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When a patient complains of episodic fever accompanied by unexplained arthritis, peritonitis, pleurisy, or skin rash, this disorder should be considered. The disease-related gene codes for a protein that guides a neutrophil's participation in inflammation; the protein's existence implies an entire regulatory pathway hitherto unknown. At least two other mendelian periodic fever syndromes have also been described.
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Daniel L. Kastner (1998) studied this question.
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