轻度胃肠炎伴发良性婴幼儿惊厥临床研究

目的　了解轻度胃肠炎伴发的良性婴儿惊厥 (BICE)在华北地区的发病概况。方法　自 1999年 1月至 2 0 0 1年 4月对因急性胃肠炎 (GE)合并无热惊厥的患儿进行临床观察和出院后随访。结果　在观察研究时间内共有 898例GE患儿住院 ,伴无热惊厥者 16例 ,其中男 9例 ,女 7例 ,发病年龄最小 4 5个月 ,最大 2 6个月 ,1～2岁者共 12例。临床GE症状不严重 ,伴无热惊厥呈全身强直阵挛样发作 ,发作间期EEG(12例 )和视频脑电图监测 (4例 )主要改变为 4～ 5Hzθ波活动和正常睡眠EEG背景波 ,无一例有棘 慢波或尖波发放。其它实验室检查包括 :血清电解质、血糖、肝功能检查均正常 ,3例做脑脊液检查、10例做头颅CT检查结果均正常。惊厥发作易控制。出院后随访 14例 (平均间隔 1年 2个月 )未见惊厥复发 ,生长发育如正常儿。结论　轻度GE可以伴有无热惊厥 ,其发生比例约为 1 78% (16/898) ,惊厥形式为全身强直 阵挛样发作 ,易于控制 ,预后良好 ,故可称之为BICE。     

轻度胃肠炎伴婴幼儿良性惊厥26例

目的探讨轻度胃肠炎伴婴幼儿良性惊厥(BICE)的临床特征。方法回顾性分析26例BICE临床资料,观察病情,行血液常规、生化、大便常规及脑影像学(CT/MRI)和视频脑电图等相关检查,按照BICE的诊断标准,除外热性惊厥及癫。予抗惊厥药物对症治疗,随访2 a。结果 26例均有BICE典型的临床特征,高发时间为当年11月份-次年3月份。胃肠炎以轮状病毒多见,主要症状为轻度腹泻或(和)呕吐伴无热惊厥。发作形式为全身强直-阵挛发作,或局限发作泛化为全面性发作。脑CT/MRI检查正常或轻度异常,随访0.5 a有3例复发,复发次数≤3次,经治疗惊厥症状全部消失。23例2 a内无复发,发作前后精神运动发育正常。结论对于年龄6个月～3岁患儿,有轻度腹泻、呕吐伴无热惊厥,无酸碱失衡、电解质紊乱及血糖异常,无脑电图异常及脑脊液改变者可考虑BICE,尽早确诊,不必要长期复杂的检查及抗癫治疗。     

轻度胃肠炎伴婴幼儿良性惊厥的临床分析及随访研究

目的分析轻度胃肠炎伴婴幼儿良性惊厥(BICE)的临床特点及预后。方法对436例BICE患儿的临床资料进行回顾性分析,并对其中206例患儿进行1.5~7年的预后随访,邀请部分家长完成Weiss功能缺陷量表以评价患儿远期的社会功能。结果患儿发病年龄多在13~24月龄;发病时间多在9月至次年2月;惊厥主要表现为全身强直-阵挛发作,多在发病24 h内发作,每次持续时间多在5 min内,可有丛集性发作。206例患儿随访过程中,除1例仍有癎样放电,其余脑电图复查均恢复正常,所有患儿家属认为患儿智力正常,无明显性格改变。基于部分BICE患儿家长填写的Weiss功能缺陷量表显示,BICE患儿的远期社会功能与年龄、性别相匹配的健康对照儿童比较差异无统计学意义。结论 BICE多发生于1~2岁婴幼儿,多表现为短暂的全面性发作,部分可表现为丛集性发作,但极少进展为癫癎,预后良好。     

