癫患儿心理行为问题的对照研究

癫患儿因心理行为障碍而影响生活质量 ,其生活质量明显低于正常儿童[1] 。为了探讨小儿癫可能存在的视感知障碍及其他心理行为问题 ,我们对 42例癫患儿的神经心理功能进行了综合评估 ,现报告如下。对象和方法1.对象 :癫组 :我院神经内科经临床和脑电图检查确诊的癫患儿 42例 ,均为特发性癫。男 2 9例 ,女 13例 ;年龄 8～ 12岁 ,平均 10岁 3个月 ;病程平均 2年 5个月 ;皮博迪图画词汇测验 (ＰＰＶＴ)测试智商为 (94± 18)分 ;无脑性瘫痪等合并症。对照组 :为随机抽取的武汉星光小学健康儿童 30例。男 2 0例 ,女 10例 ;年龄 8～ 1…     

事件相关电位P300评价癫癎患儿认知损害的研究

为了解Ｐ3 0 0 在评估癫患儿认知损害的价值 ,我们对1996年 5月～ 1999年 4月在我科就诊的 72例癫 (强直 阵挛性发作 )患儿Ｐ3 0 0 检测结果进行了研究分析。报告如下。对象和方法1 对象 :(1)根据 1983年全国小儿神经专题讨论会制定的我国小儿癫发作分类标准 ,将我院确诊的 72例强直 阵挛发作性癫 (癫组 )患儿进行分析。其中男 39例 ,女 33例 ;年龄 6～ 15岁 ,平均 8 5岁。城市患儿 2 8例 ,农村患儿 44例。服苯巴比妥 (ＰＢ) 38例 ,卡马西平 (ＣＢＺ) 34例。 (2 )对照组 40例 ,为同期门诊健康检查者或某郊区小学生 ,年龄6～ 15岁 …     

癫癎样放电对脑皮层高级功能的影响及治疗对策

脑电图的癫样放电与癫发作有高度相关性。发作间期的癫样放电常提示脑内有癫刺激区 (ｉｒｒｉｔａｔｉｖｅｚｏｎｅ)。但有些人从未出现过癫发作或仅偶然有癫发作 ,脑电图却长时间存在数量不等的癫样放电 ,特别是在睡眠期。仅有脑电图上的癫样放电而不引起临床可见的癫发作称为临床下癫样放电 (ｓｕｂｃｌｉｎｉｃａｌｅｐｉｌｅｐｔｉｆｏｒｍｄｉｓｃｈａｒｇｅｓ,ＳＥＤ)。一般来说 ,仅有脑电图的癫样放电而无癫发作不足以诊断癫 ,也不需要给予药物治疗。但频繁或长期的癫样放电会对脑功能产生什么样的影响 …     

学龄期癫痫患儿认知功能的初步探讨

目的探讨亚临床发作对癫痫患儿认知功能的影响。方法对27例服用单药治疗、近期无临床发作的原发性癫痫患儿及11例正常儿童进行神经心理测验及事件相关电位P3测定。依据测验中EEG结果将癫痫患儿分为亚临床发作组及无亚临床发作组。比较三组间各认知功能指标的差异。结果亚临床发作癫痫组较无亚临床发作组总智商低，且汉字结构辨认P3潜伏期延长；无亚临床发作癫痫组较正常组汉字结构辨认P3波幅低；未发现P3潜伏期与总智商之间具有相关性。结论亚临床发作可影响癫痫患儿的认知功能。汉字结构辨认P3潜伏期是反映认知功能的敏感指标。     

小儿癫癎的诊断

癫是小儿时期常见的神经系统疾患。据统计 ,1 4岁以下小儿癫的年发病率为 46～ 83/ 1 0万 ,而 9岁以下更高 ,达 72～ 86/ 1 0万。小儿正值生长发育期 ,癫发作的临床表现、实验室检查的结果与评价、病因与转归均与成人有很大不同。然而 ,在确立癫的诊断时 ,均须包括以下三个方面的内容 :(1 )其临床发作究竟是癫性的、还是非癫性的 ;(2 )若确系性发作 ,进一步确认是什么类型的发作 ?抑或属于某一特殊的癫综合征 ?(3)尽可能明确或推测其癫发作的病因。一、确认是否癫发作(一 )确认癫的主要依据1 .详细而准确的发作病史 :…     

小儿癫癎持续状态危险因素的病例-对照研究

目的　分析小儿癫持续状态 (SE)的危险因素。方法　对 77例发生SE的住院患儿和1 54例未发生SE的癫患儿进行病例 对照研究 ,采用多元Logistic回归模型分析病因、发作类型等 2 4个因素与SE的关系。结果　单因素分析显示SE的危险因素包括 :有明确病因、既往SE史、癫控制不良、复杂部分性发作、部分继发全身性发作、不典型失神发作、头颅CT和 (或 )MRI弥漫异常、脑电图背景活动异常、局灶性放电和用药依从性差 ;多因素分析显示 :有明确病因 (OR =5 62 )、既往SE史(OR =4 50 )、复杂部分性发作 (OR =2 96)、部分继发全身性发作 (OR =2 40 )、脑电图背景活动异常(OR =2 0 6)、用药依从性差 (OR =1 72 ) 6项OR值的差异有显著性 ,显示其为SE的独立危险因素。结论　有明确病因、既往SE史、复杂部分性或部分继发全身性发作、脑电图背景活动异常、用药依从性差为SE的危险因素 ,可增加小儿SE发生的危险性     

