Sensory evoked potentials in patients with Rett syndrome through the lens of animal studies: Systematic review

ObjectiveSystematically review the abnormalities in event related potential (ERP) recorded in Rett Syndrome (RTT) patients and animals in search of translational biomarkers of deficits related to the particular neurophysiological processes of known genetic origin (MECP2 mutations).MethodsPubmed, ISI Web of Knowledge and BIORXIV were searched for the relevant articles according to PRISMA standards.ResultsERP components are generally delayed across all sensory modalities both in RTT patients and its animal model, while findings on ERPs amplitude strongly depend on stimulus properties and presentation rate. Studies on RTT animal models uncovered the abnormalities in the excitatory and inhibitory transmission as critical mechanisms underlying the ERPs changes, but showed that even similar ERP alterations in auditory and visual domains have a diverse neural basis. A range of novel approaches has been developed in animal studies bringing along the meaningful neurophysiological interpretation of ERP measures in RTT patients.ConclusionsWhile there is a clear evidence for sensory ERPs abnormalities in RTT, to further advance the field there is a need in a large-scale ERP studies with the functionally-relevant experimental paradigms.SignificanceThe review provides insights into domain-specific neural basis of the ERP abnormalities and promotes clinical application of the ERP measures as the non-invasive functional biomarkers of RTT pathophysiology.     

放射性心脏损伤动物模型相关实验研究进展

当利用放射线对胸部恶性肿瘤进行治疗时，位于纵隔的心脏会不可幸免受到照射，从而诱发放射性心脏损伤（radiation-induced heart disease, RIHD）。随着手术以及放化疗技术的提升，肿瘤患者生存时间得到延长，使得RIHD这一放疗远期并发症被越来越多的报道。因此，学者们对于RIHD的研究逐渐升温。目前国内外学者关于该疾病尚未形成统一的认识，临床上缺乏有效阻止其发生的方法。动物模型研究可为临床该疾病治疗及预防提供可靠证据，为此本文回顾分析近年来放射性心脏损伤动物模型实验研究情况，旨在为后续实验开展及临床应用提供参考。     

Addressing risk management difficulties in children with food allergies

Risk is a concept inherent in every medical procedure. It can be defined as the probability of an adverse event in a defined population over a specified period of time. In the frame of food allergy management, it might be related to a diagnostic procedure, a treatment, or the consumption of foods. The risk of an adverse event can also be augmented by individual factors. This rostrum article discusses various aspects faced by children with food allergies in the light of risk, and their practical implications. Identifying personal risks for severe reaction, such as unstable asthma, and correcting them whenever possible also contribute to a reduction of the risk inherent to food allergy. Among the facets discussed, oral food challenges (OFC) are the most common diagnostic procedures implying an inherent risk. The risk of OFCs can be minimized by correct indication and timing of the test, a safe setting, as well as by ensuring that the patient is otherwise well without potential stressor potentially increasing the risk of a more severe reaction. Oral immunotherapy (OIT) has been studied as a potential treatment for increasing the threshold dose for reaction, and thus reducing the risk of accidental reaction. Nevertheless, the procedure is not devoid of risk as the patients may and do often react during the course of the procedure. Ingestion of trace amounts in processed foods, mainly in community settings such as restaurants, schools, or day care, represents a potential risk of reactions, although for a minority of patients. Precautionary allergen labeling (PAL) is a widespread strategy to reduce the potential risk of reactions due to traces. However, PAL is currently inefficient due to inconsistent labeling, also not indicating a clear maximum amount possibly present in the manufactured food. Finally, cost-effectiveness needs to be considered in risk management, as many risk reduction procedures are clearly not cost-effective.     

Influence of SNPs in cytokine-related genes on the severity of food allergy and atopic eczema in children

Although many single nucleotide polymorphism (SNP) studies have reported an association of atopy, allergic diseases and total serum immunoglobulin E (IgE) levels, almost all of these studies sought risk factors for the onset of these allergic diseases. Furthermore, many studies have analyzed a single gene and hardly any have analyzed environmental factors. In these analyses, the results could be masked and the effects of other genes and environmental factors may be decreased. Here, we described the correlation between four genes [interleukin (IL)-4 (C-590T), IL-4 receptor (A1652G), FCER1B (G6842A) and STAT6 (G2964A)] in connection with IgE production; the role of IL-10 (C-627A) as a regulatory cytokine of allergy; and the severity of food allergy (FA) and atopic eczema (AE) in 220 Japanese allergic children. In addition to these SNPs, environmental factors, i.e., patient's attitude, indoor environment, and so on, were also investigated in this study. Our study was retrospective, and the correlation was analyzed by our defined clinical scores divided into three terms: worst symptoms, recent symptoms and general amelioration at the most recent examination during the disease course. Our results indicated that IL-10 AA, the genotype with lower IL-10 production, is associated with higher IgE levels in the serum (p < 0.0001, estimate; 0.912). Marginal liver abnormalities were observed in the subject group with both FA and AE (p < 0.1191, estimate; 0.1490). Our defined clinical scores enabled evaluation of various aspects of disease severity. Based on the scores, while no single SNP selected in this study determined severity, the combination of the SNP with laboratory data and environmental factors appeared to determine severity.     

胰胆管合流异常动物模型的建立

目的建立胰胆管合流异常的动物模型。方法选用健康杂种猫10只。术前禁食12 h,3.5%戊巴比妥钠麻醉后,取上腹正中切口约6 cm切开各层至腹腔。于胆总管入十二指肠处旁边,切开胰腺背膜,解剖胰管;靠近十二指肠处分别纵向切开胰管、胆管长约4~6 mm的切口。6-0线间断吻合切口,造成类似人类的胰胆管合流的共同通道。术后20天胆道造影。结果术后动物精神、食欲良好,无萎靡、烦燥等表现,造影显示胰胆管合流共同通道延长。结论本动物模型最接近于人类的胰胆管合流异常生理,优于其他动物模型。     

高钙预处理对未成熟心肌的保护作用机制

目的 探讨高钙预处理对未成熟心肌保护作用及其可能的机制。方法 采用Langendorff离体心脏灌注模型，24只新生日本长耳大白兔分为对照组（I／R），高钙预处理（HCP）组，多黏菌素B（PMB）组和5-hydroxydecanoate（5-HD）组4组，分别行不同的处理方法。以血流动力学指标、生化指标和心肌超微结构作为观察指标。结果 HCP组心功能恢复优于I／R、PMB和5-HD组（P〈0．05），心肌生化指标优于I／R、PMB和5-HD组（P〈0．01），心肌超微结构损伤较I／R、PMB和5-HD组明显减轻，各指标I／R、PMB和5-HD组间差异无统计学意义（P〉0．05）。结论 高Ca^2＋预处理对未成熟心肌具有明显的保护作用，其机制是可能是通过蛋白激酶C激活和线粒体ATP敏感性钾通道开放起作用。     

中药膳食配合运动干预对肥胖性脂肪肝的影响

目的 探讨中药膳食配合运动干预对肥胖性脂肪肝的影响。方法 将68例脂肪肝患者随机分为对照组和观察组各34例。对照组常规给予低脂饮食和易善复、大黄庶虫丸等降酶保肝治疗；观察组按常规方法治疗外，采用中医辨证施食予以活血化淤、健脾利湿的中药膳食疗法配合运动干预．疗程均为1年。结果 治疗后观察组体重、甘油三酯、总胆固醇、谷丙转氨酶等显著低于对照组（均P〈0．01）。结论 中药膳食配合运动干预对肥胖性脂肪肝疗效显著。