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目的观察皮肤黏膜淋巴结综合征(MCLS)患儿临床特征,分析其诊断和治疗对预后的作用。方法随机抽取2001年12月-2008年12月我院MCLS患儿30例,回顾性分析其临床资料,观察典型性病例与不典型性病例间的临床特征区别;分析免疫球蛋白(IVIG)治疗无效的原因及对策;比较IVIG不同注射量的远期疗效。结果在临床症状上,除肛周脱屑外,典型MCLS病比非典型MCLS病高(P<0.05);在发病时间上,除肛周脱屑外,典型MCLS的各种临床表现均比非典型MCLS患者要晚(P<0.05);对IVIG治疗无反应患儿的冠状动脉病变(CAL)发病率高于其他患儿,发热持续时间长于其他患儿,Hb、Hct、PLT、ALB等各项指标均比其他患儿低,差异有统计学意义(P<0.05)。结论不完全性MCLS的临床症状不很典型,不易被发现诊断,而肛周脱屑可作为不完全性MCLS的特异性诊断指标之一;该病如急性期发热时间较长,导致Hb、Hct、ALB等进行性降低,会影响IVIG的疗果。
Objective To observe the clinical features of children with mucocutaneous lymph node syndrome (MCLS) and analyze the effect of diagnosis and treatment on the prognosis. Methods Thirty patients with MCLS in our hospital from December 2001 to December 2008 were randomly selected. The clinical data of the patients with MCLS were retrospectively analyzed. The clinical characteristics of typical cases and atypical cases were observed. The immunoglobulin (IVIG) Invalid causes and countermeasures; compare the long-term efficacy of IVIG with different injection volume. Results In clinical symptoms, typical MCLS disease was more severe than atypical MCLS disease except perianal scaling (P <0.05). In terms of onset time, in addition to perianal scaling, the typical clinical manifestations of MCLS were significantly higher than those of SARS (P <0.05). The incidence of coronary artery disease (CAL) in children who did not respond to IVIG was higher than that in other children. The duration of fever was longer than that in other children, Hb, Hct, PLT and ALB The indexes were lower than other children, the difference was statistically significant (P <0.05). ConclusionClinical symptoms of incomplete MCLS are not typical, difficult to be diagnosed, and perianal desquamation can be used as a specific diagnostic indicator of incomplete MCLS; the disease, such as acute fever longer, leading to Hb, Hct , ALB and other progressive reduction, will affect the IVIG results.