丙戊酸钠治疗儿童失神癫的疗效

目的观察丙戊酸钠单剂治疗儿童失神癫的疗效。方法选择1990-04—2004-05在首都儿科研究所儿童医院诊断失神癫并首选口服丙戊酸患儿123例,发作未能完全控制者加用氯硝西泮,观察其疗效并进行追踪。结果123例患儿中,丙戊酸单药治疗后发作完全控制者105例,发作完全控制率84.4%,其余18例单用丙戊酸发作未能完全控制者,加用氯硝西泮后,发作均得到完全控制,全部患儿发作完全控制率为100%。追踪34例停药后的患儿9个月至10年,其中2例复发,占5.9%。结论丙戊酸单药治疗失神癫能使84.4%的患儿发作完全控制,发作不能完全控制者应加用氯硝西泮,二者联合用药可使患儿的临床发作得到完全控制,且起效快。     

儿童失神癫240例治疗及预后随访报告:应用改良的1989年儿童失神癫诊断标准

目的总结儿童失神癫(CAE)的治疗和预后,为CAE的合理用药和评价远期预后提供依据。方法对1999年10月至2005年12月北京市3家医院为研究CAE易感基因收集的CAE患儿的治疗用药、疗效及预后进行随访。CAE诊断标准参考1989年国际抗癫联盟(ILAE)提出的癫及癫综合征分类诊断标准,并制定了统一的CAE纳入标准和排除标准。根据CAE的选药原则进行治疗,评估CAE患儿的远期预后。结果3家医院共收集符合ILAECAE诊断标准的患儿339例,其中296例符合本研究制定的CAE纳入标准。296例患儿中有56例患儿因失访未得到远期预后随访结果,有随访结果者240例(81.1%),其中男94例(39.2%),女146例(60.8%)。失神发作起病年龄为3岁3个月至12岁,其中4～8岁174例(72.5%)。39例(16.2%)有热性惊厥史,18例(7.5%)有热性惊厥家族史和(或)癫家族史,5例(2.1%)有失神癫家族史。失神癫发作频率为每日5～50次,其中每日发作10～30次占80%。出现失神持续状态1例。伴全面强直-阵挛发作12例(5.0%)。所有患儿发作期EEG均表现为双侧对称同步的3Hz棘慢波爆发,头颅影像学检查均未见异常。治疗首选丙戊酸234例,其中217例(92.7%)于服药后3d至6个月发作完全控制,15例加用另一种药物(其中氯硝西泮7例、硝西泮4例及拉莫三嗪4例)后发作控制,余2例单用丙戊酸2年后仍有发作,但发作次数明显减少;首选拉莫三嗪4例,其中3例发作控制,1例服药1年发作未控制,改用丙戊酸1周后发作控制。2例首选托吡酯治疗,其中1例发作控制,1例服药5个月效果不明显,改用丙戊酸2个月后发作控制。240例患儿随访时间为2～7年,158例(65.8%)已停用抗癫药物,其中停药1年以上者96例,停药后均无复发;82例尚未停用抗癫药物患儿中,80例发作完全控制2年以上,仅有2例仍有失神发作。随访的240例患儿在校学习成绩为中等及以上者有171例(71.3%)。结论丙戊酸是治疗CAE的首选药物,对绝大多数患儿疗效好。少数用丙戊酸发作未控制者可选用拉莫三嗪或苯二氮类药物。典型CAE患儿远期预后良好。     

氟桂利嗪治疗癫共患偏头痛的疗效及安全性

目的探讨氟桂利嗪(FNZ)治疗儿童癫共患偏头痛的疗效及安全性。方法收集本科癫共患偏头痛患儿41例,随机分为试验组和对照组。患儿年龄(8.76±2.51)岁;男29例,女12例。试验组22例,对照组19例。试验组在抗癫药物(AEDs)治疗的基础上联用FNZ;对照组仅予AEDs治疗。于治疗3、6、9、12个月各随访患儿1次,评价其偏头痛、癫发作情况及用药的不良反应。结果 2组患儿年龄、性别、癫、偏头痛的发作状况及基础用药情况等的差异均无统计学意义(Pa>0.05)。治疗12个月2组癫控制的有效率均达95%以上,差异无统计学意义(P>0.05)。治疗3个月,试验组90.9%头痛缓解,明显高于对照组(68.4%)(P<0.05);治疗6、9、12个月,试验组85.7%、90.5%、85.7%头痛缓解,均高于对照组(73.7%、78.9%、72.2%),但差异均无统计学意义(Pa>0.05)。治疗3、6、12个月,试验组31.8%、33.3%、33.3%出现不良反应,均高于对照组(15.8%、15.8%、16.7%),但差异均无统计学意义(Pa>0.05)。结论 AEDs控制癫发作的同时,小剂量使用FNZ治疗癫共患偏头痛疗效良好,但不良反应较多。     

