左向右分流型先天性心脏病患儿肌钙蛋白Ⅰ的变化

目的探讨不同类型左向右分流先天性心脏病(CHD)患儿血肌钙蛋白Ⅰ(cTnⅠ)的变化。方法入选146例继发孔型房间隔缺损(ASD)、132例室间隔缺损(VSD)患儿,300例健康对照儿童,测定血cTnⅠ及脑利钠肽前体(NT-proBNP)水平,并分析其与患儿临床情况的相关性。结果 ASD组、VSD组以及对照组之间的血清cTnⅠ和NT-proBNP水平的差异均有统计学意义(H=3.89、5.27,P0.01),VSD组患儿血清cTnⅠ和NT-proBNP水平均高于ASD组,差异有统计学意义(P均0.05)。VSD组的肺循环/体循环压力比(Pp/Ps)、肺血管阻力指数(PVRI)以及标准化左心室舒张末期容积高于ASD组,差异有统计学意义(P均0.05)。多元回归分析显示,在VSD患儿中Pp/Ps对cTnⅠ水平有显著影响(β=0.81,SE=0.03,P=0.000)。结论左向右分流型CHD所引起的心脏容量与压力变化可引起心肌损伤并最终导致不可逆性的心肌重塑。cTnⅠ可用于评估VSD所引起的不同程度心肌损伤。     

左向右分流型先天性心脏病患儿心脏结构和功能改变与骨龄指数的相关性

目的探讨左向右分流型先天性心脏病(CHD),包括房间隔缺损(ASD)、室间隔缺损(VSD)、动脉导管未闭(PDA)患儿的骨龄变化以及心脏结构和功能改变与骨龄指数(BAI)的相关性。方法收集确诊左向右分流型CHD共130例,其中ASD 52例、VSD 46例、PDA 32例,超声检测各项心脏结构和功能指标,摄左手及腕关节正位片,应用最新《中国儿童骨龄评分法》图谱进行骨龄评定并计算BAI,对比分析各组骨龄以及CHD各项心脏结构和功能指标与BAI的直线相关性。结果 ASD、VSD与PDA患儿BAI的差异有统计学意义(P0.05);PDA组的BAI分别高于ASD组和VSD组,差异均有统计学意义(P均0.05)。无PAH、轻度PAH以及中重度PAH三组的BAI分别为(0.84±0.09)、(0.75±0.07)和(0.65±0.08),差异有统计学意义(F=27.77,P=0.000);小缺损组的BAI为(0.82±0.09),高于中大缺损组的(0.73±0.10),差异有统计学意义(t=3.54,P=0.002)。ASD、VSD患儿缺损大小(DS)与BAI均呈负相关(r=-0.48、-0.54,P均0.05);ASD、VSD、PDA患儿肺动脉收缩压(PASP)以及肺循环血流量/体循环血流量比值(Qp/Qs)均与BAI呈负相关(r=-0.64~-0.38,P均0.05)。结论左向右分流型CHD患儿的骨龄及BAI均显著落后于正常同龄儿童;左向右分流型CHD患儿骨龄落后程度与PASP、DS及分流量大小相关。     

左向右分流先天性心脏病儿童红细胞氧化应激状态相关研究

目的：研究左向右分流型先天性心脏病（先心病）患儿红细胞氧化应激状态,探讨其相互关系及临床意义。方法：左向右分流先心病患儿31例为实验组,再根据解剖特点分为亚组,其中房间隔缺损（ASD）7例、室间隔缺损（VSD）12例、动脉导管未闭（PDA）4例、卵圆孔未闭（PFO）6例、完全性心内膜垫缺损（复杂组）2例;门诊健康体检儿童20例为对照组,两组年龄均为1月至3岁。用酶联免疫法（ELISA 法）测定红细胞内超氧化物歧化酶（SOD）及丙二醛（MDA）含量;魏氏法检测ESR,血气分析仪（GEM Premier 3000）测定PaO2、PaCO2。结果：先心病组与对照组比较红细胞内MDA含量升高,SOD含量降低,两组比较差异有统计学意义（P<0.05）;合并心力衰竭时SOD较对照组下降更为明显,MDA含量明显升高,差异有统计学意义（P<0.01）。各解剖分型组中,SOD含量以PFO组最高,复杂组最低,PFO组与ASD组、VSD组、复杂组间比较差异有统计学意义（P<0.05）;MDA含量以VSD组最高,PFO、ASD及PDA组有所降低,复杂组最低,组间比较差异有统计学意义（P<0.05）。相关分析显示先心病组ESR及MDA含量与SOD含量的改变呈负相关（r=-0.191,r=-0.312;P<0.05）,而ESR与PaO2的变化呈正相关（r=0.216,P<0.05）。结论：左向右分流先心病患儿体内存在氧化应激,测定红细胞SOD、MDA含量可较好反映疾病严重程度。［中国当代儿科杂志,2010,12（6）：440-443］     

