β-地中海贫血448例的基因型与临床分析

【目的】　探讨β- 地中海贫血(β- 地贫)基因突变与临床病情的关系。　【方法】　应用PCR RDB技术对448例β- 地贫患儿进行基因诊断。　【结果】　检测出12 种基因突变类型, 有24 种基因组合形式。　【结论】　β°纯合子及β°/β°双重杂合子临床表现重,发病年龄、输血年龄均较早,β°/β+双重杂合子表现为中间型或重型,发病年龄及输血年龄均较晚。     

Perturbation of copper (Cu) homeostasis and expression of Cu-binding proteins in cadmium-resistant lung fibroblasts.

To probe mechanisms of cadmium (Cd) damage to the lung extracellular matrix (ECM), we developed Cd-resistant (CdR) rat lung fibroblasts (RFL6) by incubation with graded concentrations of Cd. CdR cells downregulated lysyl oxidase (LO), a copper (Cu)-dependent enzyme essential for crosslinking of collagen and elastin in the ECM, in conjunction with upregulation of other Cu-binding proteins including Cu,Zn-superoxide dismutase (SOD1), copper chaperone for SOD1 (CCS1), metallothionein (MT), and Menkes P-type ATPase (ATP7A), a Cu transporter in the membrane of the Golgi apparatus, as well as gamma-glutamylcysteine synthetase (gamma-GCS), an enzyme for glutathione biosynthesis. Reduction and loss of cytoplasmic distribution of LO in CdR cells were accompanied by its dislocation with the Menkes P-type ATPase and the endoplasmic reticulum marker. CdR cells displayed a defect in LO catalytic activity but an enhancement in Cu,Zn-SOD catalytic activity consistent with the protein expression levels of these enzymes. Although long-term Cd exposure of cells enhanced the Menkes P-type ATPase protein expression, actually, it reduced Cu-dependent catalytic activity of this enzyme in parallel with the deficiency of LO. The low level of 64Cu bound to the LO fraction and the high level of 64Cu bound to the MT fraction provide direct evidence for limitation of Cu bioavailability for LO existing in the CdR cells. These results suggest that downregulation of LO is linked with upregulation of other Cu-binding proteins and with alteration in Cu homeostasis in the CdR phenotype.     

药物不良反应国内文献题录检索系统

本文报道了药物不良反应国内文献题录检索系统的设计原理、功能特点,以输入药名及不良反应类别为例,显示了检索系统操作简便,提供信息及时准确的特点。本系统可从药名、不良反应类别及作者等多方面进行检索,可为查询者提供所需的详细资料,弥补了人工查阅文献的不足。并将继续扩大文献资料库,由题录向电报文体文摘形式转化,以贮存更多的信息。     

肠道贾第虫病42例的临床分析

目的 :观察贾第虫病的临床表现及甲硝唑对贾第虫病的治疗作用。方法 :用甲硝唑治疗 42例贾第虫病患者 ,剂量为 0 .4g,日 3次 ,连服 7～ 10 d为一疗程 ,其中服 1个疗程的有 3 5例 ,服 2个疗程的有 5例 ,服 3个疗程的有 2例。结果 :经治疗后 ,全部患者的症状如腹泻及 (或 )腹痛等胃肠道症状均消失 ,粪检结果恢复正常。患者服药期间的不良反应轻 ,仅有轻微的胃肠道反应 ,如恶心、厌食等。结论 :甲硝唑治疗贾第虫病具有疗效好、耐受性好的优点     

3，4－二羟基苯乙酮对家兔缺血－再灌心肌的影响

以超微结构、线粒体形态计量及ＬＤＨ，ＳＯＤ活性为依据，探讨３，４－二羟基苯乙酮（ＤＨＡＰ）对家兔缺血－再灌心肌细胞的影响。结果表明：ＤＨＡＰ组缺血－再灌心肌细胞的损伤、ＬＤＨ丢失及ＳＯＤ活性下降的程度均明显低于未用药组。提示该药物对缺血－再灌心肌有保护作用。     

桂枝汤各组分作用的比较

以急性毒性、镇痛及体温降低作用为指标比较了桂枝汤及其组方中五味中药的作用。证实桂枝汤的镇痛作用主要由桂皮和芍药所致；降温作用主要因桂皮和甘草所致。在方剂中各味中药可产生协同效应。桂枝汤毒性主要由桂皮和甘草所致。方剂中各药的毒性均较单独用药降低。表明桂枝汤组方后各味中药的疗效增强而毒性降低。     

Spatio-temporal expression of estrogen sulfotransferase within the hepatic lobule of male rats: implication of in situ estrogen inactivation in androgen action.

