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目的随访经导管室间隔缺损(VSD)封堵术后并发的传导阻滞,探讨其发生机制及预后。方法2002-06—2005-07山东省立医院小儿心脏科经导管介入治疗89例VSD患儿,对临床资料、心电图、心脏超声、心血管造影资料及随访结果进行分析。结果89例患儿应用Amp latzer膜部VSD封堵器67例,国产对称型VSD封堵器20例,Amp latzer导管封堵器1例,pfm弹簧圈1例。术后出现间歇性完全性房室传导阻滞2例次,其中1例应用临时起搏器治疗,分别于术后第10天、第20天恢复窦性心律;完全性左束支阻滞3例次,完全性右束支阻滞3例次,左前半分支阻滞2例次,不完全性右束支阻滞5例次,加速性交界性心律合并完全性左束支阻滞1例次。结论传导阻滞是VSD介入治疗术后的常见并发症,经治疗预后良好,及时发现、及时处理是诊疗的关键,对心内传导系统远期的影响仍需进一步评价。     

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目的探讨经导管介入治疗小儿膜周部室间隔缺损(VSD)发生心律失常的特点及其处理。方法南京医科大学附属南京市儿童医院心血管科2002-09—2006-03经导管介入治疗VSD 108例,其中发生心律失常的患儿共计22例,动态观察22例患儿封堵术前心电图(ECG)以及封堵后发生心律失常ECG的变化及其处理。结果22例中2例在术中反复出现Ⅲ度房室传导阻滞(AVB),被迫中断;8例患儿术后3d内出现加速性交界性或室性自主心律伴干扰性房室分离,经治疗后均转为窦性心律,其中2例患儿1个月后才恢复窦性心律;6例患儿术后3d内出现束支传导阻滞,经治疗后3例恢复正常,3例仍存在不同程度的束支传导阻滞;5例患儿术后3d内发生高度AVB,其中3例经使用激素、维生素C、果糖及静滴异丙肾上腺素和临时起搏器治疗后,均在1周内恢复窦性心律,另2例患儿经治疗后,1例于10d后转为窦性心律,1例于术后2年时仍有完全性右束支阻滞 左前分支阻滞;1例患儿术后1个月随访发现完全性右束支阻滞。结论术后早期出现的心律失常多能恢复,而后期出现的传导阻滞多为器质性损伤。对已经出现传导阻滞的患儿关键是早期治疗,适当延长术后观察时间。     

小儿膜周部室间隔缺损介入治疗并发心律失常22例分析

目的探讨经导管介入治疗小儿膜周部室间隔缺损(VSD)发生心律失常的特点及其处理。 方法南京医科大学附属南京市儿童医院心血管科2002 09—2006 03经导管介入治疗VSD 108例,其中发生心律失常的患儿共计22例,动态观察22例患儿封堵术前心电图(ECG)以及封堵后发生心律失常ECG的变化及其处理。 结果22例中2例在术中反复出现Ⅲ度房室传导阻滞(AVB),被迫中断;8例患儿术后3d内出现加速性交界性或室性自主心律伴干扰性房室分离,经治疗后均转为窦性心律,其中2例患儿1个月后才恢复窦性心律;6例患儿术后3d内出现束支传导阻滞,经治疗后3例恢复正常,3例仍存在不同程度的束支传导阻滞;5例患儿术后3d内发生高度AVB,其中3例经使用激素、维生素C、果糖及静滴异丙肾上腺素和临时起搏器治疗后,均在1周内恢复窦性心律,另2例患儿经治疗后,1例于10d后转为窦性心律,1例于术后2年时仍有完全性右束支阻滞+左前分支阻滞;1例患儿术后1个月随访发现完全性右束支阻滞。 结论术后早期出现的心律失常多能恢复,而后期出现的传导阻滞多为器质性损伤。对已经出现传导阻滞的患儿关键是早期治疗,适当延长术后观察时间。     

