双侧延髓内侧梗死临床分析

目的分析并探究双侧延髓内侧梗死(BMMI)的血管病因、临床表现、MRI特征和预后,以期提高BMMI的早期诊断率.方法对青岛大学附属烟台毓璜顶医院诊治的14例BMMI患者临床资料进行分析.结果BMMI血管病因以动脉粥样硬化为主;临床症状表现为四肢瘫痪、舌瘫、感觉异常、头晕、构音障碍、饮水呛咳等,危重时出现呼吸衰竭.头颅DWI示双侧延髓内侧可呈特征性"心型"、"Y"字型、"V"字型或倒"八"字型高信号影.出院NIHSS评分、卒中进展(尤其呼吸衰竭)为BMMI的预后不良因素.结论BMMI临床少见,临床表现多样,预后较差;头颅MRI为其主要的影像学检查,有助于早期诊断.     

胶质细胞源性神经营养因子干预视网膜色素变性给药方式的研究进展

视网膜色素变性（retinitis pigmentosa，RP）的治疗目前仍处于探索阶段。胶质细胞源性神经营养因子（glial cell line-derived neurotrophic factor，GDNF）是目前研究中重要的神经营养因子，但其传统给药方式生物利用度相对较低。近年来GDNF安全有效的给药方式成为研究热点，包括经病毒载体或非病毒载体的基因工程法释药技术、经聚合物释放系统释药技术、经细胞移植释药技术、小分子触发器诱导产生GDNF等。本文就GDNF干预RP的给药方式进行综述。     

General anaesthesia for ophthalmic surgery

The majority of ophthalmic surgeries are performed as day cases under topical or regional anaesthesia with or without intravenous sedation. However, general anaesthesia is necessary in certain circumstances e.g. local anaesthetic allergy or patients who are unable to cooperate or to lie flat or still. Patients for ophthalmic surgery are frequently elderly with multiple comorbidities, such as diabetes and hypertension. Patients with rare genetic syndromes may present for eye surgery. Therefore adequate preoperative evaluation and preparation will minimize perioperative complications. The goals of general anaesthesia are smooth induction and emergence, with stable intra-ocular pressure (IOP) and akinesia of the globe. These can be achieved with a combination of intravenous and inhalational agents with or without muscle relaxants and opiates. Use of the laryngeal mask airway has the advantage of causing a smaller rise in IOP on insertion and less coughing on emergence. Total intravenous anaesthesia with propofol and remifentanil has the advantages of causing less postoperative nausea and vomiting (PONV), reduced stress response to airway intervention, rapid recovery and smooth emergence. Some eye procedures require special consideration, for example, strabismus and vitreoretinal surgery involves traction of the rectus muscles producing a higher incidence of oculocardiac reflex and PONV. Most ophthalmic surgery produces mild to moderate pain amenable to non-opioid analgesics. Intraoperative topical and regional anaesthesia reduce postoperative pain and opiate requirement. Open globe injury and a full stomach present unique challenges to prevent increase in IOP as well as protecting the airway.     

肝阴虚证证候特点及辨证用药规律的文献研究

目的:为进一步深入研究和探讨肝阴虚证候的本质,本文分析和整理肝阴虚证的病因病机、证候表征、证候属性疾病及遣方选药的规律。方法:运用检索文献并进行频数统计的方法,检索查阅1960年9月1日至2019年1月10日中国知网中医临床研究文献。结果:肝阴虚证的病因病机为肝失疏泄,气机郁滞,化火伤阴,阴液亏虚,失于濡养;证候辨证要点主要为脉弦细数,舌红少津,头晕目眩,五心烦热,口咽干燥,两目干涩;证候临床属性疾病有肝炎、肝硬化、肝纤维化、肝癌;临床上治疗肝阴虚证使用处方主要为一贯煎、补肝汤、四物汤、芍药甘草汤,临床高频使用中药为生地黄、白芍、枸杞、当归、麦冬。结论:本文对于肝阴虚证的证候特点以及辨证用药规律做出系统的总结与归纳,将有关肝阴虚证的论述联系起来,形成统一的整体,为揭示肝阴虚证的本质与临床上形成较为成熟的肝阴虚证治疗方案提供参考。     

缓解视疲劳功能中药复方保健产品配方规律及特点分析

目的通过检索特殊食品信息查询平台公布的具有缓解视疲劳功能的保健食品信息资料,以及药智数据网的中成药处方数据库中眼科中成药中有保健功能的产品信息资料,为具有缓解视疲劳功能的保健产品的组方与产品开发提供依据与参考。方法运用Microsoft Excel2016软件及中医传承辅助平台对检得的产品信息进行统计,分析其配方特点。结果共收集到141个具有缓解视疲劳功能的保健食品,其中64个(45.4%)保健食品配方中含有中药类原料。使用频次≥5的中药原料有6味,累计使用145次(72%),由高到低分别是菊花、枸杞子、决明子、桑椹、熟地黄、茯苓。通过无监督的熵层次聚类得到3个新处方。非中药原料在缓解视疲劳功能的保健食品中应用较为普遍,含有外来天然植物资源的60个产品,含叶黄素及维生素类的17个。统计眼科中成药中有保健功能的产品可知,使用频次≥4的中药原料有7味,累计使用32次(29%),由高到低分别是枸杞子、决明子、菟丝子、熟地黄、菊花、五味子、车前子。通过无监督的熵层次聚类得到1个新处方。结论保健食品在中药原料的选择上与中医药理论治疗视疲劳的原则基本契合,即滋补肝肾、平肝明目。但二者在中药原料选择范围、原料配伍、剂型种类等方面有所不同。此外,还在辨证保健理论指导下,尝试探讨应用文献数据处理统计方法筛选新配方、开发新产品,为中药复方保健产品的研发开辟了新思路、新方法。     

一腓骨肌萎缩症家系临床及基因分析

目的分析一腓骨肌萎缩症家系的临床表现及不同基因检测方法的特点。方法收集一CMT家系8名成员临床资料,并应用等位基因特异性PCR-双酶切方法及多重连接依赖的探针扩增技术(MLPA)检测PMP22基因突变情况,同时选择60名性别、年龄无明显差异的健康人做为对照组。结果该家系中患病者以行走不稳、跨阈步态,伴有弓形足为主要临床表现。该家系中5名成员经等位基因特异性PCR-双酶切及MLPA方法均检测出PMP22基因重复序列,其中出现临床症状的有4名(Ⅱ3、Ⅱ9、Ⅱ11、Ⅲ7),未出现临床症状但基因检测结果示PMP22基因重复序列的为携带者有1名(Ⅲ5),家系中余3名成员及对照组60名均未见重复序列。结论基因检测在明确CMT诊断中起重要作用,且MLPA法筛查基因时操作更简便、灵敏度更高、特异性更好。     

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??Abstract?? Systemic vasculitis is a group of vasculitis with common inflammation of the blood vessel and involves multiple organs. It can involve all kinds of vessels of the organs??showing different clinical manifestations and lacking specificity. Through summarizing the clinical manifestation and diagnosis of some common systemic vasculitis in children??we aim to improve pediatricians’s awareness of these diseases??in order to early discover??diagnose and improve the prognosis.