系统性红斑狼疮外周血单个核细胞cFLIP-L mRNA和cFLIP-S mRNA表达的研究

目的 探讨系统性红斑狼疮(system lupus erythematosus,SLE)患者外周血单个核细胞(peripheral blood monouuclear cells,PBMC)中细胞型Fas相关死亡域样白介素-1β转换酶抑制蛋白(cFLIP)表达的意义.方法 应用半定量RT-PCR方法检测38例SLE患者和21名正常人PBMC中cFLIP-L mRNA和cFLIP-S mRNA的表达水平,并与SLE疾病活动指数(SLEDAI)评分进行相关性分析.结果 ① SLE患者PBMC中cFLIP-L mRNA和cFLIP-S mRNA表达水平均明显高于正常对照组(P＜0.01);SLE患者活动组cFLIP-L mRNA表达水平显著高于非活动组(P＜0.05),cFLIP-S mRNA表达水平在SLE患者活动组与非活动组之间没有显著性差异(P＞0.05). ② SLE患者cFLIP-L mRNA表达水平与SLEDAI评分呈正相关(r=0.423,P＜0.01);而cFLIP-S mRNA表达水平与SLEDAI评分无明显相关性(r=0.270,P＞0.05).结论 cFLIP-L mRNA和cFLIP-S mRNA可能在SLE发病机制中起重要作用.     

系统性红斑狼疮自体外周造血干细胞移植风险分析

目的提高对系统性红斑狼疮(SLE)自体外周造血干细胞移植风险的认识。方法分析8例SLE自体造血干细胞移植病例的移植相关并发症。结果8例均出现移植后感染,其中真菌、巨细胞病毒和带状疱疹各1例。5例出现心血管并发症,表现为急性左心衰竭、心房颤动和频发室性早搏。没有严重的肝、肾功能损害。结论移植后感染和心血管并发症是SLE自体外周造血干细胞移植后主要移植风险。     

眼球内异物漏诊22例分析

在我院1971—1978年收治的眼球穿通伤合并眼内异物存留的131例住院患者中,因漏诊延误治疗的有22例,漏诊率为16.8%。为了吸取经验教训,提高医疗质量,现将22例漏诊病例分析如下:     

B细胞成熟抗原在系统性红斑狼疮患者外周血单个核细胞的表达

目的检测B细胞成熟抗原（BCMA）mRNA在系统性红斑狼疮（SLE）患者外周血单个核细胞（PBMC）的表达水平,探讨BCMA在SLE发病中的意义。方法纳入2006年1-11月收治的36例SLE患者,同期17例健康志愿者作为对照组,采用半定量RT-PCR法检测外周血单个核细胞中BCMA mRNA的表达,并与SLE疾病活动指数（SLEDAI）进行相关性分析。结果 SLE患者组BCMA mRNA表达水平（0.598±0.230）均明显高于正常对照组（0.411±0.309）（P〈0.05）。SLE患者BCMA mRNA表达水平与SLEDAI评分无相关性（P=0.590）。结论 SLE患者BCMA mRNA表达水平的增高,可能在SLE的发病机制中具有一定的作用。     

Laurence—Moon—Biedl氏综合征一例报告

Laurence—Moon—Biedl氏综合征是以视网膜色素变性,多指(趾),智力低下,肥胖和生殖器机能不足为特征的遗传性疾病。1886年,Laurence和Moon首先报告,后来Bardet和Biedl对本病作了完整的描述,确立为Laurence—Moon—Biedl氏综合征。国内吴厚章1954年首先报告,以后有张福星等先后对本综合征作了报告,最近我院遇到一例Laurence—Moon—Biedl氏综合征,特报告如下:     

苗族先天性无虹膜并发白内障一家系

无虹膜症是一种比较少见的先天性虹膜缺失 ,1819年首次由 Barrafa报导 ,国内已有多篇报告 ,但苗族家系尚未见报告 ,国内普查群体发病率为 1∶ 133931,通常为常染色体显性遗传外显率较高〔1〕。国内统计该病的外显率为 10 0 %。我们遇到一苗族无虹膜症并发白内障家系二代 3例 ,现报告如下。先证者女　 36岁　自幼双眼视力不良 ,羞明 ,父母健康 ,非近视通婚。 1996年 6月就诊 ,全身检查无异常 ,右眼视力0 .0 4,左眼视力 0 .0 2 ,眼位外斜 2 0度 ,双眼球呈水平震颤 ,伴旋转 ,双眼结膜 ,角膜未见异常。裂隙灯下检查未见虹膜。双晶体呈雪花状混…     

