Acrometageria: A spectrum of “premature aging” syndromes

A child with manifestations of acrogeria and metageria, two “premature aging” syndromes, is presented. Because of his indistinct phenotype and because the question has been previously raised as to whether these conditions are separate, we propose the designation of acrometageria to describe this phenotypic continuum. As there is much in common clinically between acrometageria and the syndrome of type III procollagen deficiency (Ehlers-Danlos type IV), it might be presumed that a similar pathogenesis for acrometageria exists. This possibility has been tested previously, without demonstrating specific quantitative or qualitative deficits, but with some indirect evidence that collagen metabolism is deranged in these patients. One such crude indicator is the elevation of urinary hyaluronic acid levels, demonstrated in our patient and also observed in the phenotypically distinct Werner and Hutchinson-Gilford premature aging syndromes. On one hand, it could be argued that this supports the concept that premature aging syndromes exist as a biological continuum. On the other hand, it is equally valid to argue that syndromes of premature aging are so described merely because they include recognizable changes of normal aging and that the demonstration of an underlying mutation in a collagen gene, for example, invalidates their study as models of accelerated normal aging. © Wiley-Liss, Inc.     

细胞色素b在早产新生大鼠高氧肺损伤肺组织中的表达和意义

目的探讨细胞色素b（cytochrome b，Cytb）与高氧肺损伤发生、发展的关系。方法早产新生SD（Sprague-Dawley）大鼠生后1d随机分为空气组、高氧组，高氧组持续暴露于常压氧舱中，氧浓度〉85％；空气组置于同一室常压空气中。两组分别于高氧或空气暴露后1、4、7、10和14d时提取肺组织RNA，采用半定量逆转录聚合酶链反应（RT-PCR）测定CytbmRNA表达；同时应用免疫组化方法检测肺组织切片中Cytb蛋白表达；应用Western-blot检测高氧肺损伤肺组织中Cytb蛋白水平的表达变化。结果与空气组相比，高氧暴露1d、4d CytbmRNA含量及其表达显著增强（P〈0．05）；7d后Cytb呈下降趋势，其表达较空气组减弱，但两者相比差异无统计学意义（P〉0．05）。高氧暴露后，随日龄变化肺组织Cytb免化结果与CytbmRNA表达相似；与空气组相比，高氧暴露1d、4d肺组织Cytb表达显著增强（P〈0．05）；7d后Cytb呈逐渐下降趋势，其表达较空气组减弱，但7、10d两组相比差异无统计学意义，14d极显著减弱（P〈0．01）。Western-blot实验结果：与空气组相比，高氧暴露1d、4dCytb蛋白水平表达增强但两组相比差异无统计学意义（P〉0．05）；7d后Cytb呈下降趋势，其表达较空气组减弱，但7、10d两组相比差异无统计学意义（P〉0．05），而14d显著减弱（P〈0．05）。结论85％高浓度氧暴露诱导早产新生大鼠线粒体编码的Cytb异常表达，这种变化可能参与高氧肺损伤的发生。     

Hereditary angio-oedema: new clinical observations and autoimmune screening, complement and kallikrein-kinin analyses

Objectives. To study clinical and laboratory manifestations of hereditary angio-oedema (HAE).
Subjects. Thirty-three affected members of a kindred of 63.
Results. Oedematous attacks in the skin, mucous membranes and gastrointestinal tract with fluid displacement were elicited by mental and physical stress, minor traumas, dental and surgical procedures, eruption of teeth, tonsillitis, pregnancies, and use of oestrogen-containing pills including menopausal substitution. Every adult woman with symptomatic HAE ( n =11) showed symptoms of urinary tract infections in conjunction with the attacks ( P = 0.010), and also experienced more spontaneous abortions or premature labours ( P =0.037) than healthy relatives. Patients with HAE of both sexes more frequently reported heartburn or peptic ulcers ( P =0.002). Rheumatic complaints were reported by 53% of HAE patients and 12% of their unaffected relatives ( P =0.013), but biochemical screening for 18 autoantibodies and quantitation of immunoglobulins did not reveal statistically significant differences between the two groups. C3, prekallikrein, total kininogen, high molecular weight kininogen (HK), alpha-2-macroglobulin and factor XII were not significantly different in HAE patients. In contrast, levels of C1-INH and C4 were depressed and cleaved HK increased in patients compared to unaffected relatives.
Conclusions. HAE manifests in a variety of ways, and may influence risk of spontaneous abortions and premature labour.     