小儿先天性心脏病介入治疗后心肌损伤和炎症反应的研究

目的：研究先天性心脏病（CHD）介入治疗后7 d心肌损伤和炎症反应。方法：选择77例CHD患儿,其中室间隔缺损（VSD）12例,肺动脉瓣狭窄(PS)14例,房间隔缺损（ASD）14例,动脉导管未闭（PDA）37例。检测术后即刻、术毕、术后6 h、24 h、72 h及7 d 的血清心肌酶（AST、CK、CKMB）、肌钙蛋白I(cTnI)和CRP值的变化。结果：VSD组AST水平于术后即刻,术后6 h和术后24 h明显高于其他3种类型CHD组（P<0.05）;术后即刻及术后6 h各组间CK及CKMB水平差异有统计学意义,以VSD组最高;术后72 h和术后7 d PDA组CRP水平明显高于其他3种类型CHD组（P<0.05）。与术前相比,术后6 h和24 h,4种类型CHD组AST均明显升高（P<0.05）;术后即刻和术后6 h,VSD组CK和CKMB水平较术前明显升高（P<0.05）;术后即刻和术后6 h,VSD、PDA和PS组cTnI水平较术前明显升高（P<0.05）;PDA组CRP水平术后24 h、72 h和7 d较术前明显升高（P<0.05）。结论：介入治疗CHD 7 d内可引起轻微心肌损伤,以VSD明显,主要集中在术毕至术后24 h较短时间内,未引起明显炎症反应。     

重症肺炎患儿血清N末端脑钠素原和肌钙蛋白I水平变化及意义

目的探讨重症肺炎患儿血清N末端脑钠素原(NT-ProBNP)和肌钙蛋白I(cTnI)水平的变化及其临床价值。方法重症肺炎患儿72例,分为重症肺炎无心力衰竭(HF)组(56例)与重症肺炎合并HF组(16例);另选择30例健康儿童作为正常对照组。检测并比较各组血清NT-ProBNP和cTnI水平。结果三组间NT-ProBNP和cTnI水平差异有统计学意义(P均=0.000)。重症肺炎无HF组与合并HF组的NT-ProBNP和cTnI水平高于对照组;重症肺炎合并HF组血清NT-ProBNP及cTnI水平高于重症肺炎无HF组,差异有统计学意义(P均0.01)。重症肺炎患儿中,合并HF组的cTnI异常率明显高于无HF组,差异有统计学意义(P=0.037),而二组的NT-ProBNP异常率差异无统计学意义(P=0.375);重症肺炎患儿的NTProBNP与cTnI水平呈显著正相关(r=0.85,P=0.000)。结论重症肺炎患儿心肌损害明显,NT-ProBNP、cTnI可作为辅助诊断儿童重症肺炎心肌损害及严重程度的重要血清学标志物。     

常见左向右分流先天性心脏病的治疗及时机选择

先天性心脏病(简称先心病)按血流动力学即血流方向分为三大类:左向右分流型;右向左分流型;无分流型.左向右分流型先心病系指血流由体循环通过异常交通流向肺循环;常见的左向右分流型先心病有动脉导管未闭(PDA)、继发孔房间隔缺损(ASD)、室间隔缺损(VSD),除此以外还有主肺动脉间隔缺损、冠状动脉瘘、左室右房通道等.     