Estrogen sulfotransferase (EST) catalyzes transfer of the sulfate group from phosphoadenosine phosphosulfate to estrogenic steroids. Since estrogen sulfates do not bind to the estrogen receptor with high affinity, EST can control the intracellular level of the receptor-active estrogens. Androgen action in the rat liver, as indicated by the androgenic induction of alpha 2u-globulin, is inhibited by low levels of estrogens. Thus, in situ estrogen inactivation by EST is expected to increase hepatic androgen sensitivity. During the lifespan of the animal, rat liver undergoes three distinct phases of androgen sensitivity, i.e. prepubertal androgen insensitivity, androgen sensitivity after approximately 40 days of age, and androgen insensitivity during senescence (greater than 750 days). EST in the liver is expressed only after puberty, when the liver becomes androgen sensitive. Furthermore, localization of EST and its corresponding mRNA within the lobular unit of the liver demonstrates that only androgen-responsive hepatocytes located around the central vein contain immunoreactive EST and its corresponding mRNA. These temporal and spatial correlations of EST expression and hepatic androgen sensitivity support the concept that steroid-inactivating enzymes play important roles in sex hormone action.     

^60Co消毒与高温高压消毒胚胎骨的实验研究

在12只健康家兔下颌骨体部的两侧各造成0.7cm×0.5cm×0.3cm的骨缺损区，分别置入^60Co消毒的胚胎骨和高温高压消毒的胚胎骨，发现高温高压消毒可以替代^60Co消毒胚胎骨进行移植，为临床提供了一种新的用作同种异体移植胚胎骨的消毒方法。     

叶内型肺隔离症致咯血的栓塞治疗

目的探讨叶内型肺隔离症患者咯血的栓塞治疗效果。方法结合文献复习，回顾性分析6例叶内型肺隔离症患者血管造影表现，并对畸形动脉行栓塞治疗。结果6例叶内型肺隔离症患者血管造影显示畸形动脉7支，表现为粗大、迂曲的体循环血管影；在毛细血管期可见隔离肺叶染色及引流静脉早显；静脉期显示引流静脉（肺静脉）。6例患者均进行畸形血管栓塞治疗，临床止血总有效率100％，随访1a均未复发。结论畸形血管栓塞术对叶内型肺隔离症致咯血的治疗，安全有效。     

阴股沟皮瓣显微解剖学特征及其在组织修复功能重建中的应用

目的：明确阴股沟皮瓣的显微解剖基础，为临床应用此皮瓣提供解剖依据。资料来源：应用计算机检索Medline1991-01／2005-01的相关文章，检索词“pudendal-thigh flap，micranatomy”，并限定文章语言种类为English。并且手工检索《中文科技资料目录一医药卫生）1998-01／2005-01的相关文章，检索途径：分类途径。 资料选择：对资料进行初审，并查看每篇文献后的引文。纳入标准：①随机对照的基础及临床试验。②无论是否为单盲，双盲或非盲法。排除标准：重复性研究或综述性文章。 资料提炼：共检索到42篇相关文章，21篇文献符合纳入标准，排除的21篇文献为内容陈旧或重复。 资料综合：阴股沟区动脉血液供应充足，并且位置均比较恒定，尤以阴部外动脉最为恒定。静脉回流丰富，神经支配可靠。阴股沟皮瓣是位于股内侧上方与会阴部之间的无毛区域，上界平耻骨联合上缘水平，下界为两侧坐骨结节连线，内侧界为阴囊或大阴唇外侧缘，外侧界为股内侧皱襞，自阴囊（唇）外侧缘向大腿内上方延伸约5cm包括的范围。该区动脉血液供应丰富，计有阴部外动脉，闭孔动脉前皮支，阴囊（唇）后动脉的外侧支，旋股内侧动脉的皮支，股内侧的主要动脉支，以及来自阴部内动脉的肛动脉（直肠下动脉）等，并且位置均比较恒定，其中尤其以阴部外动脉最为恒定。在阴股沟区，阴囊（唇）后动脉与旋股内动脉、闭孔动脉前支及阴部外动脉的分支存在广泛的吻合并在内收肌内侧以上的深筋膜层形成血管网。进入阴股沟区的多支血管相互吻合成网。阴股沟区静脉回流丰富，神经支配可靠。 结论：阴股沟皮瓣血运丰富，解剖简便易行，切取方便，供区隐蔽，再造性器官外形及恢复神经感觉均取得较为理想的效果，是一种值得推广的方法。