经静脉植入埋藏式心脏起搏器治疗儿童房室传导阻滞长期随访分析

目的分析房室传导阻滞患儿经静脉植入埋藏式心脏起搏器治疗、随访及并发症处理。方法 1992年5月至2013年12月贵州省人民医院心内科安置埋藏式心脏起搏器治疗症状性高度或Ⅲ度房室传导阻滞患儿11例,年龄5~14岁,平均(12.0±2.8)岁,均采用经静脉植入埋藏式心脏起搏器治疗,并随访1~22年(平均6.1年)。结果患儿经头静脉(1例)或锁骨下静脉(10例)途径植入起搏电极,7例植入心室按需型起搏器,4例植入全自动型起搏器,心房起搏电极安置于右心耳或房间隔,4例右心室起搏电极安置于右心室心尖部,7例安置于右心室间隔部。规律随访期内,起搏阈值、感知及阻抗比较差异均无统计学意义(P0.05)。1例患儿术后5年发生起搏电极断裂,予更换起搏系统;1例患儿术后3年出现左心功能不全,升级为心脏再同步化治疗后病情缓解。结论在埋藏式心脏起搏器植入、术后随访过程中,需要针对儿童的有关生理、病理特点采取相应的对策,儿童经静脉植入埋藏式心脏起搏器简便易行、安全有效。     

儿童左束支区域起搏六例临床观察

目的探讨儿童左束支区域起搏(LBBAP)的安全性及有效性.方法回顾性分析2019年1月至6月于北京安贞医院小儿心脏科住院采用LBBAP方式行永久心脏起搏器植入术的6例患儿(男1例、女5例)的临床资料、起搏心电图及参数并进行随访.组间比较采用t检验.结果6例患儿年龄9~14岁,体重26~48 kg;三度房室传导阻滞5例,右室心尖起搏术后伴心功能下降1例;1例患儿心功能降低,余5例心功能均正常;QRS波时限(95±13)ms;左心室舒张末径(LVEDD)Z值为1.85±0.65.起搏心电图V1呈右束支传导阻滞样,QRS波时限(111±20)ms,与术前相比,差异无统计学意义(t=-1.610,P>0.05).起搏阈值为(0.85±0.26)V,感知(15.0±4.3)mV,阻抗(717±72)Ω.3例可记录到P电位.起搏钉至左心室激动时间为(56±5)ms,不同输出电压下数值恒定.术后超声提示电极均位于室间隔左心室心内膜下.随访无心肌穿孔、电极脱位等并发症发生,患儿术后3个月阈值、感知及阻抗分别为(0.60±0.09)V、(16.1±3.9)mV、(662±78)Ω.左心室射血分数(LVEF)降低者LBBAP术后3d恢复正常(45%比57%).术后3个月LVEDD Z值降至(1.1±0.3),较术前明显减小(t=2.383,P<0.05).结论LBBAP可实现窄QRS波起搏,接近生理性起搏,起搏参数稳定,可快速、有效地纠正长期心动过缓所致的左心扩大及长期右室心尖起搏所致的心功能低下及心脏扩大.较大年龄儿童行LBBAP近期安全性、有效性好,远期潜在风险有待进一步观察研究.     

