左乙拉西坦添加治疗大田原综合征的疗效

目的了解左乙拉西坦(LEV)添加治疗大田原综合征(OS)的疗效。方法采用开放性自身对照研究,对确诊OS且当抗癫药物治疗无效的患儿给予添加LEV口服治疗。共入组11例。男7例,女4例;年龄29～87 d。治疗剂量从10 mg·kg-1·d-1开始,每1～2周日剂量增加10 mg·kg-1,至维持剂量40～50 mg·kg-1·d-1,分2次口服。治疗前1周和治疗后1个月及每3个月分别评价临床发作及视频脑电图。治疗期间检测肝肾功能及血常规,密切随访不良反应。结果 1例(9.1%)因出现明显的烦躁症状,在服药2周停药,停药2 d后烦躁症状完全缓解;其余10例患儿平均随访时间16.3个月(7～28个月)。1例(9.1%)发作完全控制,7例(63.6%)发作减少>50%,2例(18.1%)无效,总有效率为72.7%。6例患儿(54.5%)精神运动发育滞后有不同程度减轻。坚持服药的10例患儿均未出现明显不良反应,治疗期间未见肝、肾功能及血常规有异常改变。结论 LEV添加治疗OS具有良好疗效及安全性,可以在临床中进一步推广,但尚需多中心随机双盲安慰剂对照试验进一步支持。     

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目的 评价左乙拉西坦(LEV)治疗小儿癫痫的疗效和安全性.方法 2007年8月 2008年8月武汉市儿童医院神经内科病房和门诊收治的56例癫痫患儿口服LEV片.其中32例单药治疗;24例添加治疗,即在原有抗癫痫药物剂量及用法不变的基础上添加LEV口服.起始剂量为5.2～20.8 mg/(kg·d),2次/d;目标剂量为14.4～41.6 mg/(kg·d),2次/d.随访时间为3～12个月.结果 LEV单药治疗有效率为68.8%,无发作率为40.6%.添加治疗有效率为41.7%,无发作率为8.3%.两组比较差异有统计学意义(P<0.05).LEV副反应较少,主要为胃肠不适、头晕、嗜睡、易激惹等.结论 LEV治疗儿童癫痫有效,单药治疗疗效高于添加治疗,患儿对其有较好的耐受性.     

奥卡西平治疗儿童良性癫(癎)伴中央颞区棘波的临床观察

目的观察奥卡西平(OXC)治疗儿童良性癫癎伴中央颞区棘波(BECT)的疗效和安全性。方法用OXC治疗17例BECT患儿,分析单药治疗后1、2、4、6个月的疗效和不良反应。OXC起始剂量为5～10mg/(kg·d),每隔1周增加1次剂量5～10mg/(kg·d),维持剂量20～30mg/(kg·d)。结果本组总有效率为88.24%,服药6个月时累积控制率为70.59%,留存率为94.12%,均呈现较高比率。结论OXC治疗BECT的疗效明显,不良反应轻,安全性高。     

普罗帕酮和美托洛尔治疗病毒性心肌炎致期前收缩的比较

目的 比较普罗帕酮和美托洛尔治疗病毒性心肌炎(VM)所致室上性和室性期前收缩的疗效和不良反应.方法 将108例VM致频发室上性(11例)或室性(97例)期前收缩且需要抗心律失常药物治疗的患儿随机分为二组,分别予普罗帕酮(56例)5～7 mg/(kg·次),每6 h一次,减少或控制后改为每8 h一次,症状消失1个月,改为2次/d再持续用药3个月停药,总疗程5～6个月.和美托洛尔(52例)2～3 ms/(kg·d),分2次服用,连续服用2周,无效停药,有效持续用药3个月,总疗程4～6个月,比较二组的起效时间、疗效和不良反应.结果 普罗帕酮组的显效率71.34%(40/56例)、总有效率91.07%(51/56例),均较美托洛尔组高(分别为40.38%、69.23%)(X2=10.572,8.211 P.<0.005),但二组的有效率比较无显著性差异(X2=1.249 P0.05).普罗帕酮起效时间(8.48±6.02)h较美托洛尔短(96.21±31.24)h(P<0.05).但普罗帕酮组不良反应发生率达25.0%、心血管不良反应发生率达14.29%,较美托洛尔组高(分别为5.77%、1.92%)(Pa<0.05).结论 普罗帕酮与美托洛尔治疗VM所致的期前收缩,疗效均肯定,但前者更有效、起效更快.但均需注意其致心律失常作用,及时调整药物剂量或种类.     

