105 ㈣ ㈤ 1.5 Kuo et al., 2009 C SGPT SGOT GGT Newburger et al., 2004 atypical or incomplete KD % Wang et al., 2005 American Heart Association,

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1 104 非 典 型 川 崎 症 之 文 獻 回 顧 及 臨 床 照 護 劉 又 禎 1 侯 秋 萍 1 郭 秋 敏 2 梁 啟 迪 3 郭 和 昌 4* 摘 要 : 川 崎 症 是 一 個 病 因 未 知 的 全 身 性 血 管 炎 疾 病, 且 主 要 侵 犯 五 歲 以 下 的 兒 童, 也 是 造 成 兒 童 後 天 性 心 臟 病 的 主 因 川 崎 症 的 致 病 因 基 因 和 免 疫 致 病 機 轉 到 目 前 都 尚 未 釐 清 尤 其 是 非 典 型 川 崎 症 的 診 斷 與 護 理 照 護 對 於 臨 床 醫 護 人 員 更 是 一 大 挑 戰 ; 本 篇 報 告 是 非 典 型 川 崎 症 之 文 獻 回 顧 及 臨 床 照 護 簡 介, 內 容 包 含 川 崎 症 之 致 病 原 因 臨 床 表 徵 與 非 典 型 川 崎 症 的 臨 床 表 現 鑑 別 診 斷 治 療 及 疾 病 與 居 家 護 理 希 望 能 對 川 崎 症 及 非 典 型 川 崎 症 患 童 的 診 斷 與 醫 療 照 護 過 程 有 所 幫 助 關 鍵 詞 : 川 崎 症 非 典 型 川 崎 症 冠 狀 動 脈 病 變 護 理 照 護 Kawasaki disease, KD 1967 Tomisaku KawasakiKuo et al., Huang et al., 2009 Anderson, Todd, & Glode, 2005 Burns & Glode, ~2 Wang, Wu, Liu, Kuo, & Yang, 2005 Wang et al., 2005 Esper et al., 2005human coronavirus Lehmann et al., 2009Chang et al., 2006 Kuo et al., 2009; Liang, Kuo, Yang, Wang, & Ko, Kuo et al., 2009 ㈠ ㈡ ㈢ *

2 105 ㈣ ㈤ 1.5 Kuo et al., 2009 C SGPT SGOT GGT Newburger et al., 2004 atypical or incomplete KD % Wang et al., 2005 American Heart Association, AHA Newburger et al., 2004 Wang et al., gm/kg/dose Kawasaki disease Toxic shock syndrome Streptococcal scarlet fever Stevens-Johnson syndrome Systemic type- Juvenile Rheumatoid Arthritis < 5 > < 10 < 10 20% A 30 75% 30 50%

3 106 a b Albumin 3.0 g/dl, anemia for age, elevation of alanine aminotransferase, platelets after 7 days 450,000/mm 3, white blood cell count 15,000/mm 3, and urine 10 white blood cells/high-power field Diagnosis, treatment, and long-term management of Kawasaki disease: A statement for health professionals from the committee on rheumatic fever, endocarditis, and Kawasaki disease, council on cardiovascular disease in the young, American Heart Association. Newburger2004 American Academy of Pediatrics Intravenous immunoglobulin, IVIG Kuo, Wang,

4 107 Wang, Yu, & Yang, 2008; Kuo, Yang, et al., 2007; Yu et al., 2009 Kuo 3% Kuo, Yang, et al., 2007 Kuo et al., %~38% Tremoulet et al., % 1~2g/kg/dose 3~4% Kuo et al., 2009 Aspirin; 80~100mg/kg/day 3~5mg/kg/day 6~8 3~5mg/kg/day Hsieh et al., 2004; Kuo et al., ㈠ ㈡ ㈢ 2007 ㈠ 2gm/kg/day, mg/kg/day 5ml Kuo, Wang, et al., 2007; Kuo, Yang, et al., 2007 ㈡ aspirin G6PD glucose-6- phosphate dehydrogenase deficiency, Pickering, Reye s syndromewei et al., 2005 American Academy of Pediatrics, AAP Pickering, 2009

