WHSC1, a 90 kb SET domain-containing gene, expressed in early development and homologous to a Drosophila dysmorphy gene maps in the Wolf-Hirschhorn syndrome critical region and is fused to IgH in t(4;14) multiple myeloma [published erratum appears in Hum Mol Genet 1998 Sep;7(9):1527]

Wolf-Hirschhorn syndrome (WHS) is a malformation syndrome associated with a hemizygous deletion of the distal short arm of chromosome 4 (4p16.3). The smallest region of overlap between WHS patients, the WHS critical region, has been confined to 165 kb, of which the complete sequence is known. We have identified and studied a 90 kb gene, designated as WHSC1 , mapping to the 165 kb WHS critical region. This 25 exon gene is expressed ubiquitously in early development and undergoes complex alternative splicing and differential polyadenylation. It encodes a 136 kDa protein containing four domains present in other developmental proteins: a PWWP domain, an HMG box, a SET domain also found in the Drosophila dysmorphy gene ash -encoded protein, and a PHD-type zinc finger. It is expressed preferentially in rapidly growing embryonic tissues, in a pattern corresponding to affected organs in WHS patients. The nature of the protein motifs, the expression pattern and its mapping to the critical region led us to propose WHSC1 as a good candidate gene to be responsible for many of the phenotypic features of WHS. Finally, as a serendipitous finding, of the t(4;14) (p16.3;q32.3) translocations recently described in multiple myelomas, at least three breakpoints merge the IgH and WHSC1 genes, potentially causing fusion proteins replacing WHSC1 exons 1-4 by the IgH 5'-VDJ moiety.      

糖尿病大鼠视网膜细胞间黏附分子1表达与羟苯磺酸钙的干预效应

目的:观察糖尿病大鼠视网膜细胞间黏附分子1表达及羟苯磺酸钙对细胞间黏附分子1表达的影响。方法:实验于2004-09/2005-02在山东医学高等专科学校实验室完成。①实验分组:雄性SD大鼠55只,随机选择10只为正常对照组,其余大鼠均腹腔注射链脲佐菌素,72h后断尾取血测空腹血糖≥16.7mmol/L者为造模成功,成模大鼠随机分为糖尿病模型组20只和羟苯磺酸钙治疗组20只。②实验方法:羟苯磺酸钙组大鼠给予羟苯磺酸钙120mg/(kg·d)灌胃。其他两组大鼠灌胃等量生理盐水。③实验评估:12周后麻醉下处死动物,检测血糖、糖化血红蛋白水平;光镜下观察大鼠视网膜病理变化;以RT-PCR法检测细胞间黏附分子1mRNA表达;Westernblot法检测细胞间黏附分子1蛋白表达。结果:5只造模未成功弃之不用,余50只大鼠进入结果分析。①血糖:糖尿病模型组及羟苯磺酸钙治疗组血糖均高于正常组(P<0.01)。羟苯磺酸钙治疗后血糖有下降趋势但差异无统计学意义(P>0.05)。②糖化血红蛋白水平:糖尿病模型组高于正常组(P<0.01)。羟苯磺酸钙治疗组与正常组相比差异无显著性(P>0.05)。③细胞间黏附分子1mRNA表达:细胞间黏附分子1mRNA在糖尿病组表达最强,羟苯磺酸钙组表达低于糖尿病组(P<0.01)。④细胞间黏附分子1蛋白表达:各组均有细胞间黏附分子1表达,糖尿病大鼠较正常大鼠表达增强,羟苯磺酸钙治疗后呈下降趋势。结论:糖尿病大鼠视网膜细胞间黏附分子1表达增强,羟苯磺酸钙对糖尿病大鼠视网膜具有保护作用,其保护作用机制可能与降低细胞间黏附分子1表达有关。     

运用模糊聚类方法分析中药微量元素含量与药效的关系

目的:不同中药中各种微量元素的含量数据离散,从含量数据上很难说明不同药物的药物相似性,故运用模糊分类方法研究药物微量元素含量与药物疗效的关系,验证其在药物功效的相似性及不同种类中药定量指标评价方面的作用。方法:实验于2005-10/2006-11在吉林省中医药管理局中药微量元素分析实验室完成。用原子吸收分光光度法检测合欢花、黄芪、石菖蒲、远志、山楂、银杏叶、人参、元肉、蒲黄、酸枣仁、苏木11种中药中钾、钠、钙、镁、铁、铬、镍、锰、铜、锌元素的含量,结合锌/铜比值、镁/钙比值,以数据极差法进行统计指标的标准化处理,以数量积法算出衡量被分类对象之间的相关系数,确定论域上的模糊矩阵。结果:相关系数r=0.50时,合欢花,石菖蒲,远志,人参,元肉,蒲黄,酸枣仁7种药物聚类相似;相关系数r=0.65时,合欢花,石菖蒲,元肉,蒲黄,酸枣仁5种药物聚类相似;r=0.75时,合欢花,石菖蒲,蒲黄3种药物聚类相似。研究还发现:药物中金属元素含量丰富,而且相关系数较大的药物,其药物疗效相似。结论:运用数学与化学计量学方法建立模糊聚类分析模型,可以把离散的数据标准化处理,确定论域上的模糊矩阵可以研究不同药物间的药效相似性,可以对不同种类的中药进行定量指标的明确评价。     

