�ٴ�˼ά���������������סԺҽʦ�淶����ѵ�е�ʵ��

??The standardized resident training is an important step for training high-level professionals in clinical medicine and it is an important part of medical education after graduation. The training of residents’ clinical thinking and clinical judgement should be emphasized in all resident training programs. Some resident programs in China still focus on the teaching of important knowledge that are originated from the medical colleges. Therefore??it is urgent for faculty to adopt new teaching methods aiming at the training of clinical thinking and clinical judgement. The paper introduces a mode for teaching clinical thinking and clinical judgement in the resident training programs. The goal is to improve the level of resident physicians in clinical diagnosis and treatment in China.     

Good clinical practice and the conduct of clinical studies in pediatric oncology

This article discusses the principles that guide good clinical practice standards, with particular emphasis on how they to relate to pediatric oncology research and recent efforts at harmonization. The authors review the clinical trials process and the roles of the participants, highlighting the pivotal role of the clinical investigator and the research team, and briefly review the historical aspects of drug development regulations in the United States and the current regulatory paths for pediatric oncology drug development. Where relevant, historical events that underlie many of the regulations and their current applications are described, and practical examples are provided.     

抗心律失常药物临床应用的进展(三)

特殊临床情况下快速心律失常的处理一、先天性心脏病[3 0 ]有数种先天性心脏病可在病程晚期发生猝死 ,特别是法洛四联症、主动脉瓣狭窄与完全型大动脉转位等。其中法洛四联症是最常见的发绀型先天性心脏病。法洛四联症手术后患者中孤立性室性期前收缩和非持续性室性心动过速的发生率较高 ,所以孤立性的室性期前收缩或频发并不能作为患者高危因素的指标。如果患者无室性期前收缩 ,则能够作为患者发生室性心律失常危险性低的标志。人们对血流动力学、病史及心电图方面的指标进行研究 ,试图发现能够筛查出具有潜在危险因素的患者。这些指标包括…     

Pitfalls in the use of clinical asthma scoring

We evaluated the correlation of the Wood-Downes-Lecks clinical asthma score (CAS) with outcome in 210 consecutive known asthmatic children presenting to an urban emergency department for treatment of acute asthma. All children received standard treatment consisting of administration of beta-adrenergic agents and theophylline compounds. Clinical asthma scores were assigned before each treatment phase and before disposition from the emergency department. Ten-day follow-up information was collected on each patient by telephone. While no differences in pretreatment CASs were found between outcome groups, disposition CASs were found to be significantly higher in patients eventually admitted to the hospital as opposed to those discharged home. However, CASs were not effective in identifying either those patients who required prolonged hospitalization (greater than 24 hours) or those who sustained ongoing disability following discharge home from the emergency department. These data indicate that the CAS alone is not a reliable indicator of severity of acute asthma of childhood as judged by subsequent disability.     

Importance of clinical and epidemiological research in defining the long-term clinical care of pediatric cancer survivors

With the increasing number of long-term survivors of childhood cancer, there continues to be a critical need for development and implementation of evidence-based recommendations for clinical follow-up. In order to establish and maintain health-related follow-up guidelines, it is important to recognize the attributes of research from which the recommendations may be formulated. Issues including study design and clinical research methodology, completeness of long-term follow-up for the applicable study population, approaches for assessment of treatment-related exposures, methods utilized for ascertainment and characterization of outcomes, and recognition of potential modifiers of risk (e.g., demographic or treatment-specific factors) are all important considerations when evaluating the results of available research. For the future, greater attention will not only need to be given to further development and maintenance of recommendations for follow-up, but to the scientific evaluation of the recommendations to determine the subsequent impact on health status and quality of life among pediatric cancer survivors.     

儿童脑白质营养不良的临床遗传学研究进展北大核心CSCD

脑白质营养不良(leukodystrophy,LD)是一组以原发性神经胶质细胞和髓鞘异常为特征的遗传异质性疾病,是常见的儿童神经系统疾病,具有显著的基因型-表型相关性。近年来,高通量测序技术的进步改变了LD的诊疗模式,发育过程中自然病史的采集和多模态神经影像学的评估等精细化的表型分析为临床遗传诊断提供了重要参考。该文从临床遗传学角度切入对LD展开综述,旨在提升我国儿科医师对该病的整体认识。     

Advances in the clinical management of high-risk Wilms tumors

Outcomes are excellent for the majority of patients with Wilms tumors (WT). However, there remain WT subgroups for which the survival rate is approximately 50% or lower. Acknowledging that the composition of this high-risk group has changed over time reflecting improvements in therapy, we introduce the authors’ view of the historical and current approach to the classification and treatment of high-risk WT. For this review, we consider high-risk WT to include patients with newly diagnosed metastatic blastemal-type or diffuse anaplastic histology, those who relapse after having been initially treated with three or more different chemotherapeutics, or those who relapse more than once. In certain low- or low middle-income settings, socio-economic factors expand the definition of what constitutes a high-risk WT. As conventional therapies are inadequate to cure the majority of high-risk WT patients, advancement of laboratory and early-phase clinical investigations to identify active agents is urgently needed.     

���Ӷ�ͯ��֪���ܵ��ٴ�Ӧ��

??Assessments and surveillance of cognitive function could bring great benefits to clinical diagnosis and treatment. By reviewing the latest research progresses on cognitive development in children and the underlying neural and genetic mechanisms??this article aims at spreading to clinical staffs the importance of examining the cognitive development in children patients in order to better improve clinical diagnostic and therapeutic strategies.     

Advances in the clinical management of high-risk Wilms tumors

Outcomes are excellent for the majority of patients with Wilms tumors (WT). However, there remain WT subgroups for which the survival rate is approximately 50% or lower. Acknowledging that the composition of this high-risk group has changed over time reflecting improvements in therapy, we introduce the authors’ view of the historical and current approach to the classification and treatment of high-risk WT. For this review, we consider high-risk WT to include patients with newly diagnosed metastatic blastemal-type or diffuse anaplastic histology, those who relapse after having been initially treated with three or more different chemotherapeutics, or those who relapse more than once. In certain low- or low middle-income settings, socio-economic factors expand the definition of what constitutes a high-risk WT. As conventional therapies are inadequate to cure the majority of high-risk WT patients, advancement of laboratory and early-phase clinical investigations to identify active agents is urgently needed.     

Age-related differences in the clinical presentation of food-induced anaphylaxis

Food-induced anaphylaxis may be more difficult to recognize in younger children. We describe age-related patterns in the clinical presentation of children with anaphylaxis, which may facilitate the early recognition and treatment of this potentially life-threatening condition.