儿童混合性结缔组织病9例临床分析

摘要

目的 总结分析儿童混合性结缔组织病(MCTD)的临床及转归特点.方法 对2001-2009年间确诊为MCTYD的9例患儿的临床表现、实验室检查、诊治及随访情况进行总结分析.结果 9例患儿均有雷诺现象,其次常见的症状包括关节肿痛、手指肿胀、硬指、发热、乏力、贫血、活动后气短等.血液系统受累4例,其中3例轻度贫血,1例血小板减少.1例肾活检提示符合狼疮肾炎IIA型.实验室指标中红细胞沉降率增快8例,IgG升高7例,C4降低3例,CH50升高5例,CK升高4例(该4例患儿均行肌电图检查,3例未见明显肌源性损害,1例为可疑肌源性损害).自身抗体ANA阳性9例,抗Ul-RNP抗体阳性9例,抗SSA抗体低滴度阳性3例.6例行肺功能检查,4例存在弥散功能障碍.6例行肺部高分辨CT检查,2例示肺间质病变.超声心动图检查示肺动脉高压3例,合并右心增大、主动脉增宽2例,轻度肺动脉高压1例,合并少量心包积液1例.3例行食管造影检查.未见明显食管蠕动障碍.多数患儿入院前曾诊断为系统性红斑狼疮、幼年特发性关节炎、雷诺现象、结缔组织病、发热原因待查等,不规律接受激素或免疫抑制剂治疗者4例.9例患儿经确诊后予规范激素和(或)免疫抑制剂治疗,8例病情好转,1例病情控制无明显进展,无死亡病例.结论 儿童MCTD为多系统受累,早期以雷诺现象、关节症状、发热多见,在尚未出现肺部症状时可存在肺功能、肺部高分辨CT异常,可伴有肺动脉高压,误诊率较高.早期完善相关免疫指标、超声心动图、肺功能、肺部高分辨CT、食管造影等检查可帮助诊断.规范的激素及免疫抑制剂治疗对病情转归及预后极为重要,需长期对患儿进行随访观察.%Objective To understand the prevalence of Staphylococcus aureus carrying Panton-Valentine leuko-cidin gene (PVL gene) in isolates from children and adult. Methods The 16SrRNA gene, PVL gene and mecA gene of Staphylococcus aureus from 66 children' s isolates and 76 adult isolates were determined by multiplex PCR. After that, the SCCmec genetype and subtype of methicillin-resistant Staphylococcus aureus (MRSA) were determined by the other multiplex PCR. Results Among Staphylococcus aureus strains isolated from 66 children, 7 isolates were MRSA (10.6%). Among Staphylococcus aureus strains isolated from 76 adults, 39 isolates were MRSA positive (10.6%). The positive rate of MRSA in adult was significantly higher than that in children (X2 = 26.73, P < 0.01). The positive rate of PVL gene in children' s isolates and adult isolates were 47.0% and 7.9% respectively. The positive rate of methicillin-sensitive Staphylococcus aureus (MSSA) carrying PVL gene in children' s isolates and adult isolates were 49.2% and 5.4% respectively. The differences between children' s isolates and adult isolates were all statistically significant (P < 0.01). The PVL gene in 2 children isolates was SCCmecIV. 4 adult MRSA carried PVL gene, one was SCCmec I , one was SCCmec II , and the other two were SCCmec HI. Conclusions The rate of Staphylococcus aureus carrying PVL gene in children' s isolates is higher than that in adult isolates. The Staphylococcus aureus carrying PVL gene in children' s isolates is primarily MSSA.