室间隔缺损介入封堵术后完全性左束支传导阻滞临床分析北大核心CSCD

目的：观察并分析室间隔缺损（VSD）封堵术后出现完全性左束支传导阻滞（CLBBB）患儿的临床特点及预后,以指导临床实践。方法回顾性分析2011年1月至2013年12月于广东省人民医院心血管儿科行经皮 VSD 封堵术后出现 CLBBB 的11例患儿的临床资料,根据 CLBBB 出现的时间和临床症状,采用不同的治疗方案,并随访观察其预后。结果11例患儿手术时中位年龄3.9岁（3.4～17.5岁）,术后出现 CLBBB的中位时间为2.8个月（1 d ～25.4个月）,其中4例为术后1周内出现,1例为术后1周～1个月,6例为术后1个月以上,最迟出现为术后25.4个月。11例患儿中,5例经药物治疗早期心电图恢复正常;3例经药物治疗无效,其中2例行外科手术取出封堵器并修补 VSD,术后1例恢复正常心律,1例转为不完全性右束支传导阻滞,另外1例未行手术取出封堵器,仍为 CLBBB;3例患儿因 CLBBB 出现时间晚（≥6个月）且无临床症状,未予特殊处理。至随访结束,4例患儿心电正常;4例持续存在 CLBBB;1例转为右束支传导阻滞;2例转为正常心电后再次复发 CLBBB,其中1例复发病例出现左心室增大,最后因心功能衰竭死亡。结论 VSD 封堵术后早期及中远期均有可能发生 CLBBB,早期出现的 CLBBB 经及时治疗,可恢复正常心电;持续存在的 CLBBB 可致心肌收缩不同步引起心功能不全。对 VSD 封堵术后出现的 CLBBB 应及时予适当治疗,并长期密切随访。     

膜部室间隔缺损封堵术后完全性左束支传导阻滞治疗效果分析

目的探讨儿童膜部室间隔缺损(VSD)封堵术后完全性左束支传导阻滞(CLBBB)的治疗及预后。方法回顾分析2009年2月至2019年6月就诊的18例VSD封堵术后CLBBB患儿的临床资料。结果 18例接受VSD封堵术并经心电图检测确诊CLBBB患儿的平均年龄为5.69±2.33岁(3岁2个月～10岁5个月),男性11例、女性7例,随访时间中位数5年(3个月～10年)。14例无心力衰竭患儿中4例经糖皮质激素治疗CLBBB即恢复,随访3～6个月各项指标均无异常;8例随访5～10年,无不适,心电图无变化,心功能、左室射血分数、心房利钠肽均无异常,但左室舒张末期内径(LVDD)增大;2例接受封堵器取出及VSD修补术,1例术后出现完全性右束支传导阻滞,余无异常,另1例术后出现完全性房室传导阻滞,植入起搏器,随访1年QRS波时限较术前缩短,心功能无异常,LVDD增大。4例合并心力衰竭患儿中3例行心脏再同步化治疗(CRT)植入术,1例行左室起搏,术后QRS波均150 ms;1例行CRT术后3天因急性心力衰竭死亡,另3例随访1～2年心功能明显改善。结论对于VSD封堵术后早期出现CLBBB患儿,糖皮质激素可能有效,若无效,可选择手术取出封堵器,但有发生完全性房室传导阻滞的风险。若合并心力衰竭,CRT或左室起搏治疗可能有一定效果。     

