儿童大型动脉导管未闭介入封堵术治疗的临床效果

目的：探讨Amplatzer法介入治疗大型动脉导管未闭(PDA)的临床效果。方法：227例大型PDA患儿,其中合并肺动脉高压者63例,应用Amplatzer封堵器行动脉导管封堵术,年龄中位数为3.2岁,体重中位数为10.6 kg,PDA最窄处直径中位数5.7 mm。结果：227例患儿中,216例封堵成功（95.2％）。合并肺动脉高压者治疗前肺动脉平均压为45±19 mm Hg,封堵术后降为22±12 mm Hg（P<0.05）。216例封堵成功患儿中,术后造影显示即刻完全封堵109例（50.5％）,术后24 h超声心动图检查完全封堵者181例（83.8％）。术后6个月和12个月超声心动图检查示封堵成功的患儿均无残余分流。结论：Amplatzer法介入封堵治疗儿童大型PDA是一种安全有效的方法。     

动脉导管未闭介入治疗临床观察

目的探讨Amplatzer封堵器治疗动脉导管未闭(PDA)的疗效、安全性及可靠性。方法12例经临床、心电图、X 线和心脏彩色多普勒超声检查确诊PDA患儿,采用美国AGA公司生产Amplatzer自膨性蘑菇状封堵器,选择封堵器的型号较PDA最窄径大2 mm,用6F传送鞘经静脉置入,术后即刻行胸主动脉造影。手术前、后均行血液动力学监测,术后24 h,3、6、12 个月作心脏彩色多普勒超声及X线检查。结果PDA最窄径18～6.3 mm,手术成功率100%,术后即刻听诊杂音消失11例, 轻度收缩期杂音1例。15 min后胸主动脉造影示11例完全闭塞(91.6%),随访3～12个月,患儿均无PDA再通及封堵器移位。结论Amplatzer封堵器操作简便,安全有效,成功率高,是目前介入治疗PDA最理想方法。     

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。     

蘑菇伞封堵器介入治疗婴幼儿动脉导管未闭的临床研究

目的 评价3岁以内婴幼儿动脉导管未闭应用蘑菇伞封堵器介入治疗的临床效果.方法 全组21例,年龄9个月～3岁,平均(2.2±0.8)岁,体重6.0～13.2 kg,平均(12.6±2.5)kg.主动脉侧位造影确定动脉导管的形态和导管最窄处直径,选择合适封堵器.经静脉途径置入封堵器.术后24 h、1个月、3个月、1年、2年及3年复查胸部X线平片及超声心动图和心电图观察封堵器的位置、残余分流情况、肺动脉压及心脏大小.结果 21例患儿植入封堵器均获成功,技术成功率为100%.造影测定PDA最窄处直径2.5～10.9(5.8±2.6)mm.应用直径4～18 mm大小的PDA封堵器进行封堵.随访无严重并发症.结论 应用蘑菇伞封堵器治疗婴幼儿期动脉导管未闭具有操作简便、安全有效、技术成功率高及封堵效果好等优点.     

165例5岁以下小儿房间隔缺损的介入治疗

目的总结双盘封堵器(Amplatzer)关闭年龄小、体重轻患儿的继发孔型房间隔缺损(ASD)的病例。方法1998年8月至2004年5月,165例5岁和18kg以下小年龄组继发孔型ASD病例接受Amplatzer封堵器介入治疗。所有患儿均经临床体检、X线胸片、心电图、经胸超声心动图(TTE)确诊为继发孔型ASD。TTE观察和测量ASD和房间隔(IAS)最大径,测量球囊导管测量ASD最大伸展径,必要时加用食道超声(TEE)测定,筛选后的患儿依此选择封堵器。结果163例成功封堵ASD,成功率98.8%。本组ASD最大径(8～30)mm,平均(18.3±5.1)mm,选择封堵器直径(8～30)mm,平均(18.6±5.0)mm,P>0.05。Qp/Qs=3.3±2.0。147例(89.0%)为单纯单孔ASD病例;6例为多孔ASD,其中3例伴有房间隔瘤样改变,均用一个封堵器成功封堵ASD。另外12例合并其他心内畸形,其中6例合并肺动脉瓣狭窄(PS),6例合并动脉导管未闭(PDA)。右心容量超负荷术后明显改善。本组中大ASD占60.0%(100)例。操作上有一定难度。结论Amplatzer封堵器关闭5岁以下儿童房间隔缺损是可行的,但不主张2岁以下行介入治疗。严格掌握适应证;良好的小儿心血管内外科条件是成功封堵的基本保证。     

