甘利欣辅助治疗皮肌炎、系统性红斑狼疮疗效观察

目的 对甘利欣辅助治疗皮肌炎、系统性红斑狼疮的临床疗效及安全性进行临床评价。方法 采用非盲法半随机对照法，试验组10例，对照组11例，均据病情需要使用激素，试验组辅以静滴甘利欣250～300mg/d，观察2～3周。结果 皮肌炎、系统性红斑狼疮对照组的总有效率为72.7%，试验组为80%；但两组间差异无统计学意义（P〉0.5）。皮损好转及生化酶学改变亦无统计学意义。不良反应轻微。结论 甘利欣辅助治疗皮肌炎、系统性红斑狼疮的临床疗效尚需进一步研究。     

Evaluation of the Crithidia assay to distinguish between auto-immune chronic active hepatitis and systemic lupus erythematosus

The aim of this study was to determine if the Crithidia luciliae assay for auto-antibodies to double-stranded DNA, often positive in systemic lupus erythematosus, is always negative in auto-immune chronic active hepatitis (CAH) as has recently been suggested. Twenty-five patients were identified as having auto-immune CAH. Mean duration of follow-up was 10.5 years. Antinuclear antibodies were detected in 92%, smooth muscle antibodies in 76% and antimitochondrial antibodies in 16%. Antibodies to double-stranded DNA were detected by the Crithidia assay in four patients (16%). Two of these patients had positive tests on only one occasion and no features of systemic lupus erythematosus. In the other two the assay was persistently positive. During follow-up both developed arthritis and serositis but the liver lesion remained the dominant clinical feature. It was concluded that there is significant serological overlap between auto-immune CAH and systemic lupus erythematosus making the Crithidia assay unreliable in distinguishing between them.     

一种用于基因表达谱分析的基因芯片方法

目的：通过研究SLE患者外周血基因表达谱的改变，建立一种新型的基因芯片技术用于分子发病机理的研究。方法：提取总RNA，逆转录合成单链cDNA、双链cDNA，体外转录合成生物标记的cRNA与基因芯片进行杂交，通过抗体的检测标记荧光染料Cy3，基因芯片扫描仪进行图像扫描。结果：该方法有较高的重复性和稳定性，SLE患者与正常对照组相比较，鉴定出94个基因存在表达差异。结论：该方法能在较少起始标本量的情况下，有效地进行SLE致病基因的筛选，更好的理解SLE发生的分子机理，并且可在其它疾病研究中推广应用。     

Effects of chlorpromazine as a systemic vasodilator during cardiopulmonary bypass in neonates

OBJECTIVES: Vasodilator use during cardiopulmonary bypass is important in pediatric cardiac surgery, but the full range of their effects on hemodynamics remains to be clarified. We studied the effects of chlorpromazine, a potent alpha-blocking agent, in neonates. METHODS: Subjects were 60 neonates undergoing arterial switch operations for complete transposition of the great arteries with an intact ventricular septum. Of these, 37 received 2.1 to 6.5 mg/kg of chlorpromazine during cardiopulmonary bypass (CPZ group) and 23 received no vasodilator (control group). We then compared hemodynamic parameters between groups during and early after surgery. RESULTS: The systemic vascular resistance index and mean arterial pressure during cardiopulmonary bypass were significantly lower in the CPZ group (p < 0.05), but systolic pressure 15 minutes after cessation of cardiopulmonary bypass did not differ between groups. The rise in peripheral temperature during rewarming after hypothermia was significantly higher and the acid-base status 40 minutes after cardiopulmonary bypass less acidotic in the CPZ group. Urine output during cardiopulmonary bypass was higher in the CPZ group. CONCLUSIONS: Chlorpromazine effectively counteracts systemic vasoconstriction induced by cardiopulmonary bypass without serious side effects in neonatal cardiac surgery.     

