吉兰—巴雷综合征与空肠弯曲菌感染

吉兰-巴雷综合征是自身免疫介导的周围神经病,主要的病理改变是周围神经组织小血管淋巴细胞、巨噬细胞浸润,神经纤维脱髓鞘,严重病例可继发轴索损害。吉兰—巴雷综合征的发生是由于易感人群感染病原菌后,机体针对病原菌抗原成分产生相应抗体,该抗体与人体神经节苷脂发生交叉免疫反应;同时在多种细胞因子的协同作用下,影响神经传导功能,最终产生不同程度的髓鞘脱失,严重者可导致轴索变性。空肠弯曲菌是其中最常见的病原菌,本文就吉兰—巴雷综合征与空肠弯曲菌感染的关系予以综述。     

糖皮质激素联合免疫球蛋白治疗机械通气的吉兰巴雷综合征的临床疗效分析

目的:分析糖皮质激素联合免疫球蛋白治疗需要机械通气的吉兰巴雷综合征的临床疗效。方法:回顾性分析2008年1月至2016年7月西安市六家医院收治的采用机械通气的吉兰巴雷综合征患者的临床资料,并对其进行随访,比较单用免疫球蛋白与糖皮质激素联合免疫球蛋白的疗效差异。结果:两组在出院时及出院后6个月的总有效率(MRC及HFGS的改善率)、死亡率、机械通气时间、住院时间、在ICU住院时间、出院时及出院后6个月的MRC总得分和HFGS得分以及并发症发生率比较差异均无统计学意义(P0.05)。结论:对于机械通气的吉兰巴雷综合征患者,在使用免疫球蛋白的基础上增加糖皮质激素的使用,对其短期(出院时)及较长期(出院后6个月)的临床疗效和安全性均无显著影响。     

鹦鹉热衣原体感染致复视1例

鹦鹉热衣原体是一种胞内寄生的革兰阴性菌,可引发人畜共患病。鹦鹉热衣原体感染多表现为呼吸道症状,在中枢神经系统受累时通常表现为脑膜炎、颅神经麻痹、吉兰巴雷综合征以及癫痫。本文介绍了1例鹦鹉热衣原体致重症肺炎且合并颅神经损害的病例,经过多西环素抗感染治疗后肺部感染及颅神经病变好转。该病例报告不仅帮助临床医师进一步熟悉鹦鹉热衣原体感染累及中枢神经系统的临床表现,更有助于其了解鹦鹉热病原体目前的诊断手段。     

糖尿病酮症酸中毒合并吉兰-巴雷综合征2 例临床分析

目的:研究糖尿病酮症酸中毒(diabetic ketoacidosis,DKA)合并吉兰-巴雷综合征(Guillain-Barrésyndrome,GBS)的临床特点,以探讨其临床表现、治疗、预后及发病机制。方法:回顾性分析2例DKA合并GBS患者的病例资料,对其病史、临床表现、电生理学、脑脊液改变、治疗进行总结。结果:2例DKA患者均急性起病,两例患者发病前1周均有呼吸道感染史,尽管DKA得到了纠正,患者出现呼吸衰竭后被发现四肢力弱。肌电图提示周围神经损害。脑脊液示细胞蛋白分离。给予免疫球蛋白后,例1在出现肢体瘫痪后18天恢复至正常,遗留有四肢末端麻木感,例2在出现肢体瘫痪后1年肌力恢复正常。结论:DKA合并GBS临床少见,多发生在DKA纠正后一周左右出现四肢迟缓性瘫痪,早期给予免疫球蛋白治疗,预后相对良好,目前发病机制尚不清楚。     