膜部室间隔缺损介入封堵术心律失常356例

目的探讨室间隔缺损（VSD）介入治疗心律失常的发生及其防治措施。方法对356例先天性心脏病VSD患者在介入治疗术中,术后7d内,3、6和12个月时的心律失常进行记录和分析。结果术中新出现的心律失常有室性心律失常356例,阵发性室上性心动过速12例,右束支传导阻滞15例,左束支传导阻滞4例,窦性停搏2例,窦性心动过缓10例,房室传导阻滞14例,其中Ⅱ度房室传导阻滞2例,Ⅲ度房室传导阻滞4例。术后7d内发生心律失常：房性期前收缩1例,室性期前收缩3例,阵发性室上速2例,完全性右束支传导阻滞6例,完全性左束支传导阻滞5例,Ⅲ度房室传导阻滞4例,2例经股静脉插入人工心脏临时起搏器。术后3～12个月共发现2例新发的完全性右束支传导阻滞。结论心律失常是VSD封堵术的常见并发症;术中操作规范、及时药物治疗可有效减少和避免心律失常发生。     

经导管封堵膜周部室间隔缺损术后传导阻滞11例

目的 分析经导管膜周部室间隔缺损(PMVSD)封堵术后传导阻滞发生及转归情况.方法 2004年4月-2006年12月介入治疗PMVSD患儿64例.男38例,女26例;年龄1.3～12.0岁.采用AGA公司偏心型Amplatzer VSD和深圳先健公司对称型VSD封堵器,输送鞘直径为6～9 F.所有患者在X线透视及超声监测下通过建立股动静脉轨道,经右心系统释放封堵器,术后心电监护、检查心电图观察传导阻滞情况.采用SPSS 12.0软件进行统计学分析.结果 PMVSD患儿64例接受治疗,61例封堵成功,封堵术后传导阻滞11例.其中2例完全性房室传导阻滞,1例双束支传导阻滞,8例单纯性右束支传导阻滞(RBBB).其中6例发生于术后72 h,5例发生手术后第4～7天.均予药物治疗.治疗后2例完全恢复正常,9例出院时仍有RBBB.结论 PMVSD封堵术术后传导阻滞主要发生于介入治疗术后早期,以轻型传导阻滞为主,严重传导阻滞经治疗可恢复至窦性心律.该并发症较传统开胸修补术后发生率少见.     

膜周部室间隔缺损经导管封堵术后并发迟发性完全性房室传导阻滞2例报告并文献复习

目的 提高对膜周部室间隔缺损(PmVSD)经导管封堵术后,并发迟发性完全性房室传导阻滞(CAVB)的认识.方法 回顾2例PmVSD经导管封堵术后合并迟发性CAVB的术中和术后资料,结合文献进行分析.结果 例1患儿术中、术后无明确的危险因素,术后6个月出现持续性CAVB伴不完全性右束支传导阻滞,随访5年至今无症状.例2患儿术中出现一过性完全性左束支传导阻滞(CLBBB),术后5 d出现CAVB伴CLBBB,经激素等成功逆转后多次随访,ECG示Ⅰ度房室传导阻滞伴完全性右束支传导阻滞或CLBBB;术后9个月出现一过性晕厥,ECG证实间歇性CAVB伴CLBBB;目前已随访15个月,偶有晕厥发作.2例患儿家长均拒绝植入永久起搏器.结论 PmVSD经导管封堵术后并发迟发性CAVB难以预测,不论有无症状都必须长期随访.充分认识迟发性CAVB的自然史、发生率、发生机制、危险因素和防治对于尽可能减少此致命性并发症的发生是必要的.     

流入道型室间隔缺损介入治疗的适应证及并发症防治

目的 探讨流入道型室间隔缺损(IVSD)介入治疗适应证及并发症防治措施.方法 采用国产室间隔缺损(VSD)封堵器对46例IVSD患儿进行封堵治疗.经胸超声心动图(TTE)显示VSD直径(6.8±5.3) mm.所有患儿在透视及超声监测下通过建立股动静脉轨道,经右心系统释放封堵器,并分别于术后1、3、6、12、24个月通过TTE、心电图检查等方法,从患儿残余分流、瓣膜功能及心律方面进行随访评价.结果 封堵器置入成功40例,未成功6例,成功率82.6 %;4例封堵器释放后存在少量残余分流,术后1个月超声复查残余分流消失.随访中未发现新出现的三尖瓣返流病例;2例原为少量三尖瓣返流者,封堵术后出现中量返流;1例术前伴中度三尖瓣返流,术后出现了重度返流.4例于术后3～6 d出现Ⅲ度房室传导阻滞,常规应用激素等药物治疗,2例痊愈,2例置入临时起搏器8～10 d完全恢复;术后出现6例完全性左束支传导阻滞,5例完全性右束支传导阻滞,2例室性期前收缩,随访3个月均完全恢复.结论 部分IVSD可选择介入治疗,近中期效果良好.但应严格把握适应证,加强其并发症防治.     

