注意缺陷多动障碍混合型和注意缺陷型的神经心理特征分析

目的探讨注意缺陷多动障碍混合型、注意缺陷型与正常儿童的神经心理特征的差异。方法采用视觉记忆、Stroop效应、倒背数字、词汇流畅性、威斯康辛卡片分类、Temporal discounting等测验、分别测试124例注意缺陷多动障碍儿童（包括85例混合型和39例注意缺陷型）和124例正常儿童的视觉工作记忆、反应抑制能力、语音工作记忆、执行功能和延迟满足能力。结果ADHD儿童完成字义与字色相矛盾的字色命名时间[注意缺陷型：84（20）,混合型：98（31）]较正常组[70（28）]延长,错误数[注意缺陷型：3（3）,混合型：6（19）]较正常儿童[2（5）]增多（P〈0．01）;ADHD儿童倒背数字分数[注意缺陷型：3（3）,混合型：3（4）]较正常儿童[4（4）]低（P〈0．01）;延迟视觉记忆[注意缺陷型：19（5）,混合型19（5）]也较正常儿童[20（5）]低（P〈0．01）;ADHD儿童视觉工作记忆得分[注意缺陷型：21（3）,混合型：20（5）]较正常儿童[20（3）]明显偏低（P〈0．01）;ADHD儿童词汇流畅性错误数[注意缺陷型：1（1）,混合型2（1）]显著高于正常儿童[0（0）]（P〈0．01）;ADHD儿童的威斯康辛卡片分类错误数[注意缺陷型：15（17）,混合型：15（15）]显著高于正常儿童[13（13）],分类数[注意缺陷型：5（4）,混合型：5（4）]低于正常儿童[5（3）]（P〈0．01）;ADHD儿童的延迟满足能力显著低于正常儿童（P〈0．01）。ADHD的两种亚型之间在部分测试的结果差异有统计学意义,混合型的ADHD儿童受损更为明显（P〈0．01）。结论ADHD儿童存在视觉工作记忆、语音工作记忆、延迟满足能力、反应抑制等多项认知功能缺陷,其中混合型的儿童受损更为明显。     

注意缺陷多动障碍儿童临床特点及长期服用哌甲酯的安全性

目的：通过对注意缺陷多动障碍（ADHD）儿童临床分型、治疗前后脑电图（EEG）监测，探讨儿童ADHD临床特点及长期服用哌甲酯的安全性。方法：将2005年1月～2006年6月首都儿科研究所附属儿童医院神经内科确诊的ADHD患儿121例，予哌甲酯10～20mg／d口服，观察6～18个月。于治疗前及治疗后3、6个月予ADHD评定量表-Ⅳ-父母版（ADHDRS-IV-Parent：Inv）、临床总体印象-总体严重度量表（CGI-ADHD-S）行疗效评定，治疗前、治疗后6个月行EEG检查。结果：1．分型特点：ADHD 121例中ADHD-156例，ADHD-C54例，ADHD-HI 11例，分别占46．3％、44．6％、9．1％；2．男女患儿发病比：男104例，女17例；男女比例为6．1：1；3．疗效及EEG：哌甲酯治疗有效者85例（有效率70．2％）；治疗前、治疗后6个月EEG检查均未见异常。结论：女性ADHD患儿少于男性。临床主要以学习困难为主述，以注意力缺陷型为主；哌甲酯长期服用对ADHD患儿EEG无影响。     

儿童多动症发病机制与治疗

儿童注意力缺陷多动障碍（attention defect hyperactivity disorder，ADHD）简称多动症，主要表现为明显的注意定向障碍和注意持续时间短暂，活动过度，行为冲动伴有学习困难和品行障碍。目前国际上把ADHD分为3个主要类型：（1）注意力缺陷型；（2）多动～冲动型；（3）具有两者特征的混合型。儿童发病率较高，我国报道ADHD为1．3％-13．4％，男女比例为4．9：1，它不仅影响儿童在学校和家庭的生活，而且易造成学习困难、行为问题，部分患儿持续终身。为此不断深入研究ADHD发病机制，以便进行有效治疗是非常重要的，临床医师、教育学者在多方面分析论述，现将国内外发展进程进行概括总结如下。     