轻度胃肠炎伴发良性婴幼儿惊厥20例临床分析

目的 分析轻度胃肠炎伴发良性婴幼儿惊厥的特点.方法 2003年7月～2006年5月对我院儿科20例4～30个月的轻度胃肠炎伴发良性婴幼儿惊厥患儿进行临床观察和出院后12个月以上的随访.结果 20例发病年龄最小4个月,最大30个月,1～2岁共14例.冬季发病10例,秋季发病8例.3 d内发生惊厥17例,均为全身发作,持续时间短,不超过5 min,一次病程中惊厥平均发作1.8次.临床急性胃肠炎症状不严重,伴无热惊厥呈全身强直阵挛发作,间期EEG正常,血清电解质、血糖、肝功能检查均正常,10例做脑脊液检查正常,12例做头颅CT检查结果均正常.全部病例无癫(癎)和热性惊厥家族史,出院随访17例有2例惊厥复发,17例患儿生长发育如正常儿.结论 轻度胃肠炎伴发良性婴幼儿惊厥有以下特点:(1)1～2岁高发,冬秋季多发,无家族史;(2)无热惊厥于病程的1～5 d发生,全身性发作为主,无脱水酸中毒;(3)血清电解质、血糖、肝功能、脑脊液检查、发作间期EEG均正常;(4)部分病例可复发,次数≤2次,病程少于1年,预后好.     

轻度胃肠炎伴婴幼儿良性惊厥在婴幼儿急性腹泻伴惊厥疾病谱中的地位及意义

目的探讨轻度胃肠炎伴婴幼儿良性惊厥(BICE)在婴幼儿急性腹泻伴惊厥疾病谱中的地位及意义。方法对2009-2011年收治的急性腹泻并有惊厥症状患儿的住院资料进行回顾性分析。结果 184例急性腹泻伴惊厥的病例中,轻度胃肠炎伴婴幼儿良性惊厥58例、热性惊厥49例、癫癎43例、病毒性脑炎19例、低钠性脑病6例、高钠性脑病5例、中毒性脑病2例、低钙惊厥2例。BICE患儿年龄为(17.47±7.90)个月,全身症状轻,脱水轻或无,惊厥多呈全面性强直或强直阵挛发作,发作持续时间短,多发生在病程前2 d;轮状病毒阳性率为65.52%;血常规、生化、脑脊液、CT/MRI、脑电图等无明显异常。BICE患儿入院后予补液治疗,在首次惊厥后给予肌注苯巴比妥,住院过程惊厥再发者立即静脉注射地西泮,均住院2～5 d治愈出院。结论 BICE为婴幼儿急性腹泻伴惊厥中的常见疾病,临床应以对症治疗为主,应避免不必要的检查和过度药物治疗。     

轻度胃肠炎伴婴幼儿良性惊厥24例

目的：了解伴轻度胃肠炎婴幼儿良性惊厥（BICE）患儿临床经过。方法：对本院2002年12月-2004年2月收治的BICE患儿24例进行2年～3年4个月随访，临床发作有成簇惊厥发作倾向，排除电解质异常、热性惊厥、癫痫及癫痫综合征、症状性癫痫及神经运动发育异常，观察患儿惊厥的复发率及预后。结果：脑电图中央区小尖波4例；CT示脑外部积水1例；经2年随访观察，因胃肠道症状再发惊厥2例，因高热再发惊厥2次1例；随访患儿均未应用抗癫痫药，智能发育正常。结论：BICE患儿惊厥发作次数较少。部分患儿惊厥呈成簇发作，患儿临床预后均良好。     

轻度胃肠炎伴良性婴幼儿惊厥的临床研究(英文)

目的：认识轻度胃肠炎伴良性婴幼儿惊厥(BICE)的临床特点,做到正确诊断和合理治疗。方法：对年龄在3～36个月的轻度胃肠炎伴惊厥的患儿进行临床观察和出院后18个月以上的随访。结果：12例诊断为BICE,发病年龄(16.0±6.5)个月,6例(50%)冬季发病,9例(75%)3d内发生惊厥,为全身性或部分性发作,7例(58%)有早期频繁发作,一次病程中惊厥平均发作2.1次。发作间期EEG、脑影像学、血生化及脑脊液检查正常。惊厥停止后未行抗痫治疗,3例(25%)复发,复发≤2次,病程最长为8个月。所有病例精神运动发育正常。结论：本组BICE具有以下特点:1～2岁高发,冬季多发,无家族史;无热惊厥多出现于病程的早期,全身性或部分性发作,早期频繁发作多见;血电解质、血生化、脑脊液、脑影像学和发作间期脑电图正常;部分病例可复发,病程少于1年,预后好。惊厥停止后不推荐应用抗癫痫药。     