194例吉兰巴雷综合征患者的临床特点分析

目的:研究吉兰-巴雷综合征(Guillain-Barre Syndrome,GBS)患者的的临床特点,分析GBS病情严重程度的影响因素。方法:回顾性分析西京医院2011年9月至2016年1月收治的194例GBS患者的病历资料,分析GBS患者的人口学特征及临床特点及与病情轻重、是否需要机械通气相关的因素。结果:194例GBS患者中,男性多于女性,青中年发病比例最高,平均年龄为42.91±17.72岁,秋夏季为高发季节,前驱事件以呼吸道感染最多见,首发症状以运动障碍最多见,需要机械通气者占12.37%(24/194)。发病高峰期重型患者占58.76%(114/194)。临床分型中最多见分型为纯运动型77.84%(151/194)。通过对GBS患者起病急性期病情轻型与重型两种分型临床资料的比较发现,有无运动障碍、有无颅神经损伤、是否需要机械通气、高峰期MRC、高峰期Hughes评分5种因素在两组间差异有统计学意义(P0.05)。在GBS患者机械通气及非机械通气两组之间,运动障碍、病情轻重、高峰期MRC评分、高峰期Hughes评分4种因素在两组间差异有统计学意义(P0.05)。结论:GBS患者男性多于女性,秋夏季为高发季节,轴索型为主要电生理分型。急性期有无运动障碍、高峰期MRC、高峰期Hughes评分3种因素为影响病情轻重及是否需要机械通气的共同影响因素。     

临床循证实践面临的困难与对策

进20年来,循证医学获得长足发展,然而临床循证实践与循证医学两者之间还存在巨大的鸿沟,医师临床循证实践的开展情况仍受到各种因素的制约。文章从科学证据、医护人员、患者、组织和卫生系统等五个方面对科学证据转化为循证实践面临的困难进行了系统回顾,并从临床循证实践指南开发、医学教育、信息化建设、医疗机构管理和加强卫生系统的作用等角度提出了促进循证实践开展的建议。

     

循证医学在慢性充血性心力衰竭药物治疗中的应用

循证医学(evidence-based medicine,EBM)是近年来国际临床医学领域迅速发展起来的新学科,强调"为每位病人作出医疗决定时,应明确而仔细地使用现有的最好证据"。本文利用循证医学的方法,参照若干著名的大规模多中心的随机对照试验(ran- domized controlled trial,RCT)来评估目前几类临床常用药物在治疗慢性充血性心力衰竭中的作用,试图找出针对不同NYHA等级患者的最佳治疗建议。     

抑郁症治疗的循证医学研究进展

循证医学是近年来国际上临床医学领域迅速发展起来的新学科,已成为当前国际医学研究中的热点之一,是指对病人的诊 断、治疗、预防、康复和其他决策应建立在当前最佳临床研究证据,是遵循证据的临床医学,强调收集最佳证据。其理念的科学性 和有效性迅速渗透到医学领域的众多学科,循证精神卫生也相继提出并发展。抑郁症是由各种原因引起的以抑郁为主要症状的 一组心境障碍或情感性障碍,近几年能够有效运用循证医学方法为治疗抑郁症寻找最佳临床证据,制定循证诊疗指南成为了临 床医生的迫切要求。本文就抑郁症治疗方面的循证医学研究进展做一综述。     

循证医学在急诊住院医师培训中的应用

循证医学（Evidense—based medicine,EBM）是一门新兴临床学科,著名流行病学专家David Sackett教授将循证医学定义为：慎重、准确和明智地应用能获得的最好研究依据来确定患者的治疗措施。其实质就是医生诊治患者的一个过程,是精益求精地认识疾病的本质,从而去寻求最佳证据,并联系的实际情况,取得最佳治疗效果。循征医学的3个基本要素是临床医生的个人专业技能、最好的科学证据、患者的价值和愿望。     

成人急性感染性腹泻治疗新进展

成人急性感染性腹泻与儿童感染性腹泻在发病率、严重程度、预后等方面有所不同,其治疗以补液、止泻为主,必要时使用抗微生物药物。近年来,成人急性感染性腹泻在补液治疗和益生菌、新止泻药物应用等方面更新了一些新的循证医学证据,在进一步确保现有药物安全性的同时促进了腹泻治疗思路与手段的发展。     

