Kim Na Yeon, Choi Deok Young, Jung Mi Jin, Jeon In-Sang
Department of Pediatrics, Gil Medical Center, School of Medicine, Gachon University of Medicine and Science, Incheon, Korea.
Pediatr Cardiol. 2008 Nov;29(6):1110-4. doi: 10.1007/s00246-008-9221-4. Epub 2008 May 15.
Kawasaki disease (KD), an acute systemic vasculitis that primarily affects infants and young children, is occasionally refractory to initial high-dose intravenous immunoglobulin (IVIG). Some patients with KD develop peripheral gangrene as a fatal complication, though this is rare. There has been no case reported in the literature of complicated peripheral gangrene that completely resolved without sequelae. Recently, we encountered a patient with KD which was refractory to IVIG, later developing peripheral gangrene. For the treatment of a peripheral gangrene dexamethasone, methotrexate, and an anticoagulant were administered, resulting in resolution of the gangrenous lesions without sequelae. Early diagnosis and treatment can reduce permanent sequelae and mortality from refractory KD complicated by peripheral ischemia.
川崎病(KD)是一种主要影响婴幼儿的急性全身性血管炎,偶尔对初始大剂量静脉注射免疫球蛋白(IVIG)治疗无效。一些KD患者会发生周围坏疽这一致命并发症,不过这种情况很罕见。文献中尚未报道过复杂的周围坏疽完全治愈且无后遗症的病例。最近,我们遇到一名对IVIG治疗无效的KD患者,随后发展为周围坏疽。对于周围坏疽的治疗,给予了地塞米松、甲氨蝶呤和一种抗凝剂,坏疽性病变得以消退且无后遗症。早期诊断和治疗可减少难治性KD合并周围缺血导致的永久性后遗症和死亡率。