注意缺陷多动障碍儿童共患抽动障碍临床特征及影响因素分析

目的 分析注意缺陷多动障碍（ADHD）儿童共患抽动障碍（TD）的临床特征及影响因素。方法 观察 312 名 ADHD 儿童共患 TD 情况,分析 ADHD 共患 TD 类型、不同亚型 ADHD 患儿的 TD 共患率,并对 13 个 ADHD 儿童共患 TD 的可能影响因素进行了单因素及多因素 logistic 回归分析。结果 312 名 ADHD儿童共患 TD 42 例（13.5%）,ADHD 混合型（ADHD-C）TD 的共患率为 24.1%,高于 ADHD 多动-冲动型（ADHD-HI,10.9%）和 ADHD 注意缺陷型（ADHD-I,8.8%）患儿（P结论 ADHD 共患 TD 受多种因素影响,应早期针对主要的危险因素进行干预。     

事件相关电位P300在儿童注意缺陷多动障碍诊断中的应用

目的 探讨事件相关电位（ERP）P300在儿童注意缺陷多动障碍（ADHD）诊断中的应用。方法 应用意大利Amplaid MK-15诱发电位系统，采用听觉Oddball刺激序列，对ADHD 30例与正常儿童45例进行P300测定。观察ERP主要波形N1、P2、N2及P3的潜伏期和波幅变化。结果 1．ADHD组靶刺激P300潜伏期明显延迟，与对照组比较，两组有显著性差异（P＜0．01）；ADHD组靶刺激P300波幅降低，但与对照组比较，差别无显著性（P＞0．05）；ERP其他主要波形N1、P2、N2的潜伏期和波幅两组间无显著性差别。2．P300试验在ADHD的临床诊断参考值为靶刺激P300潜伏期＞370ms，可疑诊断值范围为3500－370ms。其特异度为84．5％，灵敏度为66．7％。结论 靶刺激P300潜伏期测定可协助ADHD临床诊断。该方法操作简便，客观性强，患儿易合作，为临床诊断ADHD提供了神经生理学依据。     

注意缺陷多动障碍与多巴胺-β-羟化酶基因Taq I酶切多态性的关系

目的探讨注意缺陷多动障碍（ADHD）与多巴胺-β羟化酶基因（DBH）第5内含子TaqI酶切多态性的关系。方法依据DSM-IV诊断标准，选取76例ADHD核心家系，采用以家系为基础的遗传统计学方法研究DBH的TaqI等位基因A1、A2和基因型（A1／A2，A2／A2，A1／A1）的分布不同，探讨两者之间有无显著性差异。结果以单体型相对风险的方法分析DBH内含子TaqI等位基因A1、A2传递结果x^2=3．09P〉0．05，基因型比较x^2=3．39P〉0．05。提示DBH多态性与ADHD关联不显著，但有增加ADHD患病趋势。根据传递不平衡检验分析DBH内含子TaqI等位基因A1、A2传递结果P=0．136，提示TaqI的两个等位基因在ADHD患儿的传递中差别不显著，可能A2等位基因有增加ADHD患病的趋势。结论DBH内含子TaqIA2等位基因可能增加注意缺陷多动障碍患病的趋势。     

脑电图在儿童注意缺陷多动障碍中的应用

注意缺陷多动障碍（attention deficit hyperactivity disorder，ADHD），又名儿童多动症，近年来发病率较高。临床上以注意力集中困难、多动或行为冲动、智力基本正常等表现为特征。现将我院1998年1月-2005年12月就诊的67例ADHD患儿的脑电图特点分析如下。     

