左乙拉西坦治疗儿童失神癫痫的疗效及安全性观察

目的观察左乙拉西坦治疗儿童失神癫痫的疗效及安全性。方法2009年1月至2010年6月前瞻性入选我院收治的55例儿童典型失神癫痫患者,分组采用左乙拉西坦（研究组）、丙戊酸（对照组）单药治疗,并进行1年的治疗和随访观察,比较两组患者治疗前、治疗6个月及12个月时的癫痫发作频率、脑电图异常率、临床有效率及不良反应发生情况。结果研究组25例、对照组30例均完成了治疗1年的随访评估。两组患者治疗后癫痫发作频率均无较治疗前加重情况;两组治疗6个月和12个月时癫痫发作频率（t值分别为0．760、0．670,P值分别为0．450、0．504）和脑电图异常率（χ2值分别为0．120、0．043,P值分别为0．729、0．836）比较差异无统计学意义;两组患者治疗后6个月、12个月时临床有效率比较差异无统计学意义（χ2值分别为0．051、0．078,P值分别为0．821、0．780）。研究组2例（8．0％）出现轻微不良反应（1例烦躁,1例嗜睡）;对照组2例（6．7％）出现轻微不良反应（1例体重明显增加,1例出现恶心、呕吐）。两组患者治疗后无死亡等严重不良事件发生。结论左乙拉西坦作为一种新型抗癫痫药物对于儿童失神癫痫的单药治疗疗效肯定,并显示了较好的安全性。     

螺旋CT对外耳道病变的诊断价值

目的提高对外耳道病变螺旋CT影像的认识和重视。方法对经病理证实的34例患者的36耳CT影像进行回顾性分析。结果本组36耳外耳道病变中,其中外耳道胆脂瘤伴肉芽者15耳,外耳道胆脂瘤6耳,外耳道乳头状瘤6耳,外耳道骨瘤1耳,外耳癌2耳。外耳道肉芽5耳,血管瘤1耳。结论外耳道病变虽较少行CT检查,但CT对外耳道疾病的诊断及鉴别诊断有重要价值。     

内耳畸形患者的人工耳蜗植入术

目的：探讨内耳畸形患者人工耳蜗植入手术的方法和临床经验。方法：1997-2001年共实施多导人工耳蜗植入术180例,其中伴有双侧内耳畸形患者25例。就其不同原因所致内耳畸形患者的人工耳蜗植入手术特点及术后开机调试特点与耳蜗形态正常的人工耳蜗植入进行了比较。结果：（1）所有内耳畸形患者人工耳蜗植入术后都有听觉;（2）内耳畸形人工耳蜗术中容易出现“镫井喷“,其中Mondini畸形术中出现“镫井喷“的现象比其他畸形多见;（3）植入电极大部分完成插入蜗内,少数遗留蜗外电极;（4）术后无面瘫、脑脊液瘘等并发症;（5）术后开机调试发现部分数值比正常形态耳蜗植入偏高,但听阈无明显区别。（6）经过语言康复训练,听力及言语能力均有不同程度的提高。结论：伴有内耳发育畸形患者的深度感音性聋可以行人工耳蜗植入,植入效果与耳蜗发育正常患者的人工耳蜗植入效果基本一致。     

癫痫患者P50听觉诱发电位的研究

目的 探讨Ｐ５０在癫痫患者检测中的临床价值。方法 对２５例癫痫患者进行Ｐ５０测试,分析Ｐ５０波的潜伏明和波幅以及在不同刺激频率时Ｐ５０波幅的比率。选择年龄、性别２与患者组相匹配的２５名健康受试者对照组,结果 癫痫患者组Ｐ５３波的潜伏期较长,波幅则显著减低,其中以１８例颞叶癫痫２表现显著,７例额叶癫痫２不明显。刺激频率稍加快时,患者组Ｐ５０的波幅比率明显减小。结论Ｐ５０波的潜伏期和以应异常与颞叶功能     