Miller–Fisher syndrome associated with SARS-CoV-2: a case report

SARS-CoV-2 infections are increasingly associated with neurological complications, including immune-mediated neuropathies. Miller–Fisher syndrome is a rare variant of Guillain-Barré syndrome characterised by the triad of ataxia, ophthalmoplegia and areflexia.Here we present a case of Miller–Fisher syndrome following COVID-19 infection. The clinical presentation was a short history of a rapidly-progressive peripheral sensorimotor neuropathy with bulbar dysfunction and facial weakness following mild COVID infection. Examination revealed global areflexia and a broad-based ataxic gait. CSF analysis revealed albuminocytological dissociation and neurophysiological testing later supported the diagnosis. The patient required high flow nasal oxygen therapy for respiratory dysfunction in a level 2 care setting and received immunological treatment with intravenous immunoglobulins.We conclude that Miller–Fisher syndrome needs to be considered in patients presenting with new sensorimotor dysfunction following SARS-COV-2 infection. Early recognition is key given the propensity to cause life-threatening respiratory failure, and early administration of immunological treatment is associated with improved prognosis.     

Antibodies against gangliosides: a link between preceding infection and immunopathogenesis of Guillain-Barré syndrome

Autoantibodies against gangliosides GM1 and GQ1b, characteristic cell surface glycolipids of the nervous system, are present in specific clinical types of GuillainBarré syndrome (GBS). Close associations of anti-GM1 with acute motor axonal neuropathy, and of anti-GQ1b with Miller Fisher syndrome, strongly suggest that these antibodies contribute to neuropathy pathogenesis. Immune responses against gangliosides are suspected to originate as a result of molecular mimicry between gangliosides and lipopolysaccharides of Campylobacter jejuni, the most frequent infectious trigger of GBS. Thus, antibodies against gangliosides may link C. jejuni infection with the precipitation of neurological disease.     

Studies of cardiac ganglia in pre- and postnatal rabbits

Summary This investigation was undertaken to describe the ultrastructure of cardiac ganglia in rabbits from day 18 of gestation to day 35 postpartum. Special attention was directed to the types of synaptic contacts made with the principal neurons and with the small granule-containing cells. The cardiac ganglia in all animals consisted mainly of parasympathetic postganglionic neurons, supporting cells, and small granule-containing (small intensely fluorescent) cells. The neurons received afferent synaptic terminals of two types. One type contained mainly small clear vesicles typical of most cholinergic terminals. The second type contained mainly small dense-core vesicles (these were most prominent after treatment of the animal with 5-hydroxydopamine), and were considered to be adrenergic terminals. These adrenergic terminals are probably part of an inhibitory system in the ganglia. The small granule-containing cells received typical afferent synaptic terminals of the cholinergic type, and also formed specialized contacts with certain axonal terminals. These latter specializations are considered to be reciprocal synapses which probably have a role in modulating ganglionic transmission.Supported by the Kentucky Heart Association and the Heart Association of Louisville and Jefferson County     

Families with recurrent cases of Waardenburg-Klein syndrome

Deaf children with the type I Waardenburg--Klein syndrome were studied. Secondary cases were found in 14 unrelated and 1 incest families. In 10 families probands and all their affected relatives had the type I Waardenburg--Klein syndrome. In 4 families patients were discovered with both type I and type II syndromes. In an incest family the proband seemed to have the type III, while his mother and father (sibs) had type II and their grandmother the type I syndrome. These results contradict the hypothesis claiming the origin of different types of Waardenburg--Klein syndrome to be due to the action of different genes. It is proposed that types I and II, or all types of the syndrome are caused by a single dominant gene. Potential mechanisms for clinical polymorphism of Waardenburg--Klein syndrome are related to incomplete penetrance and varying expression of this gene.     