单胺氧化酶基因多态性与注意缺陷多动障碍智商的相关性研究

目的探讨单胺氧化酶基因30bp VNTR(MAOA uVNTR)多态性对中国汉族注意缺陷多动障碍(ADHD)患儿智商的影响。方法对1999年9月至2003年10月在北京大学精神卫生研究所门诊就诊、符合美国《精神障碍诊断与统计手册(4版)》(DSM IV)诊断标准的333例中国汉族ADHD患儿使用中国 韦氏儿童智力量表评定智商,并检测MAOA uVNTR多态性。按照基因型将样本分为低活性基因型组和高活性基因型组,比较两组间智商(包括总智商、言语智商、操作智商)差异,并对影响智力的相关因素进行多元线性回归分析。结果单因素分析结果显示,影响ADHD患儿智商的因素有MAOA uVNTR多态性和父亲文化程度,其中低活性基因型组患儿总智商、言语智商和操作智商得分明显高于高活性基因型组,差异均有统计学意义(P值分别为0.006、0.011和0.042)。本科及以上文化程度父母其子女智商最高,高中(中专大专)次之,初中及以下者最低。父亲文化程度对患儿总智商、言语智商及操作智商等的影响差异均有统计学意义(P值分别为0.015、0.048和0.040);母亲文化程度对患儿总智商影响差异有统计学意义(P=0.33)。经多因素分析,只有MAOA uVNTR基因型进入回归方程,对患儿的总智商产生影响(P=0.021)。结论MAOA uVNTR多态性与ADHD患儿智商相关。     

注意缺陷多动障碍综合征患儿睡眠结构的初探

目的 探讨注意缺陷多动障碍（ADHD）综合征患儿的睡眠结构，睡眠中[XCJIANX5.tif]性放电及睡眠周期性肢体运动（PLMS）的情况；比较ADHD各亚型间睡眠结构的差异。方法 利用多导睡眠监护仪对2005年6月至2006年11月在首都儿科研究所神经科门诊就诊的符合DSM-Ⅳ诊断标准的58例ADHD患儿及30名正常儿童进行整夜睡眠结构监测。结果 ADHD组58例，其中4例睡眠监测未完成，实际完成54例。ADHD组中混合型（ADHD-C）31例（57.4％，31/54），注意缺陷型（ADHD-I）15例（27.8％，15/54），多动/冲动型（ADHD-H）8例（14.8％，8/54）。①与对照组比较，ADHD组快速动眼期（REM）潜伏期短、睡眠潜伏期延长和睡眠效率降低，差异有统计学意义（P<0.05）；②ADHD-C患儿睡眠Ⅱ期百分比较ADHD-I增加，差异有统计学意义（P<0.05）；③ADHD组PLMS发生率为37.0%（20/54），对照组PLMS发生率为13.3%（4/30），差异有统计学意义（P<0.05）；④ADHD组和对照组EEG未见[XCJIANX5.tif]性放电。结论 ①ADHD患儿存在REM睡眠结构的改变、入睡困难及睡眠效率降低；②睡眠Ⅱ期百分比的增多可使ADHD-C较ADHD-I有更多和更重的症状；③ADHD患儿睡眠过程中PLMS发生率较对照组显著升高，PLMS也是导致ADHD患儿睡眠质量下降的原因之一。     

家长培训合并哌醋甲酯治疗对注意缺陷多动症障碍患儿家庭关系的影响

目的 了解家长培训合并哌醋甲酯治疗对注意缺陷多动障碍（ADHD）患儿家庭关系的干预效果。方法 对59 例ADHD 患儿家长进行为期5 周的家长培训,在培训前后使用Conners 儿童行为问卷（父母用）、ADHD 和对立违抗障碍（ODD）症状分级父母评量表、照料者压力问卷、亲子关系自评量表、儿童自我意识量表等进行评估。结果 和基线相比,培训后Conners 儿童行为问卷总分、品行问题及焦虑因子分下降,ADHD症状分级父母评量表各因子分（注意缺陷、多动、冲动）和对立违抗行为均有下降,照料者压力问卷总分下降,亲子关系自评量表总分、儿童自我意识量表总分以及躯体外貌属性、智力和学校行为因子分提高,以上差异均有统计学意义（P结论 ADHD 患儿家长培训能促进亲子间发展良好关系,减轻家长育儿压力。     