儿童特发性癫痫与睡眠结构的关系

目的研究特发性局灶性癫痫和全面性癫痫患儿的整体睡眠结构参数并与无癫痫的对照组比较,探讨睡眠结构与癫痫的相互关系。方法对癫痫组和对照组共47人行多导睡眠图检查(polysomnography, PSG)并分析其结果。47例中18例为特发性局灶性癫痫(idiopathic partial epilepsy, IPE),13例为特发性全面性癫痫(idiopathic generalized epilepsy,IGE)。16例为对照组,年龄及性别均与癫痫组相匹配。结果结果显示IPE组总记录时间增加(P<0.05)而IGE组非快速眼动(non-rapid eye movement, nREM)睡眠4期睡眠减少。IGE组整个睡眠期的效率降低(P<0.05)全部睡眠时间均减少(P<0.05)。结论特发性癫痫患儿睡眠结构发生了变化,其中IGE组的睡眠质量下降,而IFE组显示较多的睡眠。     

儿童特发性癫痫与睡眠结构的关系

目的研究特发性局灶性癫痫和全面性癫痫患儿的整体睡眠结构参数并与无癫痫的对照组比较,探讨睡眠结构与癫痫的相互关系.方法对癫痫组和对照组共47人行多导睡眠图检查(polysomnography,PSG)并分析其结果.47例中18例为特发性局灶性癫痫(idiopathic partial epilepsy,IPE),13例为特发性全面性癫痫(idiopathic generalized epilepsy, IGE).16例为对照组,年龄及性别均与癫痫组相匹配.结果结果显示IPE组总记录时间增加(P＜0.05)而IGE组非快速眼动(non-rapid eye movement,nREM)睡眠4期睡眠减少.IGE组整个睡眠期的效率降低(P＜0.05)全部睡眠时间均减少 (P＜0.05).结论特发性癫痫患儿睡眠结构发生了变化,其中IGE组的睡眠质量下降,而IFE组显示较多的睡眠.     

Aetiological factors in Tanzanian epileptics

The study deals with 428 patients with epilepsy who were referred to the neurology clinic, Muhimbili Medical Centre, Dar es Salaam; and analysed for aetiological factors. Of these, 146(34%) had identifiable causes which were acquired in 95%. Sixty seven (46%) of patients with identifiable causative factors were children below the age of fifteen and 35(24%) adults were above the age of thirty. The cause of epilepsy in 71% of children was childhood febrile convulsions and CNS infections while in 74% adults, it was due to head injury, tumours and vascular disease. Twice as many patients with partial epilepsy had identifiable aetiological factors than those with generalised epilepsy. Childhood febrile convulsion was commonly associated with partial epilepsy with complex symptoms while birth injury and CNS infections were associated with generalised epilepsy. Vascular causes and tumours were associated with partial epilepsy. The significance of identifiable aetiological factors and their association to various types of epilepsy is discussed. This study is a preliminary report of a major study which commenced in January, 1983 dealing with classification, clinical presentation, social factors and management of epileptic patients referred to this Clinic.     

Eustachian tube dysfunction in an intensive care unit: A prospective observational study

BackgroundEustachain tube (ET) dysfunction can aggravate the morbidity in ICU patients, but is often ignored. In this prospective observational study we followed a cohort of patients (without pre-existing ET dysfunction) requiring in-patient management, hospitalized either to ICU or to non ICU wards, for any development of ET dysfunction during hospitalization.MethodsPatients requiring hospitalization to ICU or non ICU wards from Dec 2018 to Jun 2019 were included. Those with pre-existing ET dysfunction, disease of ear/nose or sinuses, head neck tumours and history of radiotherapy or glucocorticoid therapy were excluded. All patients were evaluated by serial tympanometry. Type A curve was considered normal while type B, C1 and C2 were considered as ET dysfunction.ResultsThere were 385 patients, 258 (67.01%) males and 127 (32.99%) females in the ICU group; while non ICU group comprised 129 patients, 86 (66.67%) males and 43 (33.33%) females. ET dysfunction developed in 107 (27.79%) patients in ICU group, but only in 3 (2.3%) in non ICU group (Relative risk 11.95, 95% CI 3.86 to 36.99, P < 0.0001). Within ICU, ET dysfunction showed significant positive association with endotracheal intubation, Ramsay sedation score and number of days on tracheostomy; but not with age, male gender, number of days in ICU, mechanical ventilation or route of feeding.ConclusionOur study demonstrates high incidence of ET dysfunction in patients admitted to ICU.     

