托吡酯长期治疗癫癎的耐受性与安全性

目的　探讨长期应用托吡酯治疗儿童癫的耐受性与安全性。方法　对托吡酯治疗 2 0周后 79例有效病例进行 1～ 3年随访 ,观察其疗效和不良反应 ,分析影响疗效及不良反应的因素。结果　 6 0例发作控制病例 ,经 1.0～ 3.5年随访 ,90 %能维持原有疗效及剂量。发作频率减少≥ 5 0 %但未获控制患儿 19例 ,4 2 %经增加剂量后控制 ,2 1%与其他抗癫药联用后控制 ,2 1%维持原有效果。托吡酯单药治疗疗效维持率优于添加治疗 ,两者比较有显著差异 (P <0 .0 5 )。局灶性发作与全面性发作疗效维持率无显著差异 (P >0 .0 5 )。副作用主要出现于加量期 ,随疗程延长而减轻 ,减慢加量速度或减少剂量可使不良反应减轻或消失。托吡酯单药治疗儿童不良反应少于添加治疗 ,两者比较有显著差异 (P <0 .0 5 )。结论　托吡酯治疗儿童癫具有良好耐受性及安全性 ,单药治疗疗效维持率优于添加治疗     

托吡酯长期治疗癫痫的耐受性与安全性

目的　探讨长期应用托吡酯治疗儿童癫的耐受性与安全性。方法　对托吡酯治疗 2 0周后 79例有效病例进行 1～ 3年随访 ,观察其疗效和不良反应 ,分析影响疗效及不良反应的因素。结果　 6 0例发作控制病例 ,经 1.0～ 3.5年随访 ,90 %能维持原有疗效及剂量。发作频率减少≥ 5 0 %但未获控制患儿 19例 ,4 2 %经增加剂量后控制 ,2 1%与其他抗癫药联用后控制 ,2 1%维持原有效果。托吡酯单药治疗疗效维持率优于添加治疗 ,两者比较有显著差异 (P <0 .0 5 )。局灶性发作与全面性发作疗效维持率无显著差异 (P >0 .0 5 )。副作用主要出现于加量期 ,随疗程延长而减轻 ,减慢加量速度或减少剂量可使不良反应减轻或消失。托吡酯单药治疗儿童不良反应少于添加治疗 ,两者比较有显著差异 (P <0 .0 5 )。结论　托吡酯治疗儿童癫具有良好耐受性及安全性 ,单药治疗疗效维持率优于添加治疗     

托吡酯添加治疗儿童癫癎临床疗效观察

目的　观察托吡酯 (TPM )添加治疗儿童癫疒间 的疗效。方法　采用不同剂量 (3 2～ 12 0mg·kg-1·d-1)的托吡酯添加治疗 35例儿童部分性和 (或 )全身性癫疒间(基线发作次数的中位值 6次 /月 )的开放性研究。TPM治疗的平均时间为 (44 7± 14 2 )d (范围 2 8～ 5 82d) ,TPM持续治疗 3个月和 6个月的平均剂量为 6 75mg·kg-1·d-1。结果　 >70 %的患儿发作频率减少≥ 5 0 % ,38% (13/34)的患儿无发作持续 3个月以上 ,4 3%(14 /32 )的患儿无发作持续 6个月以上。接受不同剂量TPM的治疗反应大致相同 (P >0 0 5 )。而最常见的不良反应为中枢神经系统的表现。经过 1 5年的治疗 ,2 6 %的患儿因不良反应和发作未得到适当的控制而未继续用药。结论　TPM添加治疗儿童癫疒间具有良好的耐受性和安全性 ,并可作为基础抗癫疒间 药物失败后长期控制癫疒间 发作的广谱抗癫疒间药。     

托吡酯添加治疗难治性癫癎28例

目的　观察托吡酯对小儿难治性癫的疗效及不良反应。方法　选择 2 8例难治性癫患儿 ,添加托吡酯起始量为 0 .5～ 1.0mg/ (kg·d) ,以后每周增加 0 .5～ 1.0mg/ (kg·d) ,加量 8周后为目标控制量 ,观察加药后 16周发作情况。结果　 2 8例中有效 18例 ,无效 6例 ,有效率 6 4 .3% ,退出 4例。结论　托吡酯是治疗难治性癫有效、不良反应少的药物     

