Amplatzer封堵器治疗动脉导管未闭及房间隔缺损疗效观察

目的评价Amplatzer封堵器治疗动脉导管未闭(PDA)及房间隔缺损(ASD)的疗效。方法将12例PDA、4例ASD于X线透视和经胸超声心动图(TTE)监视下置入Amplatzer封堵器。术后复查TTE及X线检查。结果16例封堵器置入均获成功,成功率100%,术后24hTTE显示2例(12.5%)存在残余分流,分别于72h、1周后分流消失。随访6个月,TTE检查无残余分流,X线胸片示肺血流量减少。结论采用Amplatzer封堵器治疗PDA及ASD安全有效、操作简便、成功率高。     

Amplatzer封堵器治疗动脉导管未闭及房问隔缺损疗效观察

目的评价Amplatzer封堵器治疗动脉导管未闭(PDA)及房间隔缺损(ASD)的疗效。方法将12例PDA、4例ASD于X线透视和经胸超声心动图(TIE)监视下置入Amplatzer封堵器。术后复查TTE及X线检查。结果16例封堵器置入均获成功，成功率100％，术后24h TTE显示2例(12．5％)存在残余分流，分别于72h、1周后分流消失。随访6个月，TTE检查无残余分流，X线胸片示肺血流量减少。结论采用Amplatzer封堵器治疗PDA及ASD安全有效、操作简便、成功率高。     

儿童动脉导管未闭和房间隔缺损的介入治疗

目的 评价应用Amplatzer封堵器治疗儿童房间隔缺损(ASD)和动脉导管未闭(PDA)的效果、安全性夏共并发症等。方法 超声心动图及临床诊断ASD、PDA患儿，在透视和(或)经胸超声心动图(TTE)下置入Amplatzer封堵器，术后24h、1个月、3个月、6个月、1年及以后每年分别经胸超声、心电图和X线胸片检查评价治疗效果。结果 16例ASD患儿，球囊测量值13～28mm，选择封堵器直径13～30mm；25例PDA患儿。造影测量值2～7．7mm，选择封堵器直径4～12mm。技术成功率为100％，术中未发生任何并发症，无急诊手术病例。术后即刻造影或TTE显示10例存在残余分流，术后3个月TTE检查所有病例无残余分流和再通，肺动脉压下降。结论 应用Amplatzer封堵器治疗PDA和ASD，具有操作简单、安全、损伤小、成功率高等优点，适合儿童继发孔型ASD及各种类型PDA的介入治疗。     

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.     

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     

单纯经胸超声心动图引导下经皮动脉导管未闭ADOⅡ封堵术的单中心临床研究

目的总结以经胸超声心动图(transthoracic echocardiography,TTE)作为唯一影像学工具,并应用ADOⅡ封堵器开展动脉导管未闭(patent ductus arteriosus,PDA)封堵术的临床治疗经验。方法以2018年7月至2019年6月浙江大学医学院附属儿童医院收治的68例于单纯TTE下行PDA封堵术的患儿为研究对象,其中男38例,女30例,平均月龄(29. 35±16. 52)个月,平均体重(13. 09±4. 19) kg。术前通过TTE测量PDA的主动脉端内径、肺动脉端内径、最细处内径及PDA长度,并根据测量结果选择合适的封堵器。术中采用股动脉径入路,于单纯TTE引导下监测PDA ADOⅡ封堵术的全过程。术后1 d即开始进行病情观察,门诊随访1～6个月。结果 68例PDA肺动脉端平均开口直径(2. 38±0. 97) mm,主动脉端平均开口直径(4. 48±2. 70) mm,PDA平均长度(5. 37±1. 42) mm。ADOⅡ平均大小(4. 46±1. 20) mm,平均长度(4. 38±0. 79) mm,其中管型27例,漏斗形41例。64例在TTE引导下成功完成经皮PDA封堵术,平均手术时间(11. 16±5. 18) min; 4例中转为X线引导下经皮PDA封堵术。术后平均住院时间(3. 24±1. 24) d,平均住院费用(23 732. 20±1 686. 02)元。术后门诊随访1～6个月,无一例出现封堵器移位、残余分流、肺动脉和主动脉流速增快、上下肢血压反常、瓣膜损伤、心包积液、机械性溶血、外周血管损害等并发症。结论单纯TTE引导下,应用ADOⅡ经皮封堵婴幼儿PDA的可行性和成功率均较高,同时能够避免X线及造影剂造成的副反应,但对于肺动脉开口细小的PDA则需要做好传统介入封堵术预案。     

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。     

动脉导管未闭直径的不同测量方法在介入治疗中的应用

目的 评价经胸超声心动图(TTE)与心血管造影在介入治疗中测量动脉导管未闭(PDA)直径的应用价值.方法 拟行PDA堵闭的PDA患儿52例.应用TTE观察降主动脉与肺动脉间相通的动脉导管的位置、直径及长度,测量PDA最窄处直径;心血管造影选用猪尾管,行左侧位造影,测量其最窄径线.并对2种方法的测量值进行比较.采用SPSS 12.0软件进行统计学分析.结果 心血管造影显示52例PDA患者中,漏斗型45例,管型5例,漏斗型并管型、PDA结扎手术后残余分流各1例;TTE显示漏斗型46例,管型4例,窗型、PDA结扎手术后残余分流各1例.心血管造影测量的PDA直径为(3.72±2.51) mm,显著＜TTE测量值(4.47±2.31) mm,二者比较差异具有显著性意义(t=5.17 P＜0.05).结论 TTE可用作术前病例的初步筛选,心血管造影决定PDA的分型,PDA直径需结合心血管造影与TTE测量综合分析,术后有无残余分流及随访依靠TTE.TTE与心血管造影在PDA介入治疗中互相补充,缺一不可.     

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。     

应用室间隔缺损封堵器治疗婴幼儿特殊形状动脉导管未闭疗效观察

目的评价室间隔缺损(VSD)封堵器治疗婴幼儿特殊形状动脉导管未闭(PDA)的临床疗效。方法分析2017年7月至2018年7月经主动脉弓降部造影提示窗型PDA或动脉导管长度7 mm管型PDA,并选择VSD封堵器进行介入治疗的27例患儿的临床资料。结果 27例患儿中窗型PDA 12例,男7例、女5例,中位年龄8.7个月(6个月～3岁);管型PDA 15例,男6例、女9例,中位年龄18个月(11个月～5岁)。窗型PDA选择膜部VSD封堵器,管型PDA选择肌部VSD封堵器封堵;按照窗型PDA肺动脉端内径加3～6 mm、管型PDA肺动脉端内径加2～4 mm选择封堵器腰部大小。所有患儿均成功封堵,术后均无残余分流。术后24小时、1个月、3个月、6个月、12个月复查胸片提示封堵器位置良好。术后24小时、1个月、3个月、6个月、12个月降主动脉流速均较术前明显降低,差异有统计学意义(P0.05);术后左肺动脉流速较术前无变化,差异无统计学意义(P0.05)。结论使用VSD封堵器介入治疗特殊形状PDA是一种有效可行的治疗方法。     

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.