轮状病毒肠炎伴发无热惊厥患儿64例临床分析

目的 分析3岁以下轮状病毒肠炎合并无热惊厥患儿的临床特点及预后.方法 收集2010年9月至2011年3月本院门诊64例3岁以下轮状病毒肠炎合并无热惊厥患儿的临床资料,对其肠炎表现、惊厥特点、辅助检查结果进行分析,并观察其预后,随访3个月以上.结果 64例患儿(男36例,女28例)平均年龄(14.4±3.6)个月,11个月龄发病患儿数最多(21.9％),发病患儿数最多的年龄段为12个月～18个月(45.3％).73.1％患儿惊厥多发生于腹泻病程前3d,87.5％患儿表现为全身强直-阵挛,75％为单次发作.痉挛后神志恢复迅速,无进行性意识障碍.惊厥持续时间多为1～3min(48.4％).便A群轮状病毒抗原均呈阳性.血常规、便常规、血电解质检查多为正常,头颅CT均无异常,4例患儿脑电图可见少量棘慢波,2例于中央区或额部可见少量尖波发放.3例再次出现无热惊厥.随访患儿精神运动发育均正常.结论 惊厥多发生于腹泻病程前3d,全身强直-阵挛为主要表现形式,持续时间多为1～3min.1～1.5岁高发,冬春季多见.血电解质、头颅CT或脑电图等辅助检查多无异常.治疗以止痉、止泻、止吐等对症支持治疗为主,多数病例不复发,且预后良好.     

轻度胃肠炎伴良性婴幼儿惊厥8例临床分析

目的　分析轻度胃肠炎伴良性婴幼儿惊厥 (BICE)的临床特点。方法　1999年 7月至 2003年 3月,对北京中日友好医院儿科 8例年龄 3~36个月的轻度胃肠炎伴惊厥患儿进行临床观察和出院后 18个月以上的随访。结果　8例发病年龄(14 0±5 9)个月,冬季发病 4例, 3d内发生惊厥 6例,全身性发作 7例,有早期频繁发作 5例,一次病程中惊厥平均发作 2 4次。发作间期EEG背景正常,脑影像学、血生化及脑脊液检查正常。惊厥停止后未行抗疒间治疗, 3例复发,复发≤2次,病程少于 1年。全部病例无癫疒间和热性惊厥家族史,精神运动发育正常。结论　本组BICE具有以下特点: (1) 1 ~2岁高发,冬季多发,无家族史; (2)无热惊厥于病程的 1 ~5d发生,全身性发作为主,早期频繁发作多见,无脱水及酸中毒; 930血电解质、脑脊液、脑影像学和发作间期脑电图正常; (4)部分病例可复发,次数≤2次,病程少于 1年,预后好。     

以癫痫发作为主要表现的甲状腺功能亢进症

目的探讨甲状腺功能亢进症（甲亢）伴癫癎样发作的临床特点及发病机制。方法总结本院2例住院甲亢患儿,均为女童。1例3岁7个月患儿出现高代谢症状9个月余,未给予正规治疗出现抽搐发作;另1例12岁患儿已诊断甲亢并口服抗甲亢药物,症状好转后自行停药,1周后出现抽搐发作。2例患儿的发作形式均为全面性强直-阵挛发作。根据患儿临床表现、实验室检查、治疗及转归,同时回顾性分析PUBMED检索及中文医学期刊全文数据库,从1975年至今国内外相关文献报道共18例甲亢伴癫癎样发作患儿（年龄3-18岁;男7例,女11例）。结果甲亢伴发癫癎样发作形式主要为全面性强直-阵挛发作,共18例;2例青春期患者表现为肌阵挛发作。其中有3种临床表现形式：8例（8/20例,占40%）以癫痫样发作为首发症状,分别在首发症状出现1个月-2a才确诊为甲亢;癫痫样发作为继发症状9例（9/20例,占45%）,在确诊为甲亢2个月-4a出现癫痫样发作,多为甲亢控制欠佳或中断药物时发生;癫痫患者并甲亢时癫痫样发作加重3例（3/20例,占15%）,均在青春期合并甲亢时发作次数明显增加。结论甲亢可引起癫痫样发作,其发作与甲状腺激素水平升高关系密切;甲亢与癫痫可能存在某些共同的免疫发病机制。     

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.     

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.     

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.