儿童原发性干燥综合征五例并文献复习

Objective To further understand the clinic manifestations of childhood primary Sjogren's Syndrome(pSS) and enhance early diagnosis. Methods Five cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results ①Childhood pSS had various clinic presentations that were non-specific and sicca symptoms were absent or occur late in most cases. ② The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. ③ American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. Conclusion Recurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.     

肿瘤坏死因子拮抗剂治疗类风湿关节炎

类风湿关节炎（rheumatoid arthritis,RA）是以滑膜炎为主要表现的自身免疫病,可导致进行性关节的侵蚀破坏和功能丧失。几十年来,对于该病的诊疗观念已经有了显著变化,从”控制症状”发展为”改变病情”,并强调早期诊断和早期治疗的重要性。2002年,美国风湿病学院（American College of rheumatology,ACR）发表的RA治疗指南。     

喘可治注射液对大鼠抗炎和免疫调节机制的研究

目的:探讨喘可治注射液治疗支气管哮喘(简称哮喘)的作用机制.方法:60只雄性Wistar大鼠随机分为正常组、模型组、地塞米松组(0.5 ml·kg-1 ·d-1,肌内注射),高、中、低剂量(10、5、2.5 ml·kg-1·d-1,腹腔注射)喘可治注射液组.以2％卵蛋白为激发液,雾化吸入致敏,建立哮喘模型.治疗后,大鼠肺叶经HE染色后行病理切片,采用酶联免疫吸附(ELISA)法分别测定大鼠外周血中白介素-4(IL-4)和γ-干扰素(IFN-γ)浓度,采用流式细胞仪测定大鼠外周血中CD4+ CD25+T调节细胞和CD3- CD161a+自然杀伤(NK)细胞占淋巴细胞的百分比.结果:各剂量喘可治注射液组大鼠肺叶局部炎症反应均较模型组轻,IL-4浓度均明显下降,与模型组相比差异有显著性(P＜0.01),但各治疗组间、治疗组和正常组间IL-4浓度无显著差异.各治疗组IFN-γ的浓度均较模型组有所上升,其中高剂量组上升最为明显,与模型组比较有显著差异(P＜0.01).高、中、低剂量喘可治注射液组CD4+ CD25+T调节细胞百分比均明显上升,与模型组相比有显著差异(P＜0.05).CD3- CD161.+ NK细胞的百分比各组间比较无显著性差异.结论:喘可治注射液可增加血中IL-4、IFN-γ浓度,及CD4+ CD25+T调节细胞含量,纠正免疫失衡,这可能是其治疗哮喘的机制之一.     

联合使用糖皮质激素和来氟米特治疗幼年Cogan综合征1例报告并文献复习

目的探讨联合使用糖皮质激素和来氟米特治疗Cogan综合征的疗效和预后。方法对1例12岁男性Cogan综合征患儿应用甲基泼尼松龙合并来氟米特治疗并进行长期随访,分析临床疗效。结果患儿治疗24周状态良好,无疾病复发迹象。结论幼年时期特发性关节炎患儿出现眼耳症状时需要注意鉴别Cogan综合征,大剂量糖皮质激素联合来氟米特可以达到长期控制Cogan综合征的疗效。     

儿童原发性干燥综合征五例并文献复习

Objective To further understand the clinic manifestations of childhood primary Sjogren's Syndrome(pSS) and enhance early diagnosis. Methods Five cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results ①Childhood pSS had various clinic presentations that were non-specific and sicca symptoms were absent or occur late in most cases. ② The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. ③ American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. Conclusion Recurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.     

大剂量MTX治疗儿童ALL副作用防治研究

目的研究大剂量甲氨蝶呤(methotrexate,MTX)对急性淋巴细胞性白血病患儿的中枢神经系统白血病的预防,观察其毒副作用。方法13例ALL患儿接受57例次HD-MTX(每次3．0g／m^2)治疗。经综合预防措施后,观察临床症状、体征和实验室指标,总结HD-MTX的副作用发生种类、发生率及临床疗效。结果13例ALL中11例未发生CSNL,2例治疗中出现CNSL,经治疗后CR,现仍处于CCR中。13例平均CCR 31．8个月。结论儿童对HDMTX耐受性好,HD-MTX可以有效预防ALL的CNSL复发,提高长期无病生存率。     

儿童原发性干燥综合征五例并文献复习

Objective To further understand the clinic manifestations of childhood primary Sjogren's Syndrome(pSS) and enhance early diagnosis. Methods Five cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results ①Childhood pSS had various clinic presentations that were non-specific and sicca symptoms were absent or occur late in most cases. ② The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. ③ American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. Conclusion Recurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.     

儿童原发性干燥综合征五例并文献复习

Objective To further understand the clinic manifestations of childhood primary Sjogren's Syndrome(pSS) and enhance early diagnosis. Methods Five cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results ①Childhood pSS had various clinic presentations that were non-specific and sicca symptoms were absent or occur late in most cases. ② The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. ③ American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. Conclusion Recurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.     

儿童原发性干燥综合征五例并文献复习

Objective To further understand the clinic manifestations of childhood primary Sjogren's Syndrome(pSS) and enhance early diagnosis. Methods Five cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results ①Childhood pSS had various clinic presentations that were non-specific and sicca symptoms were absent or occur late in most cases. ② The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. ③ American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. Conclusion Recurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.     

依那西普对幼年特发性关节炎患儿外周血单核细胞核内核因子-κB表达的影响

目的 观察依那西普对幼年特发性关节炎(JIA)患儿外周血TNF-α水平以及单核细胞核内核因子-κB(NF-κB)表达的影响,探讨TNF-α和NF-κB在JIA发病机制中的作用.方法 以14例接受依那西普治疗的JIA患儿为观察对象,收集其用药前后的血样标本,采用ELISA法分别测定14例JIA患儿外周血TNF-α水平;并分离其外周血中单核细胞,抽提其核蛋白,采用凝胶迁移试验测定核内NF-κB水平.结果 1.应用依那西普前NF-κB水平(31.13±11.76)明显高于应用该药后(21.04±8.53)( t=4.42,P=0.001),TNF-α在治疗前水平[(24.18±7.37) ng·L-1]亦高于依那西普治疗后[(20.21±7.27) ng·L-1](t=2.251,P＝0.042); 2.NF-κB水平与TNF-α有良好的相关性(r=0.768,P=0.001),但NF-κB与单核细胞水平无相关性(r=0.212,P=0.369); 3.外周血单核细胞核内NF-κB与外周血中WBC、单核细胞、PLT、CRP、ESR水平无相关性.结论 抗TNF治疗对于JIA患儿有效,依那西普可以下调JIA患儿单核细胞核内NF-κB水平.NF-κB和TNF-α在JIA发病机制中可能起一定作用,其水平变化可以作为反映病情活动的指标之一.