儿童经胸植入左房左室心外膜永久双腔起搏器疗效探讨

目的 探讨植入左房左室心外膜永久双腔起搏器治疗儿童完全性房室传导阻滞的可行性、优越性及疗效.方法 本组11例完全性房室传导阻滞患儿,中位年龄4.0岁(0.5 ～7.6岁),其中男6例,女5例,药物治疗均无效,均植入心外膜左房左室永久双腔起搏器.本组术前均为右室临时或永久起搏方式,3例存在心功能不全.经左侧第4肋间腋前线开胸,将心外膜起搏电极固定于左心耳及左室心外膜,于腹部左季肋下制作囊袋置入脉冲发生器,经皮下隧道连接起搏电极导线.术后随访心功能变化、起搏参数和功能、心电图参数.结果 11例患儿均成功植入左房左室心外膜永久双腔起搏器.左室起搏QRS间期较术前右室起搏QRS间期明显缩短[(140+24)ms vs.(180±33)ms,t=8.8,P＜0.05].3例右室或右房右室起搏方式存在心功能受损患儿,植入左房左室心外膜起搏器随访2 ～ 14个月,左室射血分数逐渐恢复正常(65％±8％),与植入前左室射血分数(30％±15％)相比差异有统计学意义(t=5.6,P＜0.05).其余8例患儿随访期间心脏大小及左室收缩功能保持在正常范围.全部患儿随访显示起搏及感知功能良好.结论 在因条件限制需植入心外膜永久心脏起搏器的房室传导阻滞患儿,可选择左房左室心外膜起搏,以尽可能小的创伤、最大限度保护患儿的心脏功能,有效避免或逆转起搏器综合征,可作为植入心脏起搏器心外膜电极首选及常规部位.     

食管超声引导下经皮儿童房间隔缺损封堵术的疗效评价

目的探讨儿童单纯经食管超声心动图(Transesophageal Echocardiography,TEE)引导下经皮房间隔缺损(Atrial Septal Defect,ASD)封堵术的可行性、安全性和有效性。方法收集2014年11月至2016年8月单纯TEE引导下经皮封堵ASD患儿118例,男性51例,女性67例,年龄13~180个月,平均(68.39±39.12)个月,体重10~77 kg,平均(21.14±11.34)kg。术中采用TEE引导并监测房间隔缺损封堵术的全过程。术后门诊随访3~6个月,予经胸超声心动图(Transthoracic echocardio-graphy,TTE)检查,评估封堵效果。结果 TEE引导下经皮成功封堵单孔型继发孔ASD 118例。继发孔ASD大小3~24 mm,平均(9.52±4.12)mm,封堵器大小6~32 mm,平均(14.19±4.60)mm。118例均顺利完成封堵术,术后即刻TEE检查及术后3~6个月TTE随访,均未见封堵器移位、瓣膜损伤、心包积液、外周血管损害等并发症。结论 TEE引导下经皮封堵儿童ASD安全有效,成功率高,并发症的发生率低、设备投入少,易于推广。     

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。     

经心内外膜行起搏治疗的24 例体质量<8 kg 患儿临床分析

目的探讨体质量8 kg患儿行起搏器治疗的策略。方法回顾分析2009—2019年间收治的24例体质量8 kg,行永久性起搏器植入术患儿的临床资料。结果 24例患儿中,男16例、女8例,中位年龄6.5个月(1～22个月),中位体质量6.4 kg(2.4～7.9 kg),术后随访1 ～120个月,中位随访20.5个月。其中15例患儿行心内膜起搏,9例行心外膜起搏。15例患儿行心内膜起搏后发生囊袋感染3例,分别表现为囊袋局部血肿、渗出、手术切口裂开;3例患儿均行静脉抗感染治疗,其中表现为囊袋渗出及手术切口裂开的2例患儿行囊袋清创术,彻底消毒起搏发生器,另1例患儿家属拒绝再行清创术;心内膜起搏后因起搏器功能异常行二次手术3例,其中1例因三尖瓣反流加重于术后3个月更换导线,1例因起搏器感知不良于术后6年更换导线及发生器,1例术后9年因电量耗竭再行起搏器植入,考虑双腔起搏可能更符合生理特性,更换起搏模式。9例患儿行心外膜起搏术后因导线或起搏器功能异常行二次手术2例,均为电量过早耗竭,分别于术后1.5年及2年更换为心内膜起搏,使用周期较短。结论体质量8 kg患儿经心内膜及心外膜行永久性起搏器植入均具有可行性,且在严控指征下安全有效,但需注意囊袋感染,并在术中及随访中需谨慎三尖瓣反流问题。     

小儿心脏起搏器

本文介绍近年来心脏起搏技术的进展,小儿心脏起搏器及其应用。植入起搏器指征 (1)先天性完全性房室传导阻滞:1987年 Dewey 等报告,对27例先天性完全性房室传导阻滞平均随访8±3年发现,心率<50次/分13例小儿中的8例,发生猝死3例、晕厥3例或活动时疲劳2例。作者认为,这类患儿理应植入心脏起搏器。(2)先天性房室传导阻滞伴 QT 间期延长:这类患儿植入心脏起搏器后,维持β受体阻滞剂治疗可能有益。(3)先天性 QT 间期延长综合征患儿,     