5 108 warfarin warfarin cranberry Paeng, Sprague, & Jackevicius, 2007 ㈢ ~8 Newburger et al., Liang et al., 2009; Wang et al., [Wu, S. Y., & Hsu, Y. Y. (2007). Circulatory diseases and their care in children. In Y. C. Chen (Ed.), Practical pediatric nursing (5th ed., pp ). Taipei, Taiwan, ROC: Farseeing.] [Weng, P. H., Lu, H. J., & Liang, H. F. (2008). A nurse s experience with a Kawasaki syndrome child and his caregiver. Chang Gung Nursing, 19(2), ] [Chang, L. Y., & Tsai, L. J. (2007). The reaction of children to hospitalization and its care. In Y. C. Chen (Ed.), Practical pediatric nursing (5th ed., pp ). Taipei, Taiwan, ROC: Farseeing.] Anderson, M. S., Todd, J. K., & Glode, M. P. (2005). Delayed

6 109 diagnosis of Kawasaki syndrome: An analysis of the problem. Pediatrics, 115(4), e428 e433. Burns, J. C., & Glode, M. P. (2004). Kawasaki syndrome. Lancet, 364(9433), Chang, L. Y., Chiang, B. L., Kao, C. L., Wu, M. H., Chen, P. J., Berkhout, B., et al. (2006). Lack of association between infection with a novel human coronavirus (HCoV), HCoV- NH, and Kawasaki disease in Taiwan. Journal of Infectious Diseases, 193(2), Esper, F., Shapiro, E. D., Weibel, C., Ferguson, D., Landry, M. L., & Kahn, J. S. (2005). Association between a novel human coronavirus and Kawasaki disease. Journal of Infectious Diseases, 191(4), Hsieh, K. S., Weng, K. P., Lin, C. C., Huang, T. C., Lee, C. L., & Huang, S. M. (2004). Treatment of acute Kawasaki disease: Aspirin s role in the febrile stage revisited. Pediatrics, 114(6), e689 e693. Huang, W. C., Huang, L. M., Chang, I. S., Chang, L. Y., Chiang, B. L., Chen, P. J., et al. (2009). Epidemiologic features of Kawasaki disease in Taiwan, Pediatrics, 123(3), e401 e405. Kuo, H. C., Wang, C. L., Liang, C. D., Yu, H. R., Chen, H. H., Wang, L., et al. (2007). Persistent monocytosis after intravenous immunoglobulin therapy correlated with the development of coronary artery lesions in patients with Kawasaki disease. Journal of Microbiology, Immunology and Infection, 40(5), Kuo, H. C., Wang, C. L., Liang, C. D., Yu, H. R., Huang, C. F., Wang, L., et al. (2009). Association of lower eosinophilrelated T helper 2 (Th2) cytokines with coronary artery lesions in Kawasaki disease. Pediatric Allergy and Immunology, 20(3), Kuo, H. C., Wang, C. L., Wang, L., Yu, H. R., & Yang, K. D. (2008). Patient characteristics and intravenous immunoglobulin product may affect eosinophils in Kawasaki disease. Pediatric Allergy and Immunology, 19(2), Kuo, H. C., Yang, K. D., Liang, C. D., Bong, C. N., Yu, H. R., Wang, L., et al. (2007). The relationship of eosinophilia to intravenous immunoglobulin treatment failure in Kawasaki disease. Pediatric Allergy and Immunology, 18(4), Lehmann, C., Klar, R., Lindner, J., Lindner, P., Wolf, H., & Gerling, S. (2009). Kawasaki disease lacks association with human coronavirus NL63 and human bocavirus. Pediatric Infectious Disease Journal, 28(6), Liang, C. D., Kuo, H. C., Yang, K. D., Wang, C. L., & Ko, S. F. (2009). Coronary artery fistula associated with Kawasaki disease. American Heart Journal, 157(3), Newburger, J. W., Takahashi, M., Gerber, M. A., Gewitz, M. H., Tani, L. Y., Burns, J. C., et al. (2004). Diagnosis, treatment, and long-term management of Kawasaki disease: A statement for health professionals from the committee on rheumatic fever, endocarditis, and Kawasaki disease, council on cardiovascular disease in the young, American Heart Association. Pediatrics, 114(6), Paeng, C. H., Sprague, M., & Jackevicius, C. A. (2007). Interaction between warfarin and cranberry juice. Clinical Therapeutics, 29(8), Pickering, L. K. (Ed.) (2009). Red book: Report of the committee on infectious diseases (pp ). Chicago: American Academy of Pediatrics. Tremoulet, A. H., Best, B. M., Song, S., Wang, S., Corinaldesi, E., Eichenfield, J. R., et al. (2008). Resistance to intravenous immunoglobulin in children with Kawasaki disease. The Journal of Pediatrics, 153(1), Wang, C. L., Wu, Y. T., Liu, C. A., Kuo, H. C., & Yang, K. D. (2005). Kawasaki disease: Infection, immunity and genetics. Pediatric Infectious Disease Journal, 24(11), Wei, C. M., Chen, H. L., Lee, P. I., Chen, C. M., Ma, C. Y., & Hwu, W. L. (2005). Reye s syndrome developing in an infant on treatment of Kawasaki syndrome. Journal of Pediatrics and Child Health, 41(5-6), Yu, H. R., Kuo, H. C., Sheen, J. M., Wang, L., Lin, I. C., Wang, C. L., et al. (2009). A unique plasma proteomic profiling with imbalanced fibrinogen cascade in patients with Kawasaki disease. Pediatric Allergy and Immunology, 20(7),