奥卡西平治疗儿童局限性癫(间)464例

目的 观察奥卡西平(OXC)治疗儿童局限性癫(间)的疗效和不良反应,并作出评价.方法 局限性癫(间)患儿64例,41例新诊断或未经正规抗癫(间)治疗需用OXC单药治疗的患儿进入OXC单药治疗组;23例曾使用一种或多种抗癫(间)药物治疗,目前仍需联合应用OXC进行抗癫(间)治疗的患儿进入OXC添加治疗组.OXC治疗的起始剂量为4～8 mg/(kg·d),每隔1周增加1次剂量,每次增加不超过10 mg/(kg·d),维持剂量28～40 mg/(kg·d),分2次服用.进行自身对比开放性观察,分析二组6个月内的疗效及不良反应.结果 本组中单药治疗组、添加治疗组、全组的总有效率分别为85.4%、69.6%、79.7%,其完全控制率分别为53.7%、17.4%、40.6%.单药治疗与添加治疗组6个月后总有效率比较有显著性差异(P＜0.05);单药治疗组2、4、6个月总有效率比较无显著性差异(Pa＞0.05).最常见的不良反应:皮疹7例,嗜睡、头晕各3例,乏力2例,低钠血症、恶心纳差各1例. 因皮疹退出6例(9.4%),停药及对症处理后,均恢复正常.结论 OXC治疗儿童局限性癫(间)疗效确切、耐受性好、不良反应轻.     

左乙拉西坦治疗儿童失神癫(癎)的疗效

目的 探讨左乙拉西坦(LEV)治疗儿童失神癫(癎)(CAE)的疗效.方法 选择2008年1月-2010年12月于本院诊治的CAE患儿65例.男35例,女30例;就诊年龄5～14(7.8±3.5)岁.发病年龄3～12(7.1±3.0)岁.病程为1个月～4.3 a[(12.2±9.8)个月].随机分为LEV组(33例)和丙戊酸钠(VPA)组(32例).分别接受LEV及VPA治疗,LEV开始剂量为10 mg·kg-1·d-1,每周加量1次,逐渐加量至20～30 mg· kg-1·d-1,最大剂量40mg·kg-1·d-1.VPA治疗剂量为15 ～40 mg·kg-1·d-1,血药质量浓度控制在50～100mg· L-1.治疗期均为24周,观察2组发作控制的效果和药物不良反应.结果 LEV组9例(27.2％)完全控制,15例(45.5％)部分控制,总有效率72.7％;VPA组11例(34.4％)完全控制,15例(46.9％)部分控制,总有效率81.3％.2组总有效率比较差异无统计学意义(P＞0.05).2组均未出现不能耐受而中断治疗的病例,LEV组不良反应轻微,3例诉头晕,2例出现困倦、乏力;VPA组不良反应较多,5例患儿体质量明显增加,4例学习成绩明显下降.结论 LEV对大部分CAE疗效满意,且不良反应小.     