Complex Gangliosides as Autoantibody Targets at the Neuromuscular Junction in Miller Fisher Syndrome: A Current Perspective

Glycosphingolipid biology has increasingly interfaced with the field of human autoimmune neuropathy over the last two decades. There are currently over 20 distinct glycolipids that have been identified as autoantibody targets in a wide range of clinical neuropathy syndromes. This review sets out the clinical and experimental background to one interesting example of anti-glycolipid antibody-associated neuropathy termed Miller Fisher syndrome. This syndrome, comprising the triad of ataxia, areflexia, and ophthalmoplegia, correlates highly with the presence of serum anti-GQ1b antibodies, arising through molecular mimicry with microbial oligosaccharides. Anti-GQ1b antibodies mediate neural injury through binding to GQ1b-enriched sites in the peripheral nervous system, including extraocular nerves. Animal experimental evidence, along with a hypothetical background, indicates the motor nerve terminal may be a key site for anti-GQ1b antibody binding with consequent defects in synaptic transmission, as occurs in botulism and other toxinopathies. Our work in recent years on this hypothesis is summarized.     

New categories designated as healthcare‐associated and community‐associated methicillin‐resistant Staphylococcus pseudintermedius in dogs

The incidence of MRSP has been increasing, and treatment options in veterinary medicine are limited. Few previous studies of MRSP have described the relationships between the genotypes, phenotypes, and clinical backgrounds of the isolates. To gain insight into the associations between the microbiological and clinical characteristics of MRSP, we analyzed 282 Staphylococcus pseudintermedius isolates from dogs. A total of 195 (69.1%) strains were identified as mecA‐positive MRSP and were classified into mainly two genotypes: SCCmec types III (II‐III) (52.8%) and V (37.4%). SCCmec type III MRSP strains were significantly correlated with hospital admission and antimicrobial therapy of the dogs, and exhibited a homogeneous genotype similar to sequence type 71‐MRSP, which is a globally endemic clone in dogs. In contrast, SCCmec type V MRSP strains were not highly correlated with hospital admission and antimicrobial therapy and exhibited genotypic and phenotypic heterogeneity. Properties of MRSP strains SCCmec types III and V were similar to those of HA‐ and CA‐MRSA, respectively. Therefore, we designated these isolates carrying SCCmec types III and V as HA‐MRSP and CA‐MRSP, respectively. Discrimination between HA‐ and CA‐MRSP by oxacillin MIC will provide useful information for treatment and infection control measures for canine MRSP infections.     

Antibodies against gangliosides and ganglioside complexes in Guillain–Barré syndrome: New aspects of research

Guillain–Barré syndrome (GBS) is an acute autoimmune neuropathy, often preceded by an infection. Serum anti-ganglioside antibodies are frequently elevated in titer. Those antibodies are useful for diagnosis. Some of them also may be directly involved in the pathogenetic mechanisms by binding to the regions where the respective target ganglioside is specifically localized. We have recently found the presence of the antibody that specifically recognizes a new conformational epitope formed by two gangliosides (ganglioside complex) in the acute-phase sera of some GBS patients. In particular, the antibodies against GD1a/GD1b and/or GD1b/GT1b complexes are associated with severe GBS requiring artificial ventilation. Some patients with Miller Fisher syndrome also have antibodies against ganglioside complexes including GQ1b; such as GQ1b/GM1 and GQ1b/GD1a. Gangliosides along with other components as cholesterol are known to form lipid rafts, in which the carbohydrate portions of two different gangliosides may form a new conformational epitope. Within the rafts, gangliosides are considered to interact with important receptors or signal transducers. The antibodies against ganglioside complexes may therefore directly cause nerve conduction failure and severe disability in GBS. More study is needed to elucidate the roles of the antibodies against ganglioside complexes.     