The Effects of Astragalus membranaceus and Tripterygium hypoglaucum on NK Activity in Systemic Lupus Erythematosus

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Immunoadsorbent apheresis eliminates pathogenic IgG in childhood lupus nephritis

BACKGROUND: It is suggested that the highly cation-charged fraction of the IgG and IgG3 subclasses may play a pathogenic role in lupus nephritis. In contrast, immunoadsorbent therapy using a sodium dextransulfate fixed cellulose gel column-low invasive selective immunoadsorbent apheresis therapy (SDSC-IAT) has been applied to lupus nephritis with favorable results. However, elimination using pathogenic IgG by SDSC-IAT has never been investigated. METHOD: Two patients with diffuse proliferative lupus nephritis were treated using SDSC-IAT concomitant with immunosuppressive therapy. The eluates from the SDSC, and the patients' serum obtained before and just after SDSC-IAT were subjected to an IgG charge analysis using isoelectric focusing and immunoblotting, and also to laser nephelometry assay, which is used for measuring IgG subclass concentration. Indirect immunofluorescence staining was performed to detect IgG subclass deposition in the glomerulus. RESULTS: Both of the patients had an immediate decrease in anti-double-strand DNA antibody and in the circulating immune complex with a following clinical improvement. Repeated biopsies demonstrated improvement of glomerular lesions with a marked reduction of IgG and C3 deposition. The IgG of the SDSC eluates consisted of highly cation charged (isoelectric points: 9-10) fractions. In addition, IgG3 was specifically removed from the patients' serum using an SDSC among the IgG subclasses. The subclass of deposited IgG in the glomeruli showed IgG3 predominance. CONCLUSION: SDSC-IAT specifically removed the highly cation charged fractions of IgG and IgG3 from the patients' serum and the elimination of these fractions may have resulted in clinical improvement.     

C_3基因在BXSB小鼠主要脏器中的表达

用同位素标记ｃＤＮＡ探针的分子杂交方法检测了ＢＸＳＢ小鼠肝、肾、脾、胸腺各脏器中补体Ｃ_３ｍＲＮＡ的表达情况，结果表明３～４月龄雄性ＢＸＳＢ小鼠肝、肾、脾各脏器Ｃ_３ｍＲＮＡ的表达量较正常对照鼠显著增加。Ｃ_３基因的过度表达可能参与ＢＸＳＢ小鼠ＳＬＥ多脏器非感染性炎症的发生，同时提示该鼠存在Ｍ系统的大量扩增及活化。     

SLE小鼠高IgG血症Fcgr2b基因序列分析

目的: 测定系统性红斑狼疮(SLE)小鼠模型-NZB/WF1双亲NZB,NZW小鼠 Fcgr2b基因启动子区核酸序列,明确Fcgr2b基因启动子区的突变性质.方法:扩增NZB,NZW小鼠Fcgr2b基因启动子DNA进行核酸序列分析.采用ELISA法测定、比较(NZB×NZW )F1×NZW回交小鼠Fcgr2b基因B/W型与W/W型组间血清总IgG 水平.结果:NZB小鼠Fcgr2b基因启动子区与正常鼠Balb/C相比存在2个部位碱基缺失,分别为13 bp及3 bp.NZW小鼠除有3个碱基置换外,与Balb/C鼠该基因启动子区序列相同.回交小鼠Fcgr2b基因B/W型组血清总 IgG水平明显高于W/W型组(P<0.0001).结论:NZB小鼠Fcgr2b基因启动子区存在碱基缺失,且该缺失突变可能与血清总IgG水平升高有关.     

37例神经精神狼疮的临床分析

目的:对神经精神狼疮(NPLE)的临床表现、辅助检查及预后进行总结。方法:对37例伴中枢神经系统损害的SLE患者进行回顾性分析。结果:①有局灶性症状及癫痫反复发作者预后差;②MRI检查的阳性率高,但定位缺乏特异性;③神经精神狼疮(NPLE)患者的脑脊液(CSF)蛋白升高,尤其是IgG的升高是其主要特点。结论:NPLE患者症状不同预后有明显差异,MRI检查和脑脊液蛋白的测定为NPLE的诊断、治疗提供依据。