The importance of different immunosuppressive regimens in the development of posttransplant diabetes mellitus

Solid-organ transplantation is the optimal long-term treatment for most patients with end-stage organ failure. After solid-organ transplantation, short-term graft survival significantly improved (1). However, due to chronic allograft nephropathy and death with functioning graft, long-term survival has not prolonged remarkably (2). Posttransplant immunosuppressive medications consist of one of the calcineurin inhibitors in combination with mycophenolate mofetil (MMF) or azathioprine (Aza) and steroids. All of them have different adverse effects, among which posttransplant diabetes mellitus (PTDM) is an independent risk factor for cardiovascular (CV) events and infections causing the death of many transplant patients and it may directly contribute to graft failure (3). According to the criteria of the American Diabetes Association (4), diabetes mellitus (DM) is defined by symptoms of diabetes (polyuria and polydipsia and weight loss) plus casual plasma glucose concentration ≥ 11.1 mmol/L or fasting plasma glucose (FPG) ≥ 7.0 mmol/L or 2-h plasma glucose level ≥ 11.1 mmol/L following oral glucose tolerance test (OGTT). This metabolic disorder occurring as a complication of organ transplantation has been recognized for many years. PTDM, which is a combination of decreased insulin secretion and increased insulin resistance, develops in 4.9/15.9% of liver transplant patients, in 4.7/11.5% of kidney recipients, and in 15/17.5% of heart and lung transplants [cyclosporine A (CyA)/tacrolimus (Tac)-based regimen, respectively] (5). Risk factors of PTDM can be divided into non-modifiable and modifiable ones (6), among which the most prominent is the immunosuppressive therapy being responsible for 74% of PTDM development (7). Emphasizing the importance of the PTDM, numerous studies have determined the long-term outcome. On the basis of these studies, graft and patient survival is tendentiously (8) or significantly (9, 10) decreased for those developing PTDM.     

GENETICS OF CARNEY COMPLEX AND RELATED FAMILIAL LENTIGINOSES, AND OTHER MULTIPLE TUMOR SYNDROMES

Carney complex is a multiple endocrine neoplasia (MEN) syndrome that affects the adrenal cortex, the pituitary and thyroid glands, and the gonads. The complex is also associated with skin and mucosa pigmentation abnormalities and myxoid and other neoplasms of mesenchymal and neural crest origin. Thus, this syndrome also belongs to another group of genetic disorders, the lentiginoses (or lentigenoses), which include the Peutz-Jeghers, LEOPARD, arterial dissections and lentiginosis, and Laugier-Hunziker syndromes, Cowden disease and Ruvalcaba-Myhre-Smith (Bannayan-Zonana) syndrome and the centrofacial, benign patterned and segmental lentiginoses, all of which can be associated with a variety of developmental defects. The inheritance of Carney complex, just like that of the other MENs and the lentiginoses, is autosomal dominant. Genetic loci or genes have been identified for Carney complex, Peutz-Jeghers and Ruvalcaba-Myhre-Smith syndromes, but not for other lentiginoses. Elucidation of the molecular defects responsible for these disorders is expected to shed light on aspects of early neural crest differentiation, the regulation of pigmentation, the development of autonomous endocrine function, and endocrine and nonendocrine tumorigenesis.     

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.     

All you ever wanted to know about infant formulas (but were afraid to ask)

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and health care professionals) will experiment with the infant formula available and often attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.     