室间隔缺损并主动脉瓣脱垂患儿的介入治疗及疗效评价

目的探讨室间隔缺损(VSD)并主动脉瓣脱垂(AVP)介入治疗的安全性和可行性。方法选择2007年5月-2009年4月在本院住院的VSD并轻度AVP患儿43例,均行经皮VSD堵闭术,经胸超声心动图显示VSD位置及内径,左心室造影均显示VSD并AVP,造影显示VSD大小为2.6～8.3 mm。其中19例为嵴内型(又称膜周流出道型),15例为隔瓣后型,9例为膜周部。右冠瓣脱垂27例,无冠瓣脱垂12例,右冠瓣并无冠瓣脱垂4例,并局限主动脉瓣返流8例。术后进行超声心动图、心电图的随访观察。结果36例成功封堵VSD,封堵成功率83.7%;7例试封堵后再次行左心室及主动脉造影,显示主动脉瓣下有明显切迹,瓣膜返流加重而放弃堵闭术,择期进行外科手术。采用偏心封堵器15例(5～12 mm),大边小腰封堵器(4～14 mm)16例,普通对称型封堵器(5～10 mm)5例。术后即刻造影有微量残余分流4例,原有的主动脉瓣返流无明显加重;随诊3～18个月,超声心动图示4例残余分流,分别在术后3～6个月消失,主动脉瓣局限返流无进一步增加,其中6例返流减少,未出现三尖瓣狭窄、主动脉瓣狭窄及主动脉瓣穿孔,心电图示无Ⅲ度房室传导阻滞等严重心律失...     

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     

小儿膜周部室间隔缺损介入治疗的临床疗效

目的观察小儿膜周部VSD介入治疗的疗效、对心律的影响及封堵前后左心血流动力学指标变化。方法收集2006年8月-2009年12月本科44例接受介入治疗的膜周部VSD患儿。男17例,女27例;年龄2.4～13.0(5.20±2.68)岁。其中37例选用对称型封堵器,7例选用偏心型封堵器,经造影和经胸超声心动图证实封堵器位置良好,无成束残余分流,主动脉瓣和三尖瓣功能不受影响,即为封堵成功。术前,术后3 d、1个月、3个月、6个月、12个月检查其ECG及经胸超声心动图检测左心室舒张末期内径(LVDD)、左心室收缩末期内径(LVDS)、射血分数(EF)和左心室缩短分数(FS)。结果 43例封堵成功,成功率为97.7%。其中2例有不成束残余分流,分别于术后3 d和术后1个月时消失。术后新出现不完全右束支传导阻滞4例,1例12个月时转为完全性右束支传导阻滞,1例随访中不完全右束支传导阻滞消失,无高度房室传导阻滞发生。与术前相比,LVDD和LVDS在术后3 d和术后1个月时明显缩小(P<0.01,0.05),术后12个月时虽短于术前,但差异无统计学意义,EF和FS术前术后无显著变化。结论小儿膜周部VSD介入治疗是一种安全有效的方法。     

国产双盘状封堵器治疗儿童膜周部室间隔缺损效果及随访研究

目的 探讨国产双盘状封堵器治疗儿童膜周部室间隔缺损（VSD）的效果及其随访。方法 75例膜周部VSD患儿，年龄3~14岁,平均（8.5±3.6）岁，VSD直径为3.0~14.0 mm，平均（6.5±3.6）mm，采用经导管植入国产双盘状封堵器，门诊随访，进行心脏听诊、超声心动图检查有无残余分流及封堵器位置。结果 ①74例封堵器植入成功，技术成功率98.7%，1例导管未能通过室间隔缺孔；②术后即刻左心室造影示：65例（87.8%，65/74）无残余分流，9例（12.2%，9/74）存在微量至少量残余分流，超声心动图示：68例（91.9%，68/74）无残余分流、6例（8.1%，6/74）存在微量至少量残余分流；2例封堵术后3 d发生Ⅲ度房室传导阻滞，应用泼尼松及营养心肌药物治疗4~10 d后消失。1例封堵术后24 h发生溶血，经过7 d内科保守治疗治愈；③术后1个月共随访73例患儿，超声心动图示：71例（97.3%，71/73）无残余分流、2例（2.7%，2/73）存在微量至少量残余分流；④术后3个月、6个月、1年、2年和3年，随访到的病例分别为70、68、21、15和12例，存在微量至少量残余分流的2例分别于3和6个月内残余分流消失。结论 经导管植入国产双盘状封堵器治疗儿童膜周部VSD是一种安全有效的微创介入治疗方法，操作简便，成功率高，近期和远期疗效可靠。     

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.     