霉酚酸酯联合甲基泼尼松龙隔日疗法治疗儿童狼疮性肾炎的疗效

目的观察霉酚酸酯(MMF)联合中小剂量肾上腺皮质激素隔日治疗在儿童狼疮性肾炎(LN)的疗效,以探讨儿童LN的最佳治疗方案。 方法回顾性分析1999—2004年在复旦大学附属儿科医院住院的LN患儿30例,男6例,女24例,处于病情的活动期,给予MMF联合中小剂量肾上腺皮质激素隔日治疗。MMF初始剂量为20~30mg/(kg·d)(总量<1.5g/d),病情好转后每3~6个月减量1次,至0.25g/d维持。甲基泼尼松龙冲击治疗15~30mg/(kg·d)(总量≤1.0g/d),初始剂量1.0~1.5mg/(kg·d)(总量≤60mg/d)。 结果(1)30例患儿MMF治疗时间不少于6个月,为(28.6±15.1)个月,在治疗过程中未见明显副反应;(2)在肾活检的24例中,狼疮性肾炎Ⅱ型者8例在加用MMF治疗的3~6个月获得缓解;狼疮性肾炎Ⅳ型者16例,12例在治疗的3~12(6.0±1.3)个月获得缓解,1例患儿部分缓解,3例患儿经过至少6个月的足量MMF治疗无效。(3)在治疗过程中有7例患儿在减量或停药后出现疾病的复发或部分复发。(4)随访终点获得缓解的27例患儿,治疗前后身高增长无明显抑制。 结论MMF联合中小剂量的泼尼松龙能有效地治疗儿童系统性红斑狼疮,较长时间应用无明显副反应。患儿可保持相对正常的生长发育。     

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。     

小剂量托吡酯治疗Tourette综合征的效果

目的探讨托吡酯(TPM)治疗Tourette综合征的临床效果及合适剂量。方法Tourette综合征患儿79例予TPM口服,剂量从0.5mg/(kg.d)开始,渐增加至3mg/(kg.d),2次/d,于治疗前及治疗后3个月应用耶鲁抽动症整体严重程度量表(YGTSS)评估疗效,观察药物不良反应。结果除1例出现药物不良反应和2例剂量增加至3mg/(kg.d)无效果而退出观察外,余76例于治疗后2～4周症状均获显著改善,治疗前后YGTSS分值:运动性抽动分数19.63±3.09和5.05±1.74,发声性抽动分数18.95±2.56和4.82±1.94,综合损害分数24.21±5.89和10.42±3.69,严重度总分62.21±5.81和22.26±4.81。治疗前后YGTSS各分值比较均有显著差异(Pa<0.001)。76例中3例出现疲乏,1例嗜睡,2例注意力不集中,继续用药后症状消失,均不影响治疗。结论TPM治疗Tourette综合征疗效好,所需剂量小,不良反应轻,可作为治疗该病的首选药物之一。     

极低出生体重儿早期静脉营养的临床研究

目的 研究极低出生体重儿对早期静脉营养的耐受情况和疗效.方法 32例极低出生体重儿随机分为2组:经典静脉营养组(CPN)和实验静脉营养组(EPN).CPN组生后24～48 h内仅给予5%～10%葡萄糖,之后加6%小儿氨基酸和20%脂肪乳,氨基酸从1.0 g/(kg·d)开始每口递增0.5 g/(kg·d),直至3.0 g/(kg·d);脂肪乳选用含中长链脂肪酸,从0.5 g/(kg·d)开始每日递增0.5 g/(kg·d),直至3.0 g/(kg·d).EPN组生后24 h内起即予6%小儿氨基酸2.4 g/(kg·d)和脂肪乳2.4 g/(kg·d),72 h内均增至3.0 g/(kg·d),即达到足量静脉营养.1周内每天计算总热卡包括胃肠内和胃肠外热卡,检测入院72 h内和1周后监测血脂、胆红素、肾功能、血碳酸氢盐、血糖等生化指标.并记录体质量最大丢失,计算1周后可以部分胃肠营养的患儿百分比、恢复出生体质量时间和达到完全胃肠营养时间等指标.结果 与CPN组相比,EPN组的总能量摄入以及胃肠外提供热卡在生后前5 d明显增高,1周内体质量丢失较少,恢复出生体重时间和恢复完全胃肠营养时间缩短,且末增加代谢性酸中毒、脂质代谢紊乱、高胆红素血症以及肾功能损伤等并发症.结论 极低出生体重儿早期可以耐受较大剂量的静脉营养,且有一定的临床效应.     

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.