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目的对房间隔缺损(ASD)、室间隔缺损(VSD)、动脉导管未闭(PDA)的介入治疗(Amplatzer封堵器)和外科手术治疗的临床疗效进行比较。方法研究对象为2004-05—2005-05在北京安贞医院小儿心脏科住院患儿80例,符合单一左向右分流畸形ASD、VSD、PDA,根据治疗方法不同分为介入组(40例)和手术组(40例);比较2组在疗效、费用、并发症、输血量及住院时间等方面的异同;通过放免法测定2组ASD及VSD治疗前后的心钠素(ANP)水平。结果2组手术成功率均为100%;介入组均未输血,手术组均输血治疗;术后住院时间介入组少于手术组(P<0.01);治疗费用介入组高于手术组(P<0.01);ANP水平,外科手术及介入治疗后均较治疗前降低。结论单一ASD、VSD、PDA的介入治疗与手术治疗临床效果相同;介入组不需输血,术后住院时间短,但治疗费用较昂贵。     

先天性心脏病介入治疗与传统外科手术治疗的临床比较

目的对房间隔缺损(ASD)、室间隔缺损(VSD)、动脉导管未闭(PDA)的介入治疗(Amplatzer封堵器)和外科手术治疗的临床疗效进行比较。 方法研究对象为2004 05—2005 05在北京安贞医院小儿心脏科住院患儿80例,符合单一左向右分流畸形ASD、VSD、PDA,根据治疗方法不同分为介入组(40例)和手术组(40例);比较2组在疗效、费用、并发症、输血量及住院时间等方面的异同;通过放免法测定2组ASD及VSD治疗前后的心钠素(ANP)水平。 结果2组手术成功率均为100%;介入组均未输血,手术组均输血治疗;术后住院时间介入组少于手术组(P<0.01);治疗费用介入组高于手术组(P<0.01);ANP水平,外科手术及介入治疗后均较治疗前降低。 结论单一ASD、VSD、PDA的介入治疗与手术治疗临床效果相同;介入组不需输血,术后住院时间短,但治疗费用较昂贵。     

左向右分流型先天性心脏病合并心力衰竭患儿血清IGF-1和IGFBP-3的变化及意义

目的：探讨左向右分流型先天性心脏病（先心病）合并心力衰竭（心衰）患儿血清胰岛素样生长因子-1（IGF-1）和胰岛素样生长因子结合蛋白-3(IGFBP-3)的变化及意义。方法：20例健康儿童（对照组）,20例无心脏基础疾病的心衰患儿（心衰组）,20例无心衰的左向右分流型先心病患儿（先心组）,30例伴心衰的左向右分流型先心病患儿（先心+心衰组）作为研究对象。对不同组别的血清IGF-1及IGFBP-3进行比较;并对先心+心衰组患儿按心功能Ⅱ、Ⅲ、Ⅳ级分为3个亚组,对其血清IGF-1、IGFBP-3及cTnI水平进行比较及相关性分析。结果：先心组血清IGF-1及IGFBP-3水平下降,与对照组比较差异有统计学意义(P<0.01)。先心+心衰组血清IGF-1水平明显下降,与对照组及先心组比较差异有统计学意义(分别P<0.01,P<0.05)。心衰组血清IGF-1及IGFBP-3水平明显增高,与其他各组比较差异有统计学意义(P<0.01)。先心+心衰组患儿按心功能分级比较的各亚组间随心功能下降血清IGF-1水平依次降低(P<0.01),且该组患儿血清IGF-1、IGFBP-3水平与血清cTnI水平呈负相关（分别r=-0.692、-0.530,P<0.05）。结论：血清IGF-1水平可作为左向右分流型先心病病情评估的客观指标及合并心衰的危险因素,这也为该类患儿使用外源性IGF-1治疗心衰提供了临床依据。     

先天性心脏病并心力衰竭患儿血浆肾上腺髓质素和内皮素水平变化的意义

目的探讨左向右分流先天性心脏病(CHD)患儿并充血性心力衰竭(CHF)血浆肾上腺髓质素(ADM)和内皮素(ET-1)变化的意义。方法左向右分流CHD并CHF患儿18例。正常对照组20例,检测CHD患儿心力衰竭期和症状、心衰体征消失的恢复期及对照组儿童血浆ADM和ET-1。结果CHF急性期ADM、ET-1明显高于恢复期和正常对照,恢复期ADM或ET-1仍高于正常对照组(P<0.01或0.02)。结论ADM和ET-1均参与了左向右分流CHD并CHF的病理生理过程,观察其血浆水平变化,可能对判断心衰患者病情有一定意义。     

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.