7 110 Atypical Kawasaki Disease: Literature Review and Clinical Nursing Yu-Chen Liu 1 Chiu-Ping Hou 1 Chiu-Ming Kuo 2 Chi-Di Liang 3 Ho-Chang Kuo 4 * Abstract: Kawasaki disease (KD) is an acute febrile multi-systemic vasculitis of unknown etiology that primarily affects children under 5 years of age. KD has been singled out as a main cause of acquired childhood heart disease. Its etiology, genetic background, and immunopathogenesis remain unclear. Diagnosing and providing nursing care to KD patients, especially those suffering from atypical KD, present a challenge for clinicians and nurses. This report is a literature review covering pathogenesis, clinical presentation, atypical symptoms, differential diagnosis, treatment and nursing of KD and atypical KD. This review provides updated information for clinicians and nurses with care responsibilities for patients with KD and atypical KD. Key Words: Kawasaki disease, atypical Kawasaki disease, coronary artery lesion, nursing. 1 RN, NP, Department of Nursing and Pediatric Ward, Chang Gung Memorial Hospital-Kaohsiung Medical Center; 2 RN, Department of Nursing and Operation Room, Chang Gung Memorial Hospital-Chiayi Branch; 3 MD, Chief, Department of Pediatrics, Chang Gung Memorial Hospital-Kaohsiung Medical Center; 4 MD, Assistant Professor & Visiting Staff, Chang Gung Memorial Hospital-Kaohsiung Medical Center; Lecturer, Graduate Institute of Clinical Medical Science, Chang Gung University. Received: December 24, 2009 Revised: March 18, 2010 Accepted: August 9, 2010 *Address correspondence to: Ho-Chang Kuo, No. 123, Da-Pei Rd., Niaosong Hsiang, Kaohsiung County 83301, Taiwan, ROC. Tel: +886 (7) ext. 8795; erickuo48@yahoo.com.tw