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。     

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。     

动脉导管造影联合经胸超声在动脉导管未闭封堵术治疗中的应用

目的：探讨经动脉导管造影联合经胸超声能否作为动脉导管未闭封堵术的一种简化治疗方法。方法：选择欲行动脉导管未闭介入封堵术患儿40例,随机分为观察组20例和对照组20例。对照组应用传统的动脉导管未闭介入封堵技术。观察组采用简化的动脉导管未闭介入封堵术,并借助经胸超声实时监测。结果：观察组20例患儿用简化方法行动脉导管未闭封堵术均一次封堵成功,超声显示封堵器位置良好,左、右肺动脉、降主动脉血流速度均在正常范围内。观察组患儿放射线曝露时间明显少于对照组;观察组患儿无一例与血管穿刺相关的血管并发症发生,但对照组患儿发生4例血管并发症。患儿的恢复时间和滞留于监护室的时间也相应缩短;观察组总的医疗费用少于对照组。结论：经动脉导管造影联合经胸超声行PDA封堵术是一种良好的简化封堵方法。［中国当代儿科杂志,2010,12（2）：103-105］     

动脉导管未闭封堵的临床研究

目的 总结经导管介入封堵治疗动脉导管未闭（PDA）的临床经验,以探讨其指征、方法学及并发症。方法 统计10所医院共3215例患儿,其中男1331例,女1884例;≤2岁734例,〉2岁2481例。采用的介入治疗方法有6种,包括Porstmann法、Rashkind法、Sideris法、Cook弹簧栓法、Pfm弹簧栓法及Amplatzer法,其中Amplatzer法及弹簧栓法分别占总例数的73％及14％。结果 总技术成功率98％,其中弹簧栓法、Amplatzer法分别达到99．1％与99．7％,Porstmann法成功率最低,约92％。用弹簧栓法及Amplatzer法治疗的所有病例中有12例未成功,失败原因：并发艾氏综合征5例,大型窗形或短管形PDA3例,常规造影PDA显示不清1例,大型PDA合并重症肺炎、心衰1例,伴重度肺高压装置放置后血压下降1例,术中封堵器脱落、转外科手术1例。并发症：残余分流103例,股动脉血栓形成29例,术后溶血8例,封堵器脱落2例,并发主动脉缩窄2例,左肺动脉狭窄1例。结论 PDA经导管介入封堵治疗PDA创伤小、操作简便、安全、并发症低,与外科手术疗效相仿,在其适应证范围内可作为外科的替代疗法。弹簧栓封堵器适用于中小型PDA,而Amplatzer蘑菇伞状PDA封堵器则适用于中大型PDA。     

儿童动脉导管未闭介入治疗单中心疗效及中长期随访观察

目的探讨儿童动脉导管未闭(PDA)介入治疗并发症的发生及原因。方法收集2004年1月1日至2019年1月1日行介入封堵治疗PDA患儿的临床资料,比较其封堵前后及随访1年的变化。结果共收集1 408例患儿,男482例、女926例,中位月龄27.0(2.0～215.0)月,介入成功封堵1 404例(99.72%)。PDA内径术前超声测值为3.3(0.1～18)mm,主动脉造影为2.2(0.1～18)mm;选用封堵器大小为(8.47±2.52)mm。术后24小时心脏超声复查有残余分流125例,血小板减少21例,心律失常31例,溶血2例,动脉血栓3例,假性动脉瘤4例,右髂总动脉破裂1例,右肾挫裂伤1例,封堵器移位4例,降主动脉狭窄1例,肺动脉狭窄1例,三尖瓣前瓣腱索断裂1例。多元logistic回归分析显示,女性、肺动脉高压严重程度递增、封堵器直径增大为术后发生残余分流的独立危险因素(P0.05)。结论儿童PDA介入治疗安全、有效;PDA内径较大、合并中重度肺动脉高压患儿术后并发症发生率较高。     

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.