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Since the syndrome was first described in Japan in the late 1960s, thousands of cases have been reported worldwide in diverse racial and ethnic groups, plump ladies although children of Japanese descent have a higher incidence. Several thousand cases plump ladies occur annually in the USA. The male:female ratio is about 1.5:1. Eighty percent of patients are < 5 yr (median, 2 yr); true cases in teenagers or adults are rare. Cases occur year-round, but most often in spring or winter. Clusters have plump ladies been reported in communities without clear evidence of person-to-person spread. Recurrences occur in about 1% of patients. The pathology is nearly identical to infantile periarteritis nodosa, with vasculitis primarily affecting the coronary arteries but also other medium-sized and large arteries. Symptoms, Signs, and Complications The illness tends to progress in stages, beginning with fever, usually remittent and > 39° C (> 102.2° F), which is associated with irritability, often extreme, and occasional lethargy or intermittent colicky abdominal pain.
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