β-内啡肽在小儿热性惊厥及特发性癫癎发病中的作用

许多研究表明 ,β 内啡肽 (β ＥＰ)与癫、惊厥发作有关系[1 ,2 ] ,但临床研究尚不多。 1997年 4月～ 1999年 3月我们测定了 2 8例热性惊厥 (ＦＣ)、2 9例癫与 19例对照组患儿的血浆和脑脊液 (ＣＳＦ)中 β ＥＰ的含量 ,以探讨 β ＥＰ在小儿热性惊厥及特发性癫发病中的作用。对象和方法1.对象 :ＦＣ组 :2 8例 ,男 15例 ,女 13例 ;年龄 7个月～ 5岁。诊断按国内制定的标准[3 ] 。为防止混杂因素对血浆和ＣＳＦ中β ＥＰ水平的影响 ,本组病例仅包括临床诊断为上呼吸道感染合并ＦＣ ,除外先天畸形、智力发育落后或其他慢性疾病的患儿。…     

小儿癫癎发作的分类及最新进展

癫是一种发作性疾病 ,正确了解其发作特点对癫的诊断、治疗及判断预后都有重要的参考价值。随着癫机理的深入研究 ,尤其是脑电图技术的不断进步 ,对各种癫发作也有了更多的了解。早在 1981年国际抗癫联盟 (ＩＬＡＥ)分类及命名委员将癫发作分为三类 ,即部分性发作、全身性发作及不能分类的发作。这个分类不是一个单纯的临床症状学分类 ,常常需要结合病因学及脑电图资料来判断。在部分性发作中又分为单纯部分性发作 (发作时无意识障碍 )及复杂部分性发作(发作时有意识障碍 )。全身发作中包括失神发作、肌阵挛发作、阵挛发作、强…     

癫患儿心理行为问题的对照研究

癫患儿因心理行为障碍而影响生活质量,其生活质量明显低于正常儿童［1］。为了探讨小儿癫可能存在的视感知障碍及其他心理行为问题,我们对 42例癫患儿的神经心理功能进行了综合评估,现报告如下。 　　对象和方法 　　1．对象：癫组：我院神经内科经临床和脑电图检查确诊的癫患儿42例,均为特发性癫。男29例,女13例;年龄8～12岁,平均10岁3个月;病程平均2年5个月;皮博迪图画词汇测验(PPVT)测试智商为（94±18）分;无脑性瘫痪等合并症。对照组：为随机抽取的武汉星光小学健康儿童30例。男20例,女10例;年龄8～12岁,平均10岁1个月;PPVT测试智商为（98±16）分。 　　2．方法：视感知测试采用湖南医科大学修定的本顿视觉保持测验(VRT),所用图卡为难度相等的C、D、E三式共30幅无意义图形,应用A法、C法、D法进行每一式的测试,记录正确分和错误分。个性测试采用湖南医科大学修定的儿童艾森克个性问卷(EPQ),包括精神质(P)、内外向(E)、神经质(N)、掩饰性(L) 4个分量表,要求儿童对88个问题用“是”或“不是”回答,统计各量表得分。行为测试采用上海精神卫生中心修定的Achenbach儿童行为量表(CBCL),由家长根据儿童近半年的行为表现逐项填写,共113项,按“没有”、“偶尔有”、“经常有”三级进行评分。适应行为测试采用湖南医科大学修定的儿童适应行为评定量表(SAB),包括8个分量表,由专业医生向儿童家长逐项询问填写,得出独立功能因子、认知功能因子和社会/自制因子T分及适应能力商数（ADQ）。     

起病于一岁以内的癫综合征和复发性惊厥

1岁以内婴儿是小儿惊厥的高发期 ,目前国际抗癫联盟 (ＩＬＡＥ)的分类法尚不能完全涵盖此年龄组的癫综合征或复发性惊厥 ,现参考ＩＬＡＥ(1989)分类和附录[1 ] ,以及中华医学会儿科学分会神经学组对小儿癫综合征分类的建议[2 ] ,介绍如下。(一 )新生儿期1.良性家族性新生儿惊厥 (ＢＦＮＣ) :该类患儿出生史正常 ,生后神经系发育无异常 ,常于生后 2～ 3ｄ出现全身性强直阵挛样发作 ,有时伴一侧或面肌阵挛、强直姿式或呼吸暂停。无发热 ,血清钠、钾、钙、镁和血糖、胆红素正常 ,脑脊液检查正常 ,发作间期脑电图 (ＥＥＧ)正常 ,头颅ＣＴ…     

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.     

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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     

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.     

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.     

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   

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Understanding infant formula

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 healthcare professionals) will experiment with the infant formula available and then 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.     

Overcoming surfactant inhibition with polymers

Inhibition of the function of pulmonary surfactant in the alveolar space is an important element of the pathophysiology of many lung diseases, including meconium aspiration syndrome, pneumonia and acute respiratory distress syndrome. The known mechanisms by which surfactant dysfunction occurs are (a) competitive inhibition of phospholipid entry into the surface monolayer (e.g. by plasma proteins), and (b) infiltration and destabilization of the surface film by extraneous lipids (e.g. meconium-derived free fatty acids). Recent data suggest that addition of non-ionic polymers such as dextran and polyethylene glycol to surfactant mixtures may significantly improve resistance to inhibition. Polymers have been found to neutralize the effects of several different inhibitors, and can produce near-complete restoration of surfactant function. The anti-inhibitory properties of polymers, and their possible role as an adjunct to surfactant therapy, deserve further exploration.