肾移植术后肺部感染特征97例分析

回顾性分析解放军总医院第二附属医院1992-05/2007-07收治的97例肾移植术后肺部感染患者的临床资料,患者对治疗方案均知情同意。①肺部感染多发生于术后1￣12个月,尤以术后2￣6个月多见,大剂量激素冲击多为诱因;重症肺部感染多为混合性感染,多为巨细胞病毒、真菌、耐药菌群和条件致病菌感染为主。②97例患者中肺部感染致急性呼吸窘迫综合征25例,13例死亡,死亡率达47.3%;在2007年,4例急性呼吸窘迫综合征中仅1例死亡,死亡率下降为20%,其中,1例最长呼吸机辅助呼吸达33d,患者痊愈出院;2个或2个以上器官障碍者死亡24例,最多见于上消化道出血和肾功能衰竭。③从2006-06开始,对于急性呼吸窘迫综合征患者早期T淋巴细胞亚群CD4 /CD8 <1%,即刻完全停用钙调神经磷酸酶抑制剂和抗细胞代谢免疫抑制剂,仅给小剂量的糖皮质激素,且血肌酐平均下降100mmol/L,其中1例完全停用达25d,血肌酐完全正常。④肾移植术后肺部感染发生率高,早期依据T淋巴细胞亚群和血肌酐的变化及时有效的减少或停用抗排斥药物及抗生素经验性用药是救治成功的前提。病情危重者(特别是急性呼吸窘迫综合征)及早呼吸末正压通气,充分保护其他脏器的正常功能,合理应用糖皮质激素和纤维支气管镜,密切加强各专业学科间协作等是救治成功的关键。     

黄皮酰胺的抑制脂质过氧化和脑保护作用

黄皮酰胺是类似脑复康结构的一种化合物。实验表明po能明显延长小鼠断头后的张口呼吸时间和sc亚硝酸钠225 mg/kg后的动物存活时间。po黄皮酰胺100 mg/kg·bid×3能抑制由p0 50%乙醇(15 ml/kg)诱发小鼠肝脂质过氧化反应,使TBA反应值下降,明显激活肝和脑组织胞浆液中谷胱甘肽过氧化酶的活力。大鼠脑基底动脉条收缩实验结果表明,黄皮酰胺10^-5mol/L可明显抑制由5-HT,PGF_2x和花生四烯酸引起的血管收缩,提示黄皮酰胺有缓解血管痉挛,增加脑血流量的作用。     

应用Fura-2/AM检测分离的神经细胞内游离钙及其变化

本文以酶法制备新生大鼠脑细胞悬液,运用近年来发展起来的Fura-2技术,检测此神经细胞内游离钙(以下简写为[Ca²⁺]i)及其变化。结果表明:在静息状态下,其[Ca²⁺]i为240±5nmol/L。高钾去极化可使[Ca²⁺]i成倍增加.钙拮抗剂verapamil和Ilifedipinc能阻断高钾升高[Ca²⁺]i的作用。实验结果证明了所制备的神经细胞悬液的可用性及建立的Fura-2测定[Ca²⁺]i方法的可靠性。     

Surface coil MR imaging of abdominal viscera. Part III. The pancreas

Eight healthy volunteers and 11 patients with pancreatic abnormalities were studied using a conventional body coil and a prototype magnetic resonance (MR) surface coil. Final pathologic diagnoses included carcinoma of the head (six), body (one), and tail of the pancreas (two) and chronic pancreatitis (two). In surface coil images of the volunteers, the body and tail of the pancreas was visualized in all cases but one, and the pancreatic duct was seen in five of eight cases. In-plane spatial resolution of 0.9 X 0.9 mm and 5-mm section thickness was obtained. At the same time, pancreatic surface coil images had a twofold improvement in the signal-to-noise ratio (SNR) compared with body coil images. T1-weighted spin-echo images gave greater SNR, reduced motion artifacts, provided superior anatomic detail, and offered more diagnostic information than comparable T2-weighted images. Significant abnormalities detected only by surface coil imaging included a small tumor surrounded by reactive edema and periglandular tumor invasion. This study demonstrates that surface coil imaging of the pancreas not only is feasible but provides an improved method for examining the pancreas by MR.