伴中央颞区棘波的儿童良性癫(痫)治疗前后脑电图与认知功能的对比分析

目的 通过研究伴中央颞区棘波的儿童良性癫(痫)(benign childhood epilepsy with centro-tem-poral spikes,BECT)患儿的脑电图放电指数、智力测试得分及事件相关电位P300潜伏期,分析治疗前后脑电图放电指数与认知功能的改变.方法 选取60例BECT患儿,治疗前后均监测视频脑电图、智力测试及P300,并比较三者结果之间的差异.结果 (1)BECT患儿通过左乙拉西坦、拉莫三嗪治疗后VEEG放电较治疗前明显减少,差异具有统计学意义(P＜0.05).(2)治疗3个月、6个月P300潜伏期与治疗前比较,差异具有统计学意义(P＜0.05).(3)治疗3个月言语智商、总智商无明显改善,而操作智商提高,言语智商与总智商差异无统计学意义(均P＞0.05),治疗6个月所有患儿的言语智商、操作智商及总智商均升高,差异具有统计学意义(均P ＜0.05).(4)脑电图放电指数与P300相关性分析结果(r=0.175),与智力测试的相关性分析结果(r =0.044),呈负相关.结论 BECT患儿均存在智力受损,脑电图放电越频繁,P300潜伏期延长,智力受损害越严重.左乙拉西坦、拉莫三嗪治疗后智力均有所改善,治疗时间越长,智力改善越明显.     

奥卡西平治疗癫84例

目的探讨奥卡西平(OXC)治疗儿童癫的效果及安全性。方法收集本院儿科门诊2007年6月-2011年6月确诊为癫的患儿84例。年龄4～16岁,平均(6.24±2.33)岁;病程1.0～3.5 a,平均1.6 a。全面性发作20例,单纯局限性发作26例,复杂局限性发作21例,局限继发全面性发作17例。均予OXC治疗,治疗3个月评价疗效及药物不良反应。结果 84例癫患儿中显效48例(占57.14%),有效31例(占36.90%),无效5例(占5.95%),总有效率为94.05%。曾使用其他抗癫药物疗效不佳改用OXC 16例中,显效10例(占62.50%),有效4例(占25.0%),无效2例(占12.50)%,总有效率为87.5%。14例出现轻微不良反应。结论 OXC治疗儿童癫效果好,安全性高。     

左乙拉西坦对癫患儿血清超敏C反应蛋白、S100B蛋白的影响

目的探讨左乙拉西坦对癫患儿血清超敏C反应蛋白(hs-CRP)、S100B蛋白的影响。方法 60例癫患儿随机分为对照组30例,观察组30例。对照组采用丙戊酸钠治疗,观察组采用左乙拉西坦治疗。于治疗前和治疗6个月测定患儿血清hs-CRP和S100B蛋白的表达。结果 2组临床疗效比较,观察组控制9例,显效18例,无效3例,总有效率为90.0%;对照组控制5例,显效17例,无效8例,总有效率为73.3%,2组比较差异有统计学意义(P<0.05)。观察组治疗后患儿血清hs-CRP和S100B蛋白分别为(1.38±0.61)mg.L-1和(0.516±0.204)μg.L-1,对照组治疗后分别为(2.96±1.42)mg.L-1和(0.674±0.216)μg.L-1,2组均较治疗前下降,且观察组降低程度较对照组更明显(P<0.05)。结论左乙拉西坦可通过降低hs-CRP和S100B蛋白的表达,减轻癫患儿炎性反应,从而减轻脑损伤,效果优于丙戊酸钠。     

左乙拉西坦单药或联合用药治疗婴儿癫长程保留率的回顾性分析

目的分析左乙拉西坦(LEV)单药或联合用药治疗婴儿癫的长程保留率。方法回顾性分析2006年7月至2007年6月应用LEV治疗的婴儿癫患儿的临床资料。结果 60例服用LEV的癫患儿,部分性发作20例,全面性发作19例,癫综合征21例,其中难治性癫21例。23例LEV单药治疗,37例以LEV作为添加药物联合治疗。LEV首剂量10 mg/kg·d,每日2次口服,每周加量10 mg/kg,加量调整直至取得最佳疗效和耐受性。LEV治疗6个月、1年、2年、3年及4年的保留率分别为95.0%、75.0%、60.0%、51.7%和38.3%。最主要停药原因为缺乏疗效(43.2%)。COX回归模型提示,病程1个月(RR=2.91,95%CI:1.16~7.30)及难治性癫(RR=2.30,95%CI:1.22~4.32)是患儿停药的危险因素(P均0.05)。患儿服药后发作频率较基线水平明显减少(P0.01)。至随访结束,23例未停药患儿中,有效率100%,完全缓解率69.57%。主要不良反应为倦怠乏力(56.0%),其余为睡眠增多、烦躁不安等。结论 LEV单药或联合用药治疗婴儿癫具有较好的长程保留率、良好的疗效及耐受性。     

