二维斑点追踪显像技术评估窒息新生儿左心室纵向收缩功能与心肌损害的相关性

【摘要】目的 探讨二维斑点追踪显像技术(2D-STI)评估新生儿窒息合并心肌损害后左心室整体及局部心肌的纵向收缩功能在早期诊断窒息新生儿心肌损害中的临床价值。方法 选择2019年07月至2020年12月期间在右江民族医学院附属医院新生儿科住院的足月窒息新生儿61例，经临床确诊合并心肌损害，根据Apgar评分分为轻度组31例和重度组30例，选择同期住院出生的正常足月新生儿30例作为对照组。检测受检者的血清肌酸激酶同工酶（CK-MB）、肌钙蛋白（cTnT）、左室舒张期前后径（LVDId）、左室射血分数（LVEF）、左室短轴缩短率（LVFS）、辛普森法左室射血分数（Simpson EF）、左室三腔心整体应变（GLS-LAX）、左室四腔心整体应变（GLS-A4C）、左室两腔心整体应变（GLS-A2C）、左室整体应变（GLS-AVG）,分析GLS-AVG和CK-MB、cTnT三者的相关性。结果 三组间CK-MB和cTnT比较差异有统计学意义（P<0.05）。三组间性别、体重、胎龄均无统计学差异（P>0.05）。三组间LVDId、LVEF、LVFS、Simpson EF比较差异无统计学意义（P>0.05）。GLS-AVG与CK-MB呈负性相关（r=-0.515，P=0.000），GLS-AVG与cTnT呈负性相关（r=-0.912，P=0.000）。结论 GLS-AVG与CK-MB、cTnT具有相关性，GLS-AVG可作为窒息新生儿心肌损害早期诊断指标。     

注意缺陷多动障碍儿童执行功能的评估与干预

ADHD儿童认知障碍的主要特征之一是执行功能损害,涉及包括前额叶皮层在内的特定脑区域及多层级脑网络异常。执行功能按照功能性分为冷-热两方面:冷执行功能损害主要表现于反应抑制、工作记忆以及认知灵活性等;热执行功能损害通常涉及延迟满足、赏罚相关决策、自我调节及情绪调节等。ADHD儿童的执行功能评估应基于可信范式进行文化适应性调整。临床干预不能单一聚焦于冷执行功能,需关注儿童的身体发育以及热执行功能(情绪社会方面)的发展进程,结合其他共病与心理状况,进行全面的动态随访与干预,以达到最佳的长期疗效。     

The role of dark adaptation in understanding early AMD

The main aim of the paper is to discuss current knowledge on how Age Related Macular Degeneration (AMD) affects Dark Adaptation (DA). The paper is divided into three parts. Firstly, we outline some of the molecular mechanisms that control DA. Secondly, we review the psychophysical issues and the corresponding analytical techniques. Finally, we characterise the link between slowed DA and the morphological abnormalities in early AMD.Historically, DA has been regarded as too cumbersome for widespread clinical application. Yet the technique is extremely useful; it is widely accepted that the psychophysically obtained slope of the second rod-mediated phase of the dark adaptation function is an accurate assay of photoreceptor pigment regeneration kinetics. Technological developments have prompted new ways of generating the DA curve, but analytical problems remain. A simple potential solution to these, based on the application of a novel fast mathematical algorithm, is presented. This allows the calculation of the parameters of the DA curve in real time.Improving current management of AMD will depend on identifying a satisfactory endpoint for evaluating future therapeutic strategies. This must be implemented before the onset of severe disease. Morphological changes progress too slowly to act as a satisfactory endpoint for new therapies whereas functional changes, such as those seen in DA, may have more potential in this regard. It is important to recognise, however, that the functional changes are not confined to rods and that building a mathematical model of the DA curve enables the separation of rod and cone dysfunction and allows more versatility in terms of the range of disease severity that can be monitored. Examples are presented that show how analysing the DA curve into its constituent components can improve our understanding of the morphological changes in early AMD.     

儿童社区获得性肺炎血清维生素A的变化及与免疫功能相关性

目的 研究社区获得性肺炎(community acquired pneumonia,CAP)患儿血清维生素A(vitamin A,VA)水平及与免疫功能的相关性,为肺炎病情评估提供一定参考。方法 以入住新乡医学院第一附属医院PICU的63例重度社区获得性肺炎(severe community acquired pneumonia, SCAP)患儿(SCAP组)、普通儿科病区的30例轻度社区获得性肺炎(mild community acquired pneumonia, MCAP)患儿(MCAP组),以及同期体检的30名健康儿童(对照组)为研究对象,检测其血清中VA和免疫球蛋白(immunoglobulin,Ig)G、IgA、IgM水平,及SCAP组体内T淋巴细胞亚群(总T淋巴细胞、CD4、CD8、CD4/CD8),并对SCAP组体内VA水平及以上指标的相关性进行分析。结果 3组性别和年龄差异无统计学意义;血清中VA的平均含量分别为0.36、0.25和0.19 mg/L,CAP组VA的含量较对照组明显降低,且SCAP组明显低于MCAP组(P<0.05)。根据WHO推荐的VA诊断标准,3组VA临床缺乏/亚临床缺乏率分别为10.00%、36.67%和61.90%,差异有统计学意义(P<0.05)。CAP组血清中Ig水平较对照组明显降低,且SCAP组明显低于MCAP组(P<0.05)。SCAP组血中总T淋巴细胞、CD4、CD8和CD4/CD8的平均水平分别为53.28%、30.26%、20.24%和1.59;分析VA水平与免疫相关指标关系发现VA水平与Ig(IgG、IgA、IgM)水平、总T淋巴细胞、CD4呈正相关,与CD8水平不相关。结论 肺炎患儿血清VA水平与病情严重程度及机体免疫功能相关。     

Left ventricular noncompaction associated with a pathogenic mutation in the MYH7 gene: Known mutation,different phenotype

Left ventricular noncompaction (LVNC) is a genetically heterogeneous cardiomyopathy, with familial and sporadic forms, but genetic testing only identifies a pathogenic mutation in a minority of cases. The main complications are heart failure, embolism and dysrhythmias. Herein we report a familial case of LVNC associated with a mutation in the MYH7 gene and review the literature regarding controversies in LVNC. A 50-year-old woman was referred to the cardiology clinic for palpitations. She underwent echocardiography and cardiac magnetic resonance imaging that revealed mild left ventricular systolic dysfunction and LVNC criteria. She had several episodes of non-sustained ventricular tachycardia and received an implantable cardioverter-defibrillator (ICD). Genetic testing revealed the c.1003G>C (p.Ala335Pro) mutation in the MYH7 gene. Familial screening showed clear genotype-phenotype cosegregation, which provided strong evidence for the pathogenic role of this mutation. To the best of our knowledge, this is the first report of LVNC associated with the p.Ala335Pro mutation in the MYH7 gene. This mutation has been described in hypertrophic cardiomyopathy, suggesting that the same pathogenic sarcomere mutation may be associated with different cardiomyopathies. This case also highlights the current difficulties regarding decisions on ICD implantation for primary prevention of sudden cardiac death in LVNC.