Antibodies against gangliosides and ganglioside complexes in Guillain-Barré syndrome: new aspects of research

Guillain-Barré syndrome (GBS) is an acute autoimmune neuropathy, often preceded by an infection. Serum anti-ganglioside antibodies are frequently elevated in titer. Those antibodies are useful for diagnosis. Some of them also may be directly involved in the pathogenetic mechanisms by binding to the regions where the respective target ganglioside is specifically localized. We have recently found the presence of the antibody that specifically recognizes a new conformational epitope formed by two gangliosides (ganglioside complex) in the acute-phase sera of some GBS patients. In particular, the antibodies against GD1a/GD1b and/or GD1b/GT1b complexes are associated with severe GBS requiring artificial ventilation. Some patients with Miller Fisher syndrome also have antibodies against ganglioside complexes including GQ1b; such as GQ1b/GM1 and GQ1b/GD1a. Gangliosides along with other components as cholesterol are known to form lipid rafts, in which the carbohydrate portions of two different gangliosides may form a new conformational epitope. Within the rafts, gangliosides are considered to interact with important receptors or signal transducers. The antibodies against ganglioside complexes may therefore directly cause nerve conduction failure and severe disability in GBS. More study is needed to elucidate the roles of the antibodies against ganglioside complexes.     

保守治疗颈性眩晕的研究进展

颈性眩晕是指因颈源性因素引起的以眩晕为主的综合征,多发于40岁以上,患病率非常高,临床表现为眩晕、颈头痛、耳鸣、耳聋、眼球震颤、恶心、呕吐、肩背痛、心悸、出汗等,症状不但复杂,而且不典型,患者常同时患耳鼻喉科与神经内科的疾病。研究表明,50岁以上的眩晕患者中,约50%为颈性眩晕,其伴随症状的复杂性和高患病率严重影响了患者的健康和生活。目前,颈性眩晕的发病机制、诊断标准、治疗方法仍未完全明确,常造成误诊、误治,且尚无一种治疗手段能够彻底治愈此病。手术治疗此病较少,临床多以保守治疗为主,包括手法推拿按摩治疗、牵引治疗、针灸及理疗以及中、西医药治疗、神经阻滞治疗等。本文主要对保守治疗颈性眩晕的研究进展进行了综述。     

成人胫骨平台骨折的流行病学特征及其变化趋势的回顾性研究

摘要 目的：回顾性研究成人胫骨平台骨折的流行病学特征及其变化趋势。方法：以我院2014年1月至2019年12月期间收治的1193例成人胫骨平台骨折患者为调查研究对象,根据患者收治的时间将患者分为A组(2014年1月~2015年12月收治,n=369)、B组(2016年1月~2017年12月收治,n=394)、C组(2018年1月~2019年12月收治,n=430)。对三组患者进行统一的问卷调查,并对三组患者的年龄、职业、致伤原因、骨折分型进行归纳统计,分析探讨成人胫骨平台骨折的流行病学特征及其变化趋势。结果：2014年~2019年成人胫骨平台骨折总体流行病学特征：男性患者数量多于女性患者,年龄主要在40~59岁,以从事体力劳动患者居多,骨折致伤原因中主要以交通事故和室内活动跌倒损伤为主,骨折Schatzker分型中以II型、IV型为主。三组年龄对比有差异性,年龄有逐年升高的趋势(P<0.05)。三组性别比例有显著性差异,男女性别比例差异在逐年缩小(P<0.05)。三组患者在职业类型方面也有显著性差异（P<0.05）,但均以体力劳动者居多。在致伤原因中,A组患者以交通事故致伤为主,C组患者以室内活动跌倒损伤为主,组间对比有显著差异(P<0.05)。在骨折分型中,A组以IV型为主,C组以II型为主(P<0.05)。结论：2014年至2019年成人胫骨平台骨折中男性较多,年龄以40~59岁为主,体力劳动者为高发职业,交通事故和室内活动跌倒损伤是最常见的致伤原因,好发骨折分型为Schatzker II型、IV型。变化趋势表现为骨折发病人数逐年上升,发病年龄有老龄化趋势,致伤原因和骨折分型也在逐渐变化。