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김상옥; Sang Ock Kim; 형건덕; Chien Ter Hsing; 한지선; Ji Sun Han; 김병근; Byung Geun Kim; 김하연; Ha Yeon Kim; 정원태; Won Tae Chung; 이성원; Sung Won Lee
The Korean journal of medicine, 12/2009, Letnik: 77, Številka: 7Journal Article
PFAPA증후군은 주기적인 발열을 특징으로 하는 질환군으로 면역학적 조절이상이 그 원인으로 생각되어지고 있고, 소아에서 주로 보고되고 있으나 성인에서도 원인을 알 수 없는 주기적인 발열소견이 보일 때 의심해 보아야 할 질환이다. 이에 저저들은 성인에서 발생한 PFAPA증후군을 진단하고 치료에 반응을 보인 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다. In 1987, Marshall et al. described a new periodic fever that was later named periodic fever, aphthous ulcers, pharyngitis, and cervical adenitis (PFAPA) syndrome. Other periodic fever diseases, such as recurrent tonsillitis, a number of infectious diseases, juvenile idiopathic arthritis, Behcet`s disease, cyclic neutropenia, familial Mediterranean fever (FMF), familial Hibernian fever (FHF), and hyperglobulinema D syndrome must be excluded as diagnoses. The diagnostic criteria were proposed in 1989 and modified 10 years later. Adult onset PFAPA syndrome has rarely been reported and has not been reported in Korea. We diagnosed PFAPA syndrome with characteristic clinical findings in a 50-year-old man, who showed good response to steroid therapy. (Korean J Med 77:S1332-S1336, 2009)
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