托吡酯添加治疗难治性癫痫28例

目的　观察托吡酯对小儿难治性癫的疗效及不良反应。方法　选择 2 8例难治性癫患儿 ,添加托吡酯起始量为 0 .5～ 1.0mg/ (kg·d) ,以后每周增加 0 .5～ 1.0mg/ (kg·d) ,加量 8周后为目标控制量 ,观察加药后 16周发作情况。结果　 2 8例中有效 18例 ,无效 6例 ,有效率 6 4 .3% ,退出 4例。结论　托吡酯是治疗难治性癫有效、不良反应少的药物     

托吡酯对癫患儿酸碱平衡的影响

目的探讨托吡酯对癫患儿酸碱平衡的影响。方法31例癫患儿均给予托吡酯单药治疗。初始剂量为0.5~1.0 mg/(kg.d),每周加量0.5~1.0 mg/(kg.d),4~8周达治疗量,为4.0~8.0 mg/(kg.d)。于治疗前、治疗2个月行血气分析。结果托吡酯治疗前碱剩余为(-0.77±2.16)mmol/L,HCO3-为(24.0±1.22)mmol/L。服用托吡酯2个月后,碱剩余为(-6.76±1.02)mmol/L,与治疗前比较无显著差异(t=15.63 P<0.01);HCO3-为(16.64±2.30)mmol/L,与治疗前比较差异显著(t=13.83 P<0.01)。其中1例出现明显呼吸深快,予口服碳酸氢钠治疗,症状消失。余患儿均未出现代谢性酸中毒临床表现。结论癫患儿在服用托吡酯时可引起酸碱平衡的变化。     

托吡酯治疗小儿癫痫的临床研究

目的：观察托吡酯(TPM)添加或单一治疗小儿癫痫部分性发作及继发性全身性发作的疗效和不良反应。方法：采用开放性试验的方法进行添加及首诊单药的托吡酯治疗。8周加量期及12周稳定观察期,治疗剂量由每日0.5～1 mg/kg开始，每周增加 0.5～1 mg/kg，有效剂量为每日(5.8±2.3) mg/kg，按常规计算发作减少百分比。结果：托吡酯治疗小儿癫痫32例，疗程均在6个月。总疗效：发作减少≥50%者为 26例(81.3%)，≥75%者20例(62.5%)，减少100%者13例(40.6%)。就发作类型而言，癫痫发作频率减少≥50%者中，复杂部分性发作(CPS)为81.8%，单纯性部分性发作(SPS)为87.5%，部分发作继发全身发作(SGTCS)为76.9%，未出现严重的副作用。结论：TPM对癫痫部分性发作伴或不伴有继发性发作者有效，口服安全。     

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.     

托吡酯联合用药治疗婴幼儿重症癫癎

目的 评价托吡酯(TPM)联合用药治疗婴幼儿重症癫癎的疗效及安全性。方法 对12例重症癫癎婴幼儿(年龄4~19个月)采用TPM联合用药治疗。TPM应用是根据发作形式和频率在原来一个或多个抗癫癎药物的基础上进行联合用药治疗。结果 TPM的起始剂量0.5~1.0mg／(kg·d),加量1次／1~2周,剂量为0.5~1.0 mg／(kg·d),目标剂量为4~10 mg／(kg·d)。TPM治疗时间平均为6.2个月,总有效率75％,无效率25％,完全控制为50％。临床观察局灶性发作疗效明显优于全面性强直-阵挛发作。TPM不良反应较轻,仅3例出现睡眠减少、发热及语言减少。结论 TPM联合用药治疗婴儿重症癫癎有效率较高,是一种安全性好、不良反应轻的治疗方法。     

托吡酯联合用药治疗婴幼儿重症癫痫

目的 评价托吡酯(TPM)联合用药治疗婴幼儿重症癫癎的疗效及安全性。方法 对12例重症癫癎婴幼儿(年龄4~19个月)采用TPM联合用药治疗。TPM应用是根据发作形式和频率在原来一个或多个抗癫癎药物的基础上进行联合用药治疗。结果 TPM的起始剂量0.5~1.0mg／(kg·d),加量1次／1~2周,剂量为0.5~1.0 mg／(kg·d),目标剂量为4~10 mg／(kg·d)。TPM治疗时间平均为6.2个月,总有效率75％,无效率25％,完全控制为50％。临床观察局灶性发作疗效明显优于全面性强直-阵挛发作。TPM不良反应较轻,仅3例出现睡眠减少、发热及语言减少。结论 TPM联合用药治疗婴儿重症癫癎有效率较高,是一种安全性好、不良反应轻的治疗方法。     

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.