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Differentiating PFAPA Syndrome From Monogenic Periodic Fevers

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FIGURE 1Flowchart showing the numbers of patients in-cluded in the study. aCriteria for exclusion fromthe study were (1) not fulfilling the inclusioncriteria for periodic fever, (2) lack of completeclinical information, and (3) recruitment andscreening for suspected cryopyrin-related dis-orders only.
TABLE 1 Mutations Found in Patients With Periodic Fever Fulfilling PFAPA Syndrome Criteria
TABLE 2 Prevalence of Main Clinical Manifestations During Fever Episodes for Patients With PFAPA Syndrome-like Phenotype, According to Genotype
FIGURE 2Frequencies of clinical manifestations in the 3 subgroups of patients.
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