Differential effects of inhaled budesonide on serum osteocalcin in children and adolescents with asthma

In recent years, measurement of serum osteocalcin has been introduced for assessment of bone turnover in patients treated with exogeneous glucocorticoids. Studies in children with asthma on inhaled glucocorticoids, however, have shown inconsistent results. The aim of the present study is to assess bone turnover in prepubertal children and in adolescents with asthma treated with inhaled budesonide using three different osteocalcin assays: the Pharmacia Osteocalcin CAP FEIA, the CIS OSTK-PR and CIS IRMA ELSA-OSTEO assays. Two studies were conducted: 1) a randomised double blind two-period crossover study of 22 prepubertal children aged 5-12 years. In one period 800 μg budesonide was given once in the morning, in the other 400 μg was given twice daily; 2) a randomised double blind placebo controlled two period crossover study of inhaled budesonide 400 μg twice daily in fourteen 13-16 year old adolescents with pubertal stages II-V. In both studies, treatment periods were of four weeks duration, and blood samples were collected at the last day of each period. In the prepubertal children none of the osteocalcin assays detected any statistically significant differences between any of the periods. In the adolescent group reduced levels of osteocalcin were seen during budesonide treatment. The suppression reached statistical significance with the CAP FEIA (P = 0.03) and the OSTK-PR (P =0.01) assays, but not with the ELSA-OSTEO assay (P = 0.06). Correlation analyses showed statistically significant correlation coefficients varying between 0.58 and 0.91 (P=0.03 and P < 0.0001, respectively). The effect of inhaled glucocorticoids on serum osteocalcin may depend on the assay applied, and inhaled glucocorticoids have differential effects in children and adolescents.     

alpha -SMOOTH MUSCLE ACTIN DISTRIBUTION IN THE PULMONARY VASCULATURE COMPARING HYPOPLASTIC AND NORMAL FETAL LUNGS

We investigated the intra-acinar pulmonary vascular muscularization in the developing human fetal lung between the 17th and 24th gestational weeks, that is, during the canalicular phase of lung development. Fifteen hypoplastic and 25 normal developed lungs were included in this study using monoclonal alpha -smooth muscle (sm) actin antibodies for smooth muscle detection. Computer-aided image analysis was performed for morphometrical measurements and statistical evaluation. Alphasm-actin-immunoreactive intra-acinar vessels down to a luminal diameter of less than 10 mu m were detected in hypoplastic as well as in normally developed lungs. Crucial differences presented as follows: significantly higher density of intra-acinar vessels, especially due to alpha -sm-actin-negative vessels less than 30 mu m in luminal diameter, in the control group; significantly higher alpha -sm-actin immunoreactivity per section unit as well as per vessel in the hypoplastic lung group. As suggested by others, alpha-sm-actin-positive cells of the intra-acinar vessel wall in the developing human lung were demonstrated to be smooth muscle cells, their immediate precursors, and pericytes. We conclude that the increased alpha -sm-actin immunoreactivity represents muscularization of the vessel wall in functional terms and may be regarded as one structural cause among others for the establishment of persistent fetal circulation in hypoplastic lungs.     

Représentations émotionnelles de la maladie chez 22 enfants drépanocytaires

Background

The emotional and psychological impact of chronical disease among children is considerable. The aim of this study was to explore the emotional representations of sickle cell children followed up at Bordeaux University Hospital in both qualitative and quantitative ways.

Methods

Prospective observational study, conducted from February to May 2010 among 22 sickle cell children (SS, SC and Sβ) followed at Bordeaux University Hospital and among their parents. A questionnaire evaluating depressive symptoms and emotional representations was proposed to children and to their parents separately, measuring their child's emotions. Children were asked to draw themselves during sickle cell crisis and without any painful episode, in order to illustrate their perception of their disease.

Results

Emotional and psychological impact on sickle cell children was important in this study. Eighty-six percent of children have commonly had negative feelings such as sadness, anger or fear. Thirty-six percent of them had depressive symptoms. Parents largely underestimated this impact. Drawings and answers to the questionnaire emphasized an important lack of disease understanding, social consequences, and depressive affects.

Conclusion

Psychological and emotional difficulties in sickle cell children should be identified and supported. Resources for psychological and educational support are necessary to improve the quality of life of sickle cell patients in France.     