外耳道囊性腺样癌的手术治疗

目的：研究外耳道囊性腺样癌的临床手术治疗，方法：回顾分析我院1987－2001年收治的经病理确诊为外耳道囊性腺样癌患者12例，男7例，女5例，年龄27－60岁，平均41．3岁。6例局限于外耳道者行外耳道磁样切除加部分外耳道骨壁磨除或乳突根治术；5例侵及范围广累及中耳、乳突，甚至岩锥者行颞骨侧位切除加腮腺部分或全切除术；1例侵及颅内者行颞骨切除及侧颅底手术，切除颅内肿瘤并以帽状腱膜、带蒂颞肌瓣修补。结果：1例随访1年，9例随访均在2年以上，最长者13例，其中8例（初诊4例，复发者4例）术后无复发，2例复发并有肺转移，其中1例死亡，1例带癌生存至今，2例失访，结论：广泛彻底手术切除是治疗本病的关键。     

The association of uvulo-palatoglossal junctional ulcers with exanthem subitum: a 10-year paediatric outpatient study

A 10-year follow-up of children having exanthem subitum (ES) seen in an outpatient paediatric clinic, Kuala Lumpur, Malaysia shows that uvulo-palatoglossal junctional (UPJ) ulcer is a reliable early clinical sign of ES. During this period, 1,977 children (1,086 males, 891 females) had adequate follow-up from the age of 3 months to 24 months old. 897 children (478 males, 419 females) were noted to have UPJ ulcers. Of these 897 children, 855 (459 males, 396 females) presented with the classical clinical features of ES of maculopapular rash following 3 to 4 days of fever. The positive predictive value and the negative predictive value of UPJ ulcers in the clinical diagnosis of ES are 95.3% and 100% respectively. Among the 855 children with clinical features of ES, a provisional diagnosis of ES could be made in 781 children during the pre-eruptive phase by the presence of the UPJ ulcers. The other 74 children already had the rash at the time of consultation at the clinic. The peak age of occurrence of ES was 6 months old with 98.2% of the total cases of ES seen between the age of 4 and 12 months. There was no significant gender difference in the incidence of ES nor any seasonal variation. Mild to moderate diarrhoea was the other commonly associated clinical feature which usually presented from the third febrile day onwards.     

聋哑儿童听力语言训练探讨

报告对138例聋哑儿童的语言训练,其中,男85例,女53例,先天性聋哑20例,后天性聋哑118例,5岁以下聋哑者125例。认为主要是利用感觉途径的训练方法,叫作“多感觉方法”,即“看,听,摸”。作者体会聋童语言训练是否成功,家长是一个很重要的决定因素。对于聋童做好早期诊断,及时矫治是防哑的关键。     

Epilepsy awareness among parents of school children--a municipal survey

A pilot study was undertaken to assess the knowledge, attitude and practice of parents of school-going children in a semi-urban municipal area of Bengal. An open-ended questionnaire with 14 questions were put in local language. A total of 1068 parents were interviewed with 76.9% males and 23.1% females. Most of them (60.7%) having an educational level above primary but below higher secondary school level; 41.4% surveyed believe epilepsy is caused by an infection and 40.8% have the idea that it is contagious too; 80.3% have the idea that epilepsy can be treated but 7.3% interviewed carry the idea that it cannot be treated at all. Only 35.9% responders are ready to allow an epileptic child to attend school and sit together with other children. In terms of first-aid management only 36.6% mentioned some useful first-aid measure. The general public attitude is reflected in this study. False belief and negative attitude leads to barrier in children education. Larger and comprehensive community-based educational programme is very much essential to bring about a change in negative attitude towards epilepsy.     

模拟帕金森病的表达人α-synucleinA53T转基因小鼠的早期嗅觉功能观察

目的通过对表达人α-synucleinA53T的转基因小鼠嗅觉功能的检测和比较,确立一个可供研究帕金森病早期嗅觉障碍发病机制的模型。方法选取不同月龄的表达人α-synucleinA53T的转基因(TG)小鼠与同窝野生型(WT)对照小鼠;转棒试验评估10月龄TG小鼠随意运动功能是否发生变化;DAB法观察10月龄小鼠脑黑质多巴胺能神经元,进一步验证小鼠是否发生运动功能改变。通过气味鉴别及适应试验,观察小鼠对已熟悉的同种气味的短期记忆能力及适应能力,判断小鼠对陌生气味的辨别能力;通过长间隔记忆试验,检测小鼠对已经暴露过的气味在一定间隔时间后再次暴露的记忆能力;通过隐藏颗粒试验,检测小鼠对食物气味的感知能力,反映嗅觉阈值的水平。结果转棒试验和多巴胺能神经元观察结果均显示10月龄TG小鼠的随意运动功能尚未发生改变。6月龄TG小鼠出现对气味辨别能力的下降,对新旧两种气味的识别时间比较,差异无统计学意义(P=0.120);10月龄TG小鼠表现出更明显的气味识别缺陷(P=0.295)。6月龄TG小鼠寻找到食物的时限长于WT小鼠(P=0.015)。各年龄段小鼠短期记忆及适应能力均正常;但9月龄TG小鼠表现出长期(两次试验间隔时间60、80、100 min)记忆能力减退。结论表达人α-synucleinA53T的转基因小鼠在运动功能改变之前出现嗅觉障碍,表现在气味辨别、记忆、感知能力等方面,能够很好地模拟帕金森病早期嗅觉障碍的表现。     