膜周部室间隔缺损介入治疗并发症的分析

目的　探讨膜周部室间隔缺损 (ventricularseptaldefect ,VSD)经导管介入治疗的并发症及其预防。方法　 2 0 0 2年 7月 - 2 0 0 4年 5月 ,2 6 2 (男 138,女 12 4 )例膜周部VSD患儿 ,年龄 2～ 18(9 3± 5 8)岁 ,体重 11.0～ 6 5 .0 (平均 30 5 )kg。 3例合并动脉导管未闭 ,4例合并房间隔缺损 ,1例合并动脉导管未闭和房间隔缺损。结果　 2 6 2例患儿术前经胸超声检测VSD大小为 2 7～ 13.0mm(平均 6 5mm) ,术中心室造影测量VSD大小为 1 3～ 14 .0mm(平均 6 8mm) ,2 5 6 /2 6 2例 (97 7% )封堵成功。所选封堵器大小为 4～ 16mm (平均 8 6mm)。严重并发症 8例 (3 1% )。其中高度房室传导阻滞(atrioventricularconductionblock ,AVB) 5例 (2 0 % ) ,溶血 2例 (0 8% )。封堵器明显移位 1例 (0 4 % )。5例AVB患儿治疗后除 1例术后 5 0d安装永久起搏器外 ,其余 4例均恢复正常窦性心律。溶血患儿经常规治疗后恢复正常。封堵器明显移位则采用心脏外科手术处理。其他并发症有 :1例 (0 4 % )术后 6个月复查有微量残余分流 ;5例 (2 0 % )术后新出现主动脉瓣微量返流 ;4例 (1 6 % )术后新出现三尖瓣少量返流 ;75例 (2 9% )术后出现间歇性加速性交界性心律或加速性室性自主心律伴干扰性房室脱节 ,治疗后均恢复     

儿童膜周部室间隔缺损介入治疗的临床评价

目的　评价经导管室间隔缺损封堵术介入治疗膜周部室间隔缺损 (VSD)的安全性及临床疗效。方法　 2 0 0 2年 11月～ 2 0 0 4年 7月 ,共 5 0例膜周部室间隔缺损的患儿接受了Amplatzer偏心状封堵器经导管介入治疗。男 2 6例 ,女 2 4例 ;平均年龄 (9 1± 4 8)岁 (2～ 17岁 )。其中 1例合并主动脉窦窦瘤 ,2例为外科修补术后残余漏 ,1例合并镜面右位心。经胸超声心动图 (transthoracicechocardiography ,TTE)提示 ,VSD的平均直径 (4 8± 0 9)mm (3～ 7mm )。封堵前右心导管提示 ,肺循环血流量 /体循环血流量 (Qp/Qs)比值平均为 1 32 (1 1～ 2 0 )。 4例中等量左向右分流 ,其余均为少量左向右分流。所有患儿在X线透视及超声监测下通过建立股动静脉轨道 ,经右心系统释放封堵器。并分别于术后 1、3、6、12个月进行超声心动图、心电图、X线胸片随访评价。结果　 4 7例患儿封堵器置入成功 ,技术成功率为 94 % ,无死亡病例。术后出现少量残余分流 2例 (<3mm) ,完全性左束支传导阻滞 1例 ,无其他严重并发症发生。所有患儿接受了平均 7个月的随访 (1～ 18个月 ) ,随访中无封堵器移位、脱落以及瓣膜损伤等并发症发生 ,1例残余分流于 6个月随访时消失。超声测量的左室舒张末径 (leftventricleend diastolicdimension     

导管介入治疗小儿先天性心脏病九例

目的应用导管介入治疗小儿先天性心脏病并对其疗效进行初步评估。方法总结我院11例行介入治疗的先天性心脏病患儿。其中室间隔缺损(VSD)和房间隔缺损(ASD)各4例、动脉导管未闭(PDA)3例。结果成功率为88%,术后随访0.5~1年,无不良并发症。结论应用导管介入治疗小儿先天性心脏病是有效的方法。     

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.     

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   

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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.     

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.     

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.     

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.