Limiting light-induced lipid peroxidation and vitamin loss in infant parenteral nutrition by adding multivitamin preparations to Intralipid

Parenteral lipids are susceptible to light-induced peroxidation, particularly under phototherapy. Ascorbic acid is protective. The aim of this study was to investigate whether dark delivery tubing and/or coadministration of multivitamin preparations could prevent peroxidation of Intralipid without undue vitamin loss. In experiments carried out on the benchtop, lipid peroxidation occurred in ambient light and was more extensive under phototherapy. Dark tubing decreased peroxide formation, but only by about 65%. In simulated clinical conditions in which solutions were pumped through standard clear or dark minibore plastic tubing, Intralipid accumulated lipid peroxides as measured by the FOX assay (280 µM) or as triglyceride hydroperoxides (52 µM). Multivitamin preparations (MVIP or completely, and were fully protective when used with dark tubing. There was loss of riboflavin (65% from Soluvit and 35% from MVIP) in clear tubing but this was decreased to 18% and 11%, respectively, in dark tubing. Ascorbate loss was 20% (MVIP) and 50% (Soluvit) and only slightly less in dark tubing. Ascorbate loss was also seen in the absence of Intralipid and is due to riboflavin-induced photo-oxidation.Conclusion: Multivitamin preparations protect Intralipid against light-induced formation of lipid hydroperoxides, and administering multivitamins with Intralipid via dark delivery tubing provides a practical way of preventing peroxidation of the lipid while limiting vitamin loss. This procedure should be considered for routine use as well as with phototherapy. Soluvit/Vitlipid) inhibited peroxide formation almost     

SociaBillyQuizz,un jeu pour l’entraînement aux habiletés sociales chez l’enfant et l’adolescent : étude exploratoire

Background

The SociaBillyQuizz is a therapeutic game designed for social skills training groups with children and adolescents. Using an entertaining method, this media requests several dimensions: exposure, cognition, communication skills, imagination, emotional expression and sign decoding. In this preliminary study, the setting includes two groups of six adolescents, one with social anxiety disorder and the other with Asperger syndrome.

Objective

To evaluate, in an exploratory study, the effects of a therapeutic device involving this game for these two clinically different groups of adolescents.

Methods

During 26 of 1 hour weekly sessions, two adolescents groups participate to a program including the SociaBillyQuizz and cognitive behavioral therapies. The groups are moderated by two therapists. The SociaBillyQuizz is a board game for two to six players; its goal is to collect points by answering instructions from the different thematic cards. There are four thematic cards: action cards (players have to do something), brainstorming cards (players have to use their imagination and demonstrate cognitive flexibility), interview cards (players have to express themselves about what they think or feel) and mystery cards (unexpected instructions). According to the groups’ clinical characteristics, some aims are specifically highlighted. In the anxiety disorder group, the cognitive dimension is privileged and in the Asperger syndrome group, we emphasise the pretend, cognitive flexibility and theory of mind. The effects are measured by the Rathus Assertiveness Schedule and the Fear Avoidance Hierarchy (FAH) for the social anxiety disorder group and by the Faux Pas Recognition Test and the Social Responsiveness Scale (parent version) for the Asperger group.

Results

These assessment tools indicate, for both groups, a significant increase of the scores corroborating the observed clinical effects. For eleven of the twelve adolescents, a clinical interview 6 months after the retest shows a continuity of therapeutic benefit.

Discussion

These early results suggest that a social skills training device featuring the SociaBillyQuizz produces clinical improvements in these two groups of adolescents. In future researches, with control group and more complete follow-up, nature and effectiveness of its contribution should be specified.

Conclusion

In this preliminary study, the SociaBillyQuizz appears to be an interesting therapeutic tool that can increase implication, motivation, participation and cohesiveness of the group. It also makes easier the cognitive-behavioural-strategies learning.     

Les effets de l’incarcération chez les mineurs

Aim of this study

In this article we wish to question the effects of incarceration on minors. The history of prison reveals that it is the work of a humanist and philanthropic discourse that ensued from the effects of revolutionary ideas. However, from the moment of its reform – that transformed it from a place of confinement to a penal institution – it has only demonstrated its dysfunctional aspect. Our objective is to initiate a reflection on the effects of incarceration, whether it be collective or individual and in particular when it involves adolescents.