Goldenhar 综合征家系的临床表型和遗传学分析

目的分析1组Goldenhar综合征家系的临床表现及遗传学特征。方法我们随访到1组4代33人的Goldenhar综合征家系，对目前存活的29人进行了临床表型和遗传学的初步分析。结果家系内有Goldenhaar综合征患者5人．临床表现具高度多样性，累及眼、耳、脊柱、颜面、口腔等多个器官和系统的发育不良，在遗传方式上属于常染色体显性遗传。从细胞遗传学水平对家系中成员进行染色体检查，未发现核型异常。结论该Goldenhar综合征家系属常染色体显性遗传，染色体检查未发现核型异常。     

Intelligent dialogue based on statistical models of clinical decision-making

The independence Bayesian model has been used widely in computer programs designed to support clinical decision-making. A reasoning strategy has been developed to enable these programs to conduct clinically pertinent dialogue and explain their reasoning. It has been implemented in a program for the diagnosis of acute abdominal pain based on the Bayesian model of de Dombal et al. Several features of the dialogue design have been adopted from artificial intelligence research, including shared initiative and critiquing. The program adopts a flexible goal-driven strategy, attempting to confirm the clinician's diagnosis or rule out the likeliest alternative. Symptoms and signs are selected in order of their expected weights of evidence in favour of the hypothesized disease.     

婴儿肝炎综合征63例临床分析

对我科1989-1990年中收治的婴儿肝炎综合征63例的临床表现、合并症、治疗等方面进行了分析。笔者认为婴儿肝炎综合征为一组临床症候群。病因诊断有其重要性。腹部B超检查有助于肝炎和胆道闭锁的鉴别,可作为常规检查方法。并认为用肾上腺皮质激素治疗有效。     

河南省50例并殖吸虫病的流行病学和临床特征

本文对１９８７－１９９５年就诊的５０例并殖吸虫病患者进行了分析，发现近年来本病在河南省有逐渐增多的趋势。患者主要集中在豫西山区及丘陵地带。各年龄组中儿童占６２％，男性多于女性。     

Transactional models of early education effectiveness - What is the message for policy?

There is now a substantial body of American research evidence demonstrating that early education programmes can have major long term effects right through into adult life. Cost-benefit analysis has enabled the data to be transformed into a compelling case for public financial investment. But insufficient attention has been paid to explaining how a short term preschool programme could have such enduring effects. The effects are best understood by applying a transactional model which shows how processes in the school and community transformed and amplified short term effects into long term outcomes. Adopting a transactional model modifies the messages for policy. The results of evaluations carried out in one society may not apply in another. In some school systems there may be more effective strategies than early education for improving educational prospects.     

Developing ‘FOCUS’: A project to promote effective practice in child and adolescent mental health services in the UK

Summary UK health policy requires child and adolescent mental health service providers to demonstrate that their services are effective. The FOCUS project has been developed to improve the availability and accessibility of research evidence and innovation, to support purchasers in the effective commissioning of services and to help providers base service provision on evidence of effectiveness and develop methods of evaluation.     

原发性腹膜后脂肪肉瘤20例报告

自１９６５年５月至１９９１年７月共收治原发性腹膜后脂肪肉瘤２０例，均经手术及病理证实。２０例中完全切除者１５例，部分切除者４例，活检１例。肿瘤切除合并肾，肠襻等邻近器官切除者达１２例。全组的５年，１０年生存率分别是５７．１％和２０．０％，完全切除组的５年１０年生存率分别是６３．６％和２８．６％。２０例病人共进行了４８次切除手术。首次手术死亡２例。着重讨论了本病的诊断、治疗和突出的临床特点。     

颈动脉加压注射复方维脑路通合剂治疗脑梗死180例临床分析

本科自 2 0 0 0年 6月以来 ,采用颈动脉加压注射复方维脑路通合剂治疗脑梗死 180例 ,并与静脉给药组相比较 ,现总结如下。1　资料与方法1.1　临床资料　治疗组 180例 ,男 117例 ,女 6 3例 ,年龄 37岁～ 76岁 ,平均年龄 5 7.2 0岁。均为颈内动脉系统脑梗死。其中大面积脑梗死 4例 ,脑梗死 85例 ,腔隙性脑梗死 91例。大面积脑梗死组 4例 ,其梗死灶涉及 2个脑叶或以上 ,均无意识障碍及脑疝者 ,在发病 10d后即脑水肿高峰期后开始颈动脉加压注射。治疗组病例选择除下列禁忌 :①严重高血压病(血压≥ 2 6 .6 / 16kPa) ;②脑出血、出血性梗死 ;③严…     

中国原发性肝癌临床分期预测肝癌肝移植预后的临床研究

目的评价“中国原发性肝癌临床分期”对肝癌肝移植疗效的预测价值。方法对1993年4月至2003年1月我科59例采用肝移植治疗的肝癌病人临床资料进行回顾性分析,按中国原发性肝癌临床分期原则进行分期,比较各期间肝癌肝移植疗效。结果Ⅰb、Ⅱa、Ⅱb、Ⅲa和Ⅲb期移植术后1年生存率分别为83·33%、66·67%、50·00%、35·71%和16·67%,各期间术后累计生存率有统计学差异(P<0·01);Ⅰb~Ⅱb和Ⅲa~Ⅲb期移植术后1年生存率分别为66·67%和25·00%,2年生存率分别为45·71%和25·00%,两组间术后累计生存率有统计学差异(P<0·05)。结论中国原发性肝癌临床分期适用于肝癌肝移植术前分期。     

90例慢性牙周炎临床分析

目的评价不同治疗方法对慢性牙周炎的临床效果。方法对90例慢性牙周炎患者,随机分成三组:龈上洁治术 抗生素组(A组),单纯龈上洁治术组(B组)和单纯抗生素组(C组)。分别给予不同的治疗,在治疗后1周、2周和4周记录牙周袋探诊深度(PPD)、探诊附着水平(PAL)、菌斑指数(PLI)、龈沟出血指数(SBI)。结果治疗1周和2周后各指标显著减轻(P<0.05),2周比1周减轻更明显(P<0.05),4周与2周比较无明显差异(P>0.05),A组各指标减轻比B组显著(P<0.05);B组比C组疗效显著(P<0.05)。结论A组作用优于B组(P<0.05),B组优于C组(P<0.05)。