临时心脏起搏治疗儿童缓慢性心律失常所致 严重血流动力学紊乱 3 例报告

目的探讨临时心脏起搏治疗在儿童缓慢性心律失常所致严重血流动力学紊乱中的作用。方法回顾分析3例以临时心脏起搏救治的缓慢性心律失常所致严重血流动力学紊乱患儿的临床资料。结果 3例患儿中1例男性12岁,2例女性均为5岁;均先以药物治疗,病情仍持续加重,在入院后10小时内安装临时心脏起搏器治疗,血流动力学障碍很快得到改善。1例男性患儿在术后10天、1例女性患儿在术后11天撤除临时心脏起搏器,经短期随访,均预后良好;1例女性患儿在术后21天家属放弃治疗,门诊随访1年,患儿存活,但有心功能不全表现。结论严重缓慢性心律失常所致血流动力学紊乱时,及时安置临时心脏起搏器装置效果良好。     

介入治疗儿童动脉导管未闭132例随访观察

目的了解介入治疗儿童动脉导管未闭(PDA)术后并发症的发生情况及其演变。方法通过询问病情及行胸片、心电图及心脏超声等辅助检查,对所完成的132例PDA患儿进行4a跟踪随访,随访时间为(28±16)个月。其中45例使用Amplatzer进口封堵器,87例使用深圳先健国产封堵器。结果术后第2天,经胸超声(TTE)证实128例封堵器位置良好,4例存在残余分流(4/132例),其中1例发生溶血(1/132例),经治疗后,症状消失。随访1个月,所有残余分流均消失。股动脉血栓形成1例(1/132例),经溶栓治疗,股动脉搏动完全恢复。9例出现短时间发热(9/132例)。随访过程中6例出现不明原因头痛。2例发生严重事件,1例术后6个月出现长期发热,TTE发现封堵器表面有赘生物形成,最终经外科开胸治疗痊愈。另1例于术后18个月死于不明原因的肺动脉高压。结论介入治疗儿童PDA是一种安全、有效方法,但同时也存在许多并发症,术后需认真而长期的随访观察。     

婴幼儿左束支区域起搏10例探讨北大核心CSCD

目的:探讨左束支区域起搏应用于婴幼儿的可行性、安全性及有效性。方法:回顾性病例总结。选择2020年9月至2021年9月于清华大学第一附属医院住院的10例心动过缓婴幼儿(年龄≤3岁)为研究对象,均具有心脏永久起搏器植入指征并行左束支区域起搏,记录术中资料(起搏参数、影像及心电图资料),完善心脏超声检查,术后门诊规律随访。术前及术后资料比较采用配对样本t检验。结果:10例患儿(男6例、女4例),年龄(1.6±0.7)岁,体重(10.3±2.5)kg,均成功接受左束支区域起搏治疗。术后心电图QRS波时限为(100±9)ms,心室起搏比例为(97±7)%。术后随访时间为6(6,12)个月。术后1周时,10例患儿左心室舒张末期内径Z值较比术前明显缩小(1.3±0.6比3.6±1.1,t=9.37,P<0.001),随访期间10例患儿心功能均保持良好,末次随访时左心室射血分数为(66±4)%。末次随访时起搏电极阈值较比术中稍有升高但均≤1.0 V,在临床可接受范围[(0.8±0.1)比(0.5±0.1)V,t=-5.27,P=0.001];电极感知及阻抗与术中对比差异均无统计学意义[(16±5)比(14±4)mV,(584±88)比(652±86)Ω,t=-0.83、2.26,P=0.426、0.050]。随访期间未见电极脱位、电极故障等并发症发生。结论:左束支区域起搏可安全有效地应用于婴幼儿,起搏QRS波时限窄且术后心功能保持良好,电极参数稳定。     

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     

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.     

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.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

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.