丙种球蛋白治疗危重早产儿临床分析

目的 为评估IVIG治疗危重早产儿的临床疗效。方法 将危重早产儿116例采取随机分为治疗组和对照组，对照组给常规综合治疗，治疗组在常规综合治疗措施上，加用IVIG静脉滴注，比较治疗组与对照组患儿病情平稳率及死亡率。结果 IVIG治疗危重早产儿病情平稳率第5天达75．8％第10天达93．3％，而对照组分别为50％和70．4％；病死率治疗组为6．4％，对照组为11．1％，均优于常规对照组。结论 危重早产儿用IVIG治疗疗效较满意，值得推广。     

腺苷预处理对人未成熟心肌缺血再灌注损伤的保护作用

目的探讨腺苷预处理对人未成熟心肌缺血再灌注损伤的保护作用及其可能机制。方法将42例5岁以下行室间隔缺损修补术的患儿随机分成腺苷组和对照组,每组21例。腺苷组在术前用腺苷预处理,对照组经相同路径滴入相同体积的生理盐水后开始转流。观察心肌肌钙蛋白-I(cTnI)、肌酸激酶同工酶(CK-MB)、心肌超微结构改变,记录和计算两组自动复跳率、T波倒置发生率、室性心律失常发生率、术后平均住院日(d)。结果腺苷预处理明显减少了cTnI、CK-MB的漏出,在心肌超微结构和心电活动方面具有良好的保护作用。结论腺苷预处理对人未成熟心肌缺血再灌注损伤具有保护作用。     

不同日龄新生小鼠视网膜血管对氧疗的敏感性

目的观察不同日龄新生小鼠对氧疗的敏感性,研究视网膜血管的发育程度与新生血管形成的关系。方法选择4、7、9、11日龄C57BL/6J新生小鼠各48只,分为高氧组和空气组,通过视网膜铺片(ADP酶染色)和视网膜切片(HE染色),定性和定量观察视网膜血管的增生情况。结果与各空气组相比,4、7、9日龄高氧组小鼠吸高氧5d,返回空气第5天时,大量新生血管形成。而11日龄高氧组小鼠在返回空气第5天未见新生血管形成。4、7、9、11日龄高氧组小鼠出氧箱第5天突破视网膜内界膜的血管内皮细胞核数目分别为25.0±3.7、47.7±5.0、18.7±2.0、2.6±1.4个,4、7、9、11日龄空气组分别为0.7±1.1、1.2±1.2、0.8±1.0、1.6±1.0个,4、7、9日龄高氧组细胞核数目明显增多,与相应的空气组比较,差异有显著性(P<0.001);11日龄高氧组细胞核数无明显增多,与11日龄空气组比较,差异无显著性。结论视网膜血管的发育程度与新生血管形成有关,视网膜血管发育越不成熟越易发生视网膜病变,而发育成熟的视网膜则不会发生视网膜病变。     

早搏联律间期缩短对血流动力学的影响

目的研究房性、室性早搏联律间期缩短与桡动脉图的关系,探讨早搏对血流动力学的影响。方法经心电图诊断为房性早搏48例及室性早搏51例,再应用心功能仪检查,通过信号处理技术对早搏桡动脉图形进行面积积分。结果①早搏桡动脉图积分面积提前率〉40%时最小,20%-40%时居中,〈20%时最大（P〈0.05）。②提前率相同情况下,左房与右房的早搏桡动脉图积分面积比较差异无统计学意义（P〉0.05）;左室与右室的早搏桡动脉图积分面积比较差异有统计学意义（P〈0.05）。③早搏时的桡动脉图积分面积最小,早搏前第一个窦性心搏居中,早搏后第一个窦性心搏最大（P〈0.05）。结论早搏对血流动力学的影响主要与早搏的联律间期长短有关,室性早搏还与其发生部位有关,早搏的代偿间歇,不能改善对血流动力学的影响。     

单发室性早搏中RT间期规律的分析

目的探讨室性早搏前后RT间期变化的规律。方法选取单发、有完全代偿间期的室性早搏心电图,测量室早及其前后相邻波群(简称室早前、室早、室早后)的RT间期,进行对比。对室早前心室舒张时间与室早RT间期变化的关系进行分析。结果RT间期由短到长的顺序为:室早<室早前<室早后。室早前心室舒张时间与室早RT间期无线性相关关系。结论室早时RT间期最短,室早后RT间期最长,这一变化规律与心室舒张时间无关。     

西部区域经济跨越式发展的动力建构和路径选择

跨越式发展是经济发展相对落后地区在借鉴和吸收先进国家和地区成功经验和优秀成果的基础上 ,通过技术和生产力的跨越、管理和制度的创新、产业结构的优化升级、经济运行质量的提升、速度和效益的并进 ,不平衡推进和超常规增长 ,最终实现经济整体跃升的一种新的发展模式。在国家宏观政策和市场机制的双重作用下 ,充分利用各种有利条件 ,努力实现区域经济跨越式发展已成为西部地区的必然选择。积极推进区域制度创新、技术创新和文化创新 ,构建西部区域经济跨越式发展的动力系统 ,既是西部区域经济跨越式发展的必由之路 ,也是西部地区当前面临的主要任务。在路径选择上必须把发挥比较优势和发挥后发优势有机结合起来 ,使二者相得益彰 ,从而走出一条具有西部特色的跨越式发展之路。