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1 [] 57 1 林 ; 27: DOI: / (Kawasaki disease) [1,2] 1967 [1] 1976 [3] -α(tumor necrosis factor-α, TNF-α)-6(interleukin-6, IL-6) [4] [5] [6] [5] [7]

2 Taiwan J Fam Med Vol. 27 No.1 (1) 1.5 (2) (3) (4) (5) (atypical or incomplete) 3 g/dl 450,000/ mm 3 WBC 10/HPF 15,000/mm 3 15,000/mm 3 100% (ESR 40 mm/hr) C- (CRP 3 mg/l) (intravenous immunoglobulin, IVIG) (aspirin) IVIG TNF-α antagonist aspirin 5 3, C 40 C 3 C CRP: 28.7 mg/l) ampicillin zithromax ampicillin ampicillin EBV diphenhydramine EBV IgG, IgM ampicillin cefazolin 1 2 CBC/DC platelet albumin ALT CRP 1

3 林 [12] (Kawasaki) (toxic shock syndrome) (Scarlet fever) (Stevens- Johnson Syndrome) <5 > < < (systemic-onset juvenile idiopathic arthritis 20% (pastia line) (target lesions) A 30-75% HSV HSV (30-50%)

4 Taiwan J Fam Med Vol. 27 No.1 (CMV IgG/ IgM) (HSV IgG/IgM) (throat swab culture) A O (antistreptolysin-o test) (virus isolation and identification) CRP (36.7 mg/l) (IVIG) >1.5cm) Bokey (aspirin 100 mg/capsule, 3# po TID) loading dose (50 mg/kg/day) 9 50% 2 5 [12] 39 C 40 C 4-6 ESR CRP [21] 2 急性期治療 aspirin

5 林 61 2 [12] 風險層級 藥物治療 身體活動 追蹤時程表 I II III aspirin 3-5mg/kg IV Wafarin (LMWH) V aspirin Wafarin (LMWH) LMWH: Low-Molecular-Weight heparin aspirin [8-10] (meta-analysis) 1,011 IVIGIVIG [11] IVIG IVIG 2 25%5% 1% [8,12] IVIG T [13] aspirin [10] (American Heart Association, AHA) aspirin [12] aspirin 3 5 aspirin [10,12] (Reye s syndrome) aspirin [12]

6 Taiwan J Fam Med Vol. 27 No.1 難治性疾病的治療 10% IVIG 36 IVIG 2AHA IVIG TNF-α cyclophosphamide [12] 359 IVIG [14] TNF-α IVIG 20 IVIG infliximab18 [15] infliximab20 IVIG 86 [16] infliximab IVIG aspirin infliximab [17] 125 IVIG [18] aspirin clopidogrel dipyridamole Heparin warfarin aspirin heparin [12] 長期追蹤處理 2004 AHA [12] 2010 (Japanese Circulation Society) [19] [20] 2 1. Kawasaki T: Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Arerugi 1967; 16: Huang SK, Lin MT, Chen HC, Huang SC, Wu MH: Epidemiology of Kawasaki disease: prevalence from national database and future trends projection by system dynamics modeling. J Pediatr 2013; 163: e1. 3. Yang HY, Lin GJ, Lee CY, Lue HC: Clinical observation of mucocutaneous lymph node syndrome. Acta Paediatrica Sinica 1986; 26: Takahashi K, Oharaseki T, Yokouchi Y: Update on etio and immunopathogenesis of Kawasaki disease. Curr Opin Rheumatol 2014; 26: Uehara R, Belay ED: Epidemiology of Kawasaki disease in Asia, Europe, and the United States. J Epidemiol 2012; 22: HC Lue, LR Chen, MT Lin, et al: Estimation

