7例新生儿肺透明膜病临床病理分析

本文从1977年7月至1983年10月尸检因各种疾病死亡的新生儿共139例,死于本病者7例。其中3例是早产儿;4例足月儿,但出生低体重,低于第10百分位.此病大多数是早产儿、低体重儿,常易误诊为窒息、早产儿生活能力不足及新生儿吸入性肺炎等。在出生后12小时尚未发病者,往往不是本病,而早产儿或低体重儿在出生12小时内出现三凹征和阵发性呼吸困难应首先考虑本病。治疗上给氧方法为持续正压给氧(CPAP)或呼吸终束给氧(PEEP)。并提出采用高频通气(HFV)。早期结扎未闭的动脉导管。     

早产儿动脉导管未闭发生与液体入量的关系探讨

动脉导管未闭 (PDA)是早产儿常见并发症 ,也是导致早产儿充血性心力衰竭、呼吸衰竭的常见原因之一。目前有关PDA发病机制、病理生理等方面学术研究较多〔1-2〕,而临床针对动脉导管未闭发生与早产儿生后早期 (<1周 )液体治疗二者关系报道较少。本文旨在探讨早产儿生后早期每日液体入量与PDA发生之间的关系 ,为临床液体治疗提供一定参考。1　对象与方法1 1　对象　 2 0 0 0年 8月— 2 0 0 2年 8月我院新生儿重症监护病房 (NICU)收治的日龄 1周内的早产儿为研究对象 ,总共 2 18例 ,男 142例 ,女 76例。低出生体重儿 (LWBI :15 0 0 g～ 2…     

氨基糖苷类药物的使用与需手术关闭早产儿动脉导管未闭的比例较高有关

目的动物实验表明氨基糖苷类药物具有剂量依赖性舒张动脉导管的作用。研究假设氨基糖苷类抗生素治疗可能与需手术关闭早产儿动脉导管未闭(PDA)的比例较高有关。方法分析62个德国新生儿重症监护病房(NICU)中收治的早产儿(出生体重<1000g或胎龄<29周)。患儿按氨基糖苷类作为一线抗生素使用情况分组。     

动脉导管未闭的药物治疗进展

正常足月儿生后24～48 h动脉导管已呈功能性关闭,而早产儿的动脉导管常不能关闭,或功能性关闭后又重新开放.有呼吸窘迫综合征的极低出生体重儿,生后第3天动脉导管未闭(PDA)的发生率约为40%[1].PDA的临床结果取决于左向右分流的程度,可加重呼吸窘迫、促发充血性心力衰竭、支气管肺发育不良、肾脏低灌注及脑缺血、继发肠缺血.70%胎龄小于28周的早产儿需要药物或手术关闭动脉导管[2].手术结扎动脉导管有血压波动、感染、乳糜胸、喉神经麻痹,甚至死亡的危险.故在大多数新生儿重症监护病房中,药物治疗PDA是首选.     

早产儿动脉导管未闭发病率及消炎痛治疗10年总结

目的探讨早产儿动脉导管未闭的发病率及消炎痛的治疗效果。方法根据早产儿及患动脉导管未闭例数计算早产儿动脉导管未闭的发病率。观察早产儿动脉导管未闭发病率与早产儿体重及胎龄的关系，探讨消炎痛对早产儿动脉导管未闭患儿的治疗效果。结果早产儿动脉导管未闭的发病率为５．０６％（６０／１１８５），出生体重≤１５００克者，其发病率为６．５４％（１０／１５５），～２０００克者为５．２５％（２７／５１４），～２５００克者为４．８８％（１９／３８９），≥２５００克者为３．１５％（４／１２７），各组发病率有显著性差异（ｘ２＝６４．８５，Ｐ＜０．０１）。早产儿动脉导管未闻发病率与胎龄无关（ｘ２＝０．３４４，Ｐ＞０．０５）。消炎痛治疗４１例，痊愈３１例（７５．８１％）。≤１５００克、２０００克、２５００克及≥２５００克等组消炎病治愈率分别１００％、９０．９１％、５３．８４％和０（ｘ２＝１３．６２，Ｐ＜０．０５），治愈率与胎龄无关。结论早产儿动脉导管未闻的发病率及消炎痛对其治愈率随出生体重增加而降低，两者与胎龄无关。     

低体重早产儿动脉导管未闭的早期手术干预

目的探讨低体重早产儿动脉导管未闭(PDA)的早期手术干预方法及疗效。方法2012年1月至2015年12月本院收治33例低体重早产儿PDA,均在NICU协助下进行手术治疗。根据治疗方案分为药物治疗失败后采用Prolene缝线结扎未闭动脉导管组(G1组)和直接采用Prolene缝线结扎未闭动脉导管组(G2组)两组,比较两组病史、临床资料、病死率及主要并发症情况。结果两组病史因素及临床资料比较,差异无统计学意义(P0.05)。G2组无一例死亡,G1组病死率为13.3%,差异有统计学意义(χ~2=6.28,P0.05),G2组并发症的发生率为11.1%,G1组并发症的发生率为33.3%,差异有统计学意义(χ~2=11.92,P0.05)。结论早期在NICU与外科协作下选用Prolene缝线结扎未闭动脉导管是PDA安全有效的治疗方式。     