婴幼儿体外循环术后继发癫痫20例分析

目的 探讨婴幼儿体外循环(CPB)术后继发癫痫的临床特征、治疗和预防。方法 1995—05—2002—05对20例婴幼儿先心病CPB术后继发癫痫的年龄、脑电图(EEG)、MRI等方面进行分析综合。结果 20例中，男14例，女7例。首发年龄：2个月一14岁，平均4岁2个月；其中小于6岁的婴幼儿多在CPB术后7d发病。微小发作(minimal seizure)及全身发作分别8例和5例。脑电图显示l周后多呈局限性或弥漫性慢波，异常放电波增多。6例行MRI检查提示局限性T2信号增强。结论 婴幼儿发病较早，年龄大者发病较晚，EEG及MRI有助于早期诊断和预后判断，对婴幼儿CPB术后癫痫治疗的重点应治疗和预防相结合。     

认知功能障碍评定方法的研究进展

认知是指人对事物的认识和知晓过程,其涉及到的内容较多,如患者的记忆、思维、推理等,是人类疾病较高的神经活动过程。而大脑对于认知的功能障碍则主要包括了感觉、记忆、思维和语言等。认知功能障碍对日常生活的影响远远大于躯体功能障碍,其反映的不仅仅是对社会的适应能力,并且还会给家庭、社会等带来很大损失。随着人们生活方式的改变,对于认知功能的康复也给予了更为广泛的关注。本文就认知功能障碍的评定方法进展做一综述。     

Ningbo thyroid dysfunction prevalence study: a cross-sectional survey in an employees-cohort

Background The prevalence and the spectrum of thyroid dysfunction in the mainland of China are not adequately understood. We performed a population-based study to determine the prevalence of major thyroid dysfunctions including overt and subclinical hyper- and hypothyroidism in a stable cohort. Methods All active and retired employees aged 20 years and older （11 067） of Sinopec Zhenhai Refining ＆ Chemical Company in Ningbo participated in the cross-sectional survey with a questionnaire and blood samples. Results A total of 10 405 individuals attended for screening. Using biochemical definitions 95.5% were euthyroid. The prevalence of former diagnosed hyperthyroidism was 1.1% in females and 0.4% in males, hypothyroidism 1.7% and 0.3%, and thyroid surgery 1.2% and 0.3%, respectively. In both sex the prevalence increased with age. Twenty-four percent of individuals with thyroid surgery or medications had abnormal thyroid-stimulating hormone （TSH） levels. In individuals without a history of thyroid disease, the prevalence of pathological TSH values in females and males were TSH 〉10 mU/L 0.60% and 0.29%; TSH 4.8-9.9 mU/L 5.71% and 2.25%; TSH 〈0.3 mU/L 0.87% and 0.41%, respectively. Overt hyper- and hypothyroidism were uncommon （0.2%, 0.3%, respectively）. The prevalence of subclinical hyper- and hypothyroidism was 0.4% and 3.4%, respectively. Subclinical hypothyroidism was more common in females （male 2.4% vs. female 5.8%, P 〈0.001） and with increasing age （P 〈0.001）. Conclusions The prevalence of thyroid dysfunction is 4.5% in the cohort. Among individuals with thyroid medications or surgery, only 75.7% were within the normal range of TSH. These results indicate that thyroid dysfunction is common in Chinese adults.     