7 林 63 of the incidence of Kawasaki disease in Taiwan. A comparison of two data sources: nationwide hospital survey and national health insurance claims. Pediatr Neonatol 2014; 55: Burns JC, Herzog L, Fabri O, et al; Kawasaki Disease Global Climate Consortium. Seasonality of Kawasaki disease: a global perspective. PLoS One 2013; 8: e Terai M, Shulman ST: Prevalence of coronary artery abnormalities in Kawasaki disease is highly dependent on gamma globulin dose but independent of salicylate dose. J Pediatr 1997; 131: Lee G, Lee SE, Hong YM, Sohn S: Is highdose aspirin necessary in the acute phase of Kawasaki disease? Korean Circ J 2013; 43: Baumer JH, Love SJ, Gupta A, Haines LC, Maconochie I, Dua JS: Salicylate for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev 2006; (4): CD Chen S, Dong Y, Yin Y, Krucoff MW: Intravenous immunoglobulin plus corticosteroid to prevent coronary artery abnormalities in Kawasaki disease: a metaanalysis. Heart 2013; 99: Newburger JW, Takahashi M, Gerber MA, et al: 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. Circulation 2004; 110: Sato YZ, Molkara DP, Daniels LB, et al: Cardiovascular biomarkers in acute Kawasaki disease. Int J Cardiol 2013; 164: Kobayashi T, Kobayashi T, Morikawa A, et al: Efficacy of intravenous immunoglobulin combined with prednisolone following resistance to initial intravenous immunoglobulin treatment of acute Kawasaki disease. J Pediatr 2013; 163: Mori M, Imagawa T, Hara R, et al: Efficacy and limitation of infliximab treatment for children with Kawasaki disease intractable to intravenous immunoglobulin therapy: report of an open-label case series. J Rheumatol 2012; 39: Son MB, Gauvreau K, Burns JC, et al: Infliximab for intravenous immunoglobulin resistance in Kawasaki disease: a retrospective study. J Pediatr 2011; 158: e Tremoulet AH, Jain S, Jaggi P, et al: Infliximab for intensification of primary therapy for Kawasaki disease: a phase 3 randomised, double-blind, placebo-controlled trial. Lancet 2014; 383: Hokosaki T, Mori M, Nishizawa T, et al: Long-term efficacy of plasma exchange treatment for refractory Kawasaki disease. Pediatr Int 2012; 54: JCS Joint Working Group. Guidelines for diagnosis and management of cardiovascular sequelae in Kawasaki disease (JCS 2008) digest version. Circ J 2010; 74: Gersony WM: The adult after Kawasaki disease the risks for late coronary events. J Am Coll Cardiol 2009; 54: Gerding R: Kawasaki disease: a review. J Pediatr Health Care 2011; 25:

8 64 [Case Report] Kawasaki Disease with Fever and Respiratory Symptoms: A Case Report Yin-Tzu Huang 1, Chien-Hung Lin 2 and, Wen-Jung Sun 1 A 5-year-old boy suffered from high fever up to 40 degrees with respiratory symptoms; diagnosis at an emergency department indicated bronchopneumonia and severe dehydration. The boy was then hospitalized and treated with antibiotics, but high fever remained persistent and was accompanied by skin rash, dry, fissured lip and other symptoms resembling those of Kawasaki disease. However, there was not enough evidence to support a diagnosis meeting the criteria of typical or atypical Kawasaki disease. After blood examination, urine analysis, cardiac sonography, and viral/ bacterial culture, we discussed with the parents about the possibility of Kawasaki disease and its coronary and cardiac complications. The next day after the discussion, the parents agreed to have the boy accept the therapy of intravenous immunoglobulin at their own expense. Fortunately, the fever subsided the following day after one dose of IVIG, and the boy was discharged from the hospital after eight days (no fever for three days). Five days after discharge, left neck swelling and periungual desquamation were noted during follow-up at clinic. The latest symptoms, together with previous ones, met the four criteria for the diagnosis of typical Kawasaki disease. Follow-up cardiac sonography revealed trivial mitral and tricuspid regurgitation. The most severe complication of Kawasaki disease is cardiovascular disease. In the United States, children diagnosed with Kawasaki disease should receive IVIG within the first 10 days of becoming ill. The treatment is most efficacious when it occurs within 7 days of the illness. It should be noted that performing the treatment within 5 days of the illness does not appear to prevent cardiovascular complications more effectively than having the treatment between 5 to 7 days as the efficacy of the treatment is more directly associated with an increased need for repeat treatment with IVIG. Long-term management and follow-up of Kawasaki disease depends on the severity and extent of coronary aneurysm or other cardiovascular complications developed during the acute phase. (Taiwan J Fam Med 2017; 27: 57-64) DOI: / Key words: cardiovascular complication, coronary involvement, Kawasaki 1 Departments of Family Medicine and 2 Pediatrics, Taipei City Hospital-Zhongxing Branch, Taipei, Taiwan. Received: March 14, 2017; Accepted: March 23, 2017.