早产儿动脉导管未闭诊治进展

动脉导管未闭(PDA)是影响早产儿死亡率和致残率的重要因素之一.胎龄和体重越小,发病率越高 [1].早产儿动脉导管管壁薄,缺乏肌肉组织,对氧等刺激收缩的因素敏感性低,对前列腺素等松弛平滑肌的因素敏感性高.     

早产儿动脉导管未闭的发病机制、高危因素及并发症

早产儿持续性动脉导管未闭(patent ductus arteriosus,PDA)可引起严重的临床后果,特别是体重小于1000g的超低出生体重儿,在新生儿呼吸窘迫综合征(RDS)恢复期间,随着肺通气和氧合的提高、肺血管阻力下降,体循环阻力增大,使流经动脉     

早产儿动脉导管未闭高危因素分析

目的探讨早产儿动脉导管未闭(PDA)的危险因素。方法对2010年7月至2011年7月在我院新生儿监护病房住院的早产儿进行回顾性研究。其中出生48h后进行超声检查诊断PDA的患儿为病例组,按2.5∶1的比例从动脉导管关闭的早产儿中随机抽取对照组。将两组早产儿的产科合并症、宫内情况、早产儿相关疾病、生后干预及相关检验指标进行对照研究。单因素分析中有统计学意义的因素进行Logistic回归分析。结果共纳入病例组96例,对照组250例。单因素分析显示胎龄、出生体重、母亲产前用药情况、早产儿相关疾病及生后干预与PDA相关。趋势卡方检验等级效应分析显示,胎龄越小,出生体重越低,PDA发生率越高,Logistic回归分析提示感染和暂时性甲状腺功能低下是PDA的独立危险因素,胎龄为独立保护因素(OR值分别为2.183、2.935和0.806),P均<0.05。结论本研究中,早产儿PDA与小胎龄、低出生体重、合并感染及暂时性甲状腺功能低下密切相关。     

婴儿及新生儿动脉导管未闭急诊手术治疗26例分析

目的探讨婴儿期重症动脉导管未闭的急诊手术治疗经验。方法回顾性分析2007年9月至2012年8月我们收治的26例婴儿及新生儿动脉导管未闭患儿临床资料,其中新生儿12例,包括早产儿10例,足月新生儿2例,术前需呼吸机辅助通气者12例;婴儿14例,术前需呼吸机辅助通气6例。均行急诊动脉导管未闭手术治疗,15例经左腋下横切口,10例经左腋下直切口进行动脉导管结扎术。结果26例均顺利完成手术,1例术中出现动脉导管破裂,予直接缝合;术后复查无残余分流,无并发症。26例随访2～72个月,生长发育良好,心功能正常,无导管再通及假性动脉瘤形成。结论婴儿及新生儿粗大动脉导管未闭需及时手术治疗,以阻断异常分流,减少肺血,纠正心衰,及时关闭动脉导管,能有效缩短重症监护时间,降低死亡率。．     

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.     

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.     

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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.     

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.     

alpha -SMOOTH MUSCLE ACTIN DISTRIBUTION IN THE PULMONARY VASCULATURE COMPARING HYPOPLASTIC AND NORMAL FETAL LUNGS

We investigated the intra-acinar pulmonary vascular muscularization in the developing human fetal lung between the 17th and 24th gestational weeks, that is, during the canalicular phase of lung development. Fifteen hypoplastic and 25 normal developed lungs were included in this study using monoclonal alpha -smooth muscle (sm) actin antibodies for smooth muscle detection. Computer-aided image analysis was performed for morphometrical measurements and statistical evaluation. Alphasm-actin-immunoreactive intra-acinar vessels down to a luminal diameter of less than 10 mu m were detected in hypoplastic as well as in normally developed lungs. Crucial differences presented as follows: significantly higher density of intra-acinar vessels, especially due to alpha -sm-actin-negative vessels less than 30 mu m in luminal diameter, in the control group; significantly higher alpha -sm-actin immunoreactivity per section unit as well as per vessel in the hypoplastic lung group. As suggested by others, alpha-sm-actin-positive cells of the intra-acinar vessel wall in the developing human lung were demonstrated to be smooth muscle cells, their immediate precursors, and pericytes. We conclude that the increased alpha -sm-actin immunoreactivity represents muscularization of the vessel wall in functional terms and may be regarded as one structural cause among others for the establishment of persistent fetal circulation in hypoplastic lungs.     

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.