癫伴慢波睡眠期持续性棘慢波20例

目的探讨癫伴慢波睡眠期持续性棘慢波(ESES)儿童的临床表现、脑电图特征及治疗措施。方法对2002年6月～2006年6月本院儿科收治的20例ESES患儿的临床资料进行分析,诊断明确后予丙戊酸钠、氯硝西泮、托吡酯单药或多药联合治疗,其中18例服药6个月后复查视频脑电图(VEEG),比较治疗前后脑电图变化。结果12例(60%)经单药或多药联合治疗,临床症状完全控制;3例(15%)联合药物治疗,每日发作次数减少(>50%);5例(25%)联合药物治疗无效。18例服药6个月后复查VEEG,5例(27.8%)患儿清醒期棘慢波基本消失,仅在慢波睡眠期有散在局灶或多灶性棘波或棘慢波;9例(50%)患儿清醒期全导有散在棘波、棘慢波出现,慢波睡眠期棘慢波量明显较前减少,4例(22.2%)无改善。结论单用或多种药物联合应用能控制多数癫患儿发作,但对消除电持续状态疗效不佳。早期诊断、系统正规治疗及长期神经心理学康复非常重要。     

不同抗癫药物治疗对癫患儿血脂的影响

目的探讨癫患儿血脂水平的改变及应用抗癫药物对血脂水平的影响。方法采用酶测定法分别测定2009年1月-2011年1月在小儿癫专科门诊随访治疗的55例癫患儿(全面性发作30例,单纯局限性发作18例,不能分类的发作7例)和30例健康体检儿童(健康对照组)血清总胆固醇(TC)、三酰甘油(TG)、高密度脂蛋白(HDL-C)、低密度脂蛋白(LDL-C)水平,分析癫患儿在单药或联合用药至少0.5 a后与健康对照组比较血脂水平的变化。结果癫患儿服用抗癫药物后引起其血脂水平的改变,与健康对照组血脂水平相比,血清TG、LDL-C显著升高(Pa<0.01),HDL-C水平显著下降(P<0.01);丙戊酸钠(VPA)单药治疗的癫患儿与健康对照组相比,TG、LDL-C升高及HDL-C降低,其差异有统计学意义(Pa<0.05,0.01),而单用VPA与联合用药组之间及两个联合用药组间血脂水平改变的差异均无统计学意义(Pa>0.05);联合用药组与健康对照组比较,其改变幅度VPA>VPA+拉莫三嗪>VPA+左乙拉西坦,但差异均无统计学意义(Pa>0.05)。结论癫患儿服用抗癫药物后存在血脂水平的改变,以单用VPA的改变最大。     

丙戊酸钠对癫癎患儿瘦素及血脂的影响

目的研究丙戊酸钠(VPA)对癫患儿体质量指数(BMI)、瘦素及血脂的影响。方法选择准备单独服用VPA治疗的癫患儿24例,分别于治疗前、治疗后3、6个月观察BMI、瘦素、总胆固醇(TC)、三酰甘油(TG)、低密度脂蛋白胆固醇(LDL-C)、高密度脂蛋白胆固醇(HDL-C)的变化。结果VPA治疗3个月在BMI、瘦素、TC、TG、LDL-C均较治疗前明显升高(P均<0.05),HDL-C变化不明显。治疗6个月,其各项指标的变化基本同治疗3个月时,仅LDL-C又回降至治疗前水平。结论VPA有导致癫患儿肥胖的作用,且多在服用VPA3个月内发生,并对血脂有一定影响。     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.     

Psychopathologie du syndrome d’Asperger et « aspérigisation » de la société contemporaine

The author has attempted here to point out, just for a start, the characteristics of Asperger syndrome from the point of view of psychopathology through a rereading of Hans Asperger's original paper (1944). This thesis merits reevaluation, if for no other reason than to fill the gaps in operational diagnostics based on the DSM. It is found by rereading that Asperger's view of the principal disturbances of autistic psychopathy include a “disturbance of natural evidence” or a “crisis of common sense”. This question of natural evidence that he evokes with regard to autistic psychopathy corresponds to W. Blankenburg's natural evidence, which constitutes a key concept for comprehending schizophrenia in the form poor-symptom (“symptomarme Schizophrenie”) that he observes in the speech of his patient Anne Rau. One can deduce from this that in terms of fundamental disturbances, Asperger syndrome and this “symptom-poor” schizophrenia overlap at the level of loss of natural evidence. It is moreover possible to classify Asperger syndrome among the disturbances of spacing in the sense meant by the evolutionary psychiatry of A. Stevens and J. Price. The author then develops our comprehension of Asperger syndrome from the point of view of the perspective proposed by the notion of resilience in people with Asperger syndrome and of the possibility for them, through these mechanisms of adaptation, to find in the organization of the personality of the “as if” type a position of relative equilibrium. They concur or overlap in the creation of crutches, of borrowed personalities secondarily legitimated by the reaction of the socius. This will end up in the production of inventions and œuvres (works). Clearly, one rarely encounters several cases that one could consider pertinently to be “successful” Asperger syndrome. Finally, the author notes that one can find a sort of isomorphism between Asperger syndrome and contemporary society when he proposes the term “asperigisation” to characterize our society, given that the equilibrium between emotion and logic is strongly disturbed in these patients, in whom logic undergoes hypertrophy while emotion is impoverished. From this perspective, the author hopes to suggest reasons for the increase in the number of cases of Asperger syndrome in the clinical setting and in society in general in our contemporary era.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.