PFAPA-Syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis) is the most frequent periodic fever syndrome in Switzerland. No diagnostic test is available to support the diagnosis. Some important diseases have to be ruled out, such as Immunodeficiency, cyclic neutropenia, chronic viral infections and rheumatologic disorders. To know the diagnosis of the PFAPA-Syndrome can help avoiding antibiotic courses for febrile episodes in infants. There is a clinical overlap to hereditary periodic fever syndromes as familial Mediterranean fever (FMF), Hyper-IgD and fever syndrome (HIDS), Tumor-necrosis factor receptor associated periodic syndrome (TRAPS) and others, in which a genetic basis for the disease has already been found.