56例难治性癫痫患儿遗传代谢异常筛查

目的 探讨小儿难治性癫（痫）（IE）不同遗传代谢异常情况,以利早期进行病因及对症治疗.方法 运用气相色谱-质谱（GC/MS）和串联质谱（MS/MS）技术对56例IE患儿尿和血标本进行包括氨基酸、有机酸及脂肪酸等代谢异常筛查,并进行尿常规、肝功能、血生化、脑部影像学及脑干听、视觉诱发电位等检查.结果 56例IE患儿中,25例（44.6％）尿筛查异常,其中全面性发作6例（24.0％）,部分性发作5例（20.0％）,全面性或部分性等多种形式发作2例（8.0％）,婴儿痉挛症12例（48.0％）.46例IE患儿进行了血筛查,13例异常.27例尿或血筛查异常IE患儿中19例智力运动发育落后或倒退（70.4％）,头颅影像学异常及脑干诱发电位异常各15例（55.6％）,血生化、血气分析异常及异常家族史各6例（22.2％）,肝功能异常及皮肤改变各5例（18.5％）.11例（19.6％）IE患儿尿或血筛查结果呈典型改变,确诊为先天性遗传性代谢病.结论 遗传代谢异常为IE常见致病原因,尤以婴儿痉挛症多见.因此对IE患儿应尽早进行先天性代谢异常筛查,以助早期诊断及治疗。     

先天性心脏病手术后并发多脏器功能障碍综合征的临床分析

目的总结小儿心脏手术后多脏器功能障碍综合征（MODS）病例的临床特点,探讨发生MODS患者中导致死亡的危险因素和防治措施。方法收集2001年1月至2010年12月本科先天性心脏病术后并发biODS患者35例,男21例,女14例;年龄（6．43±4．65）岁;体重（16．52±9．12）kg。将其中并发MODS的患者分为死亡组与存活组,进行比较,回顾性分析导致死亡的高危因素,总结脏器衰竭数目及MODS评分与病死率的关系。结果本组病死率为51．4％（18／35）。①受累器官的发生率依次为心脏100．0％（35／35）、肺脏80．0％（28／35）、肾脏60．0％（21／35）、肝脏14．3％（5／35）、血液系统11．4％（4／35）及中枢神经系统5．7％（2／35）,其中心、肺出现受损的时间最早。②脏器衰竭数目在2、3、4、≥5个时对应的病死率分别是25．O％、45．5％、77．7％、100．0％;MODS评分与本组病死率亦呈显著正相关。③与存活患者相比,死亡患者体外循环时间、主动脉阻断时间明显延长,二次转机、围术期意外事件发生率显著增高（P〈0．01）。结论心肺功能受损是导致小儿心脏术后MODS的原发病,MODS死亡率随脏器衰竭数目和MODS评分的增加而递增,死亡的高危因素为体外循环时间及二次转机、围术期意外事件,重在预防。     

儿童Williams-Beuren综合征九例临床分析

本文选取2016年3月—2017年2月重庆医科大学附属儿童医院收治的9例Williams-Beuren综合征患儿为研究对象,对其临床资料进行回顾性分析,发现儿童Williams-Beuren综合征的高危因素包括母亲孕期感冒、羊水异常、足月小样儿、孕期过长、剖宫产、前置胎盘及宫内发育迟缓。患儿均有特殊面容,且生长、智力、运动及语言均发育迟缓。肌张力异常4例,癫痫4例,高血钙症状3例;合并先天性心脏病7例。颅脑MRI检查结果异常2例,腰骶部MRI检查结果异常1例,消化系统异常1例,颅缝早闭8例,行为异常6例。综上,儿童Williams-Beuren综合征高危因素较多,且患儿临床特点较为多样,应引起重视。     

癌症患者性功能障碍相关因素分析

目的：探讨癌症患者性功能障碍的相关因素，以利于开展癌症患者性功能障碍的防治工作以及提高患者生活质量。方法：以统一自制的性功能状况问卷，对130例患各类癌症的康复期患者进行了问卷调查。结果：患者年龄和认知因素在患者性功能障碍患病率上存在显著差异；生殖系统恶性肿瘤患者其性功能障碍的总体患病率明显高于其他癌症患者。在调查因素中，肿瘤分期、患者体力状况和认知因素与患者新发性功能障碍的发生显著相关。患癌症后新发性功能障碍的患者普遍认为针对癌症的治疗对其性功能产生了不良影响。结论：癌症患者治疗后性功能障碍的发生与肿瘤分期、患者体力状况和认知因素显著相关；提示防治癌症患者性功能障碍首先要针对患者的认知错误进行干预。