遗传性无虹膜症2家系

无虹膜症是一种少见的眼遗传病 ,发病率为 1/ 6 40 0 0～ 1/10 0 0 0 0。我中心遇 2家系 ,报告如下。家系 1　先证者谢某　女　 16岁　自幼视力不良 ,畏光。上初二后 ,因视力差辍学。家族中无近亲结婚史。其父、伯父、祖父均患同样眼病。全身检查无异常发现。两眼视力均为 0 .1。眼球无震颤、眼位正。角膜透明 (直径 11mm )。双眼无虹膜 ,可见晶体周边部和悬韧带 ,晶体透明。视乳头小 ,视网膜色泽正常、茎斑中心及光消失。眼压正常。客观验光 :右 6 .0 0 DS　- 1.5 0 DC× 15 =0 .1,右 6 .2 5 DS　 - 1.5 0 DC× 170 =0 .1。不能矫正…     

环夹娩核白内障囊外摘出人工晶状体植入术

为探讨手法娩核的简便术式,对１０４例老年性和并发性白内障施行６－７ｍｍ切口环夹娩核囊外摘出及人工晶状体植入术。结论作者认为是一种适合我国国情不需昂贵设备,组织损伤小的小切口手术方法。     

99Tc-亚甲基二膦酸盐对胶原诱导性关节炎大鼠滑膜及软骨凋亡相关因子的影响

Objective Collagen induced arthritis (CIA) rats is an animal model of human rheuma-toid arthritis (RA). It is widely used in research of the pathogenesis and the therapeutic targets of RA. This paper was to investigate the therapeutic action of 99Tc-methylene diphosphonic acid (MDP) on CIA rats and its effects on the expression of apoptosis associated factor bcl-2 and bax in synoviocytes and articular chon-drocytes. Methods CIA rat models were carried out by subcutaneous injection with bovine collagen Ⅱ and incomplete Freud's adjuvant. Rats were divided into four groups: control group, CIA model group (the CIA rats were infused with physiological saline via tail vein daily), 99Tc-MDP group (the C1A rats were injected with 99Tc-Mi)P 0.04 μg 99Tc/kg via tail vein daily) and methotrexate (MTX) group (the CIA rats were in-jected with MTX 1 mg/kg via tail vein weekly). The signs of arthritis were evaluated by arthritis index (AI) scores. Immunohistochemistry was performed to detect the expression of bcl-2 and bax in synoviocytes and articular chondrocytes. SPSS 13.0 was used for data analysis. Results (1) The signs of arthritis, AI scores and pathological changes of arthrosynovitis in CIA rats were significantly improved by 99Tc-MDP orMTX. (2) The expression of bcl-2 and box in the synoviocytes of CIA model group [(39.30 ± 0.53) %, (27.37 ±2.45)%] was significantly increased compared with control group [(7.56 ± 1. 18)% , (6.14 ± 1.71) % ; q = 46.27, 24.57, all P < 0.001]. In the synovioeytes of 99Tc-M DP group and MTX group, the level of bcl-2 was remarkably decreased [(30.24 ± 2.09) %, (27.25 ± 3.33) %] compared with CIA model group (q = 13.20, 17.56, all P <0.001), while the level of bax was slightly increased and the ratio of bcl-2/bax was significantly decreased. (3)The expression level of bcl-2 and bax in the articular chondro-cytes of CIA model group [(20.20 ± 2.78) %, (36.40 ± 1.67) %] was significantly higher than control group [(9.91±4.09)%, (6.71 ±3.50)%; q=10.51, 37.01, allP<0.001]. Compared with CIA model group, the expression level of bcl-2 in articular chondrocytes of 99Tc-MDP group [(26. 58 ± 2. 52) %] and MTX group [(27.06 ± 1.92) %] was remarksbly increased [(24.26 ± 2.75) %, (23.53 ± 0.74) % ; q = 6.53, 7.01, all P < 0.001]. And the expression level of bax was significantly decread (q = 15.12, 16.04, all P <0. 001) and the ratio of bcl-2/bax was significantly increased. Conclusions The ab-normal expression of apoptosis associated factor bcl-2 and bax in synoviocytes and articular chondrocytes was closely related to the occurrence and progression of signs in CIA rats. 99Tc-MDP could improve the signs of arthritis, meanwhile regulate the expression of bcl-2 and bax in synoviocytes and articular ehondrocytes, suggesting that one of the therapeutic mechanisms of 99Tc-MDP might be related to stimulated synoviocytes apoptosis and reduced articular chondrocytes apoptosis.     

自体外周血干细胞移植治疗系统性红斑狼疮过程中并发红色酵母菌败血症一例

近年来造血干细胞移植(HSCT)已成为治疗重症难治性系统性红斑狼疮(SLE)的一种新方法,欧洲风湿病协会和欧洲骨髓移植协作组自身免疫病数据库报道,SLE总的移植相关死亡率约为9%,死亡原因主要有：疾病进展、感染、出血和器官毒性。患者死于条件致病性微生物感染者中以念珠菌属、结核分枝杆菌、巨细胞病毒感染最为常见。而SLE在自体外周血干细胞移植(APBSCT)过程中并发红色酵母菌败血症国内、外未见报道,现将近期经治的1例报告如下。