室间隔缺损经导管关闭术后传导阻滞的相关因素分析

目的探讨采用膜部室间隔缺损（室缺）双盘封堵器关闭膜周型室缺术后发生传导阻滞的相关因素。方法采用临床回顾性对照研究的方法,分析患儿年龄、性别、缺损位置、缺损直径、封堵器大小、封堵器直径与缺损直径的差值及封堵器形状与术后房室传导阻滞的相关性。结果220例患儿室缺封堵术后,发生不同类型的传导阻滞55例（发生率25％）。其中以束支传导阻滞最多见,Ⅱ度Ⅱ型以上的传导阻滞发生率较低。其构成比分别为完全性右束支阻滞（CRBBB）27．3％,完全性左束支阻滞（CLBBB）5．5％,左前半支阻滞（LABBB）23．6％,右束支阻滞合并左前半支阻滞（RBBB＋LABBB）30．9％,Ⅱ度Ⅱ型1．8％,高度3．6％,Ⅲ度7．3％。结论经双盘封堵器关闭膜部室间隔缺损术后传导阻滞的发生与患儿年龄、缺损位置、缺损直径相关。膜周型室缺与房室传导束密切的解剖关系是经导管封堵术后传导阻滞发生率较高的主要原因,术前如何通过影像学分型,选择恰当的病例,以及封堵方式和封堵器材的改进将是今后亟待解决的课题。     

小腰大边封堵器介入治疗室间隔缺损伴膜部瘤形成的评价

目的评价应用新型国产小腰大边封堵器介入治疗室间隔缺损(VSD)伴膜部瘤形成的可行性、安全性和疗效,总结其技术难点与治疗策略。方法49例VSD伴膜部瘤形成患者,造影测量VSD左室面入口直径为8~22(13.8±4.9)mm,右室面均有2个或以上出口,最大出口直径为2~14(5.6±3.1)mm。根据膜部瘤大小、形态、位置及膜部瘤组织黏连牢固程度,植入不同类型和型号国产小腰大边封堵器,封堵器直径为4~16(6.8±2.6)mm。封堵后15min重复左心室造影和经胸心脏超声检查(TTE),观察封堵即刻效果。术后连续心电图(ECG)监护5d,定期行ECG、心脏超声检查(TTE)。结果49例术后15min左心室造影、TTE显示:45例完全封堵,4例术后造影示少量分流(<3mm),1个月后超声复查无残余分流。术中并左、右束支传导阻滞分别为3例和2例,均为一过性,1周内恢复。49例室间隔膜部瘤应用国产小腰大边封堵器封堵治疗均获成功。结论经导管采用国产小腰大边封堵器治疗VSD膜部瘤疗效可靠。技术关键是通过对膜部瘤大小、形态、位置及膜部瘤组织黏连牢固程度判断确定封堵部位及合适封堵器。     

改良切口经胸微创封堵术治疗膜周室间隔缺损

目的 探讨改良切口经胸微创室间隔缺损封堵术的可行性和安全性.方法 2011年5月至2015年5月,289例单纯膜周室间隔缺损患儿在我中心接受改良切口经胸室间隔缺损封堵术.该操作采用长约1～2 cm的微创切口,无需损伤胸骨,完全食道超声引导下完成室间隔缺损封堵.术后1、3、6个月定期随访.结果 289例患儿中,277例(95.8％)成功完成微创封堵;12例封堵失败,改行常规外科修补术.277例患儿均经改良微创切口入路,切口长度1～2cm,平均(1.53±0.46)cm.均未损伤胸骨,亦未放置引流管.术后早期均未出现心包积液.膜周室缺直径平均(5.30±2.88)mm.封堵器大小4～12mm,平均(6.70±3.10)mm,包括对称封堵器191例,偏心封堵器86例.12例(4.3％)患儿术中存在少量残余分流.9例(3.2％)术后发生不完全性右束支传导阻滞;1例患儿于术后4d出现完全性房室传导阻滞,经激素治疗5d后好转.所有患儿平均住院时间(3.2±0.8)d,随访期间,没有主动脉瓣反流、恶性心律失常、封堵器脱落等严重并发症发生.至随访结束仍有4例(1.4％)存在少量残余分流.结论 改良切口经胸壁微创封堵术,不损伤胸骨,可以有效治疗膜周室间隔缺损.但其长期疗效尚需进一步随访研究.     

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.