新生儿换血治疗的血源性铅暴露

目的探讨新生儿换血治疗中使用血库血的铅暴露危险性。方法2006年6～12月对本院新生儿科37例因高胆红素血症进行换血治疗的新生儿进行换血前后血铅水平监测，并对换血使用的53份血库血进行血铅检测。血铅测定采用石墨炉原子吸收光谱法。结果53份血库血平均血铅水平为101．02μg／L，已处于铅中毒水平；而高血铅（≥100μg／L）的血库血占28．5％（15／53份），其中3份血库血样本血铅量≥200μg／L，达Ⅲ级铅中毒水平。高胆红素血症患儿换血后血铅值≥100μg／L的百分率由2．9％升高到19．0％，换血后平均血铅水平明显高于换血前（P〈0．01），血铅水平平均增加26．62μg／L，最高1例增加81．0μg／L。结论血库血铅污染明显，新生儿换血的血源存在血源性铅暴露的危险，应在采血前进行铅筛检，避免新生儿血源性铅中毒。     

新会区儿童铅接触水平与环境铅含量的调查研究

目的 了解本地区儿童血铅水平现状及环境铅含量。方法 按分层随机整群抽样的原则，抽样采集本区0—12岁儿童血样237份及本区医院产科出生新生儿脐带血样107份，采用石墨炉原子吸收光谱法分析血铅含量及本区环境铅浓度。结果：①本区儿童血铅最高值146.2ug/L，最低值21.7ug／L，几何均值为67.3ug／L，≥100ug/L者16例占6.8％；②本区新生儿脐带血血铅最高值116．5肛g儿，最低值18．6肛∥L，几何均值为52.6ug/L，≥100ug/L者5例，占4.7％；③本区环境铅浓度0.12—2.37ug／m3。结论：本区儿童血铅含量及环境铅污染程度，应引起高度重视。     

0～6岁儿童血铅监测模式的临床效果追踪评价

目的对2004年建立的0—6岁儿童血铅监测模式进行临床效果追踪评价。方法在2004年初筛的1817名0—6岁儿童中随机抽取733名儿童进行检测，采用石墨炉原子吸收光谱法进行血铅水平测定，用SPSS 13．0软件分析比较2年血铅水平状况。结果福田区儿童血铅水平经过监测干预后均值为5．23μg／dl，铅中毒率3．27％。结论0—6岁儿童血铅监测模式对降低儿童铅中毒，最大限度地减低铅对儿童的危害，有着巨大的下预作用，把0～6岁血铅监测模式纳入儿童保健工作常规，具有有效性、町行性、可持续件，适宜在全国推广。     

杂症患儿52例血铅水平及影响因素

目的探讨儿科门诊常见杂症的发病与血铅水平的关系及影响因素，以期做到早期预防、早期干预。方法随机选择门诊就诊杂症患儿52例，测其血铅并进行影响因素问卷调查。结果血铅水平〈100μg／L者为36例，占69．2％。影响儿童血铅水平的个人生活习惯主要有4个：服用钙片、进食蔬菜、吸吮手指、吃东西前洗手。结论不足以产生典型临床症状的低水平铅中毒，也可以引起儿童损伤，铅中毒与儿童不良生活习惯有关。     

808例学龄前儿童血铅水平与相关因素分析

目的 了解兰州市学龄前儿童血铅水平及影响因素。方法 采用分层整群抽样法对兰州市９所幼儿园的８０８名７～７ａ儿童进行毛细血管血铅测定，室内外尘土及环境铅测定，对儿童家庭和环境等因素进行问卷调查。结果 儿童血铅平均值为１０１．５８μｇ／Ｌ，标准差为４８．７５μｇ／Ｌ，其中３９．１％≥１００μｇ／Ｌ，工业区儿童血铅最高，其次是市中心区，环境铅与血铅呈明显的正相关（ｒ＝１８．１３ Ｐ〈０．０００１）。住在     

北京市儿童血铅水平及相关因素的调查研究

目的为了解北京市儿童血铅水平及相关因素。方法对本市９所幼儿园２４６名１～６岁儿童进行静脉血铅测定并做危险因素问卷调查，同时测定部分幼儿园的水、空气、食品、灰尘、玩具、幼儿手上灰尘中铅含量。结果本次调查儿童血铅≥１００μｇ／Ｌ者占６８．７％，（１μｇ／Ｌ＝０．００４８３μｍｏｌ／Ｌ），矿区儿童血铅水平显著高于近效区，Ｐ＜０．０１。结论儿童高血铅与空气中铅含量及某些生活行为有密切关系。     

儿童铅中毒102例临床分析

为了解目前铅中毒门诊患儿的常见症状，造成铅暴露的主要原因，摸索铅中毒门诊患儿的病史询问及诊疗方案，对上海新华医院和上海儿童医学中心半年中门诊患儿的资料进行归纳整理。结果发现铅中毒患儿102例，血铅水平几何均数129μg／L，多数患儿表现为多动和注意力不集中，学龄儿童中绝大多数表现为上课时注意力分散、学习困难、成绩差，部分患儿伴腹痛和便秘等消化系统症状，此外还有贫血及血清锌水平降低等；多数患儿能找出铅暴露的危险因素，常见因素有饭前洗手不认真，日钙摄入量低于DRI的50％，常有手—口动作如啃咬指头、铅笔或玩具，父亲吸烟或有被动吸烟史等；39例复诊患儿，经门诊指导(包括高危因素分析、环境干预、行为矫正、营养指导)及住院驱铅后血铅水平均呈不同程度下降，几何均数由148μg/L下降到117μg/L，其中l例极重度铅中毒患儿经住院驱铅治疗后血铅水平显著下降，但停药2周开始反跳，提示这种病例可能需要多个疗程的驱铅治疗，血铅水平才能稳定。     

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儿童铅中毒的处理应该遵循健康教育和环境干预为主,药物驱铅治疗为辅的原则。当儿童血铅经静脉血复查仍高于或等于450μg/L时,应在脱离铅污染源的情况下采用药物进行驱铅治疗。儿童血铅处于250~449μg/L时,部分儿童可以采用药物进行驱铅治疗,近年的研究发现对于这部分儿童采用驱铅治疗后智力发育并没有明显改善,因此美国儿科学会最近提出中度儿童铅中毒驱铅治疗没有意义[1]。根据文献报道,在我国目前除铅污染区外,绝大多数普通城市及乡村儿童的血铅高于或等于100μg/L的比例为10%~30%,高于200μg/L的比例低于1%~2%,较10年前有较明显的下降…     

儿童铅中毒诊治进展

目前铅污染已逐渐成为突出的问题，2004年中国部分儿童铅中毒防治项目组调查了15个城市共17141例0～6岁的儿童，结果发现平均儿童血铅水平为53μg／L，儿童铅中毒率为10．45％。儿童铅中毒主要来自公害性中毒、生活性中毒和母源性中毒。环境中的铅主要通过呼吸道和消化道吸收而引起人体中毒。铅以血液、软组织和骨骼的三室模式分布于体内，并且在亚临床水平已使机体产生多种生理变化。     

儿童铅中毒诊治进展

理想的血铅水平应是零,但由于环境污染体内血铅常 常并不为零.美国国家疾病控制中心(CDC)1991年将血 铅质量浓度≥100μg/L定义为儿童铅中毒(childhood lead poisoning).近来开始重视血铅≥100μg/L是一个群体流 行病学的概念,而并非临床医学上的诊断标准.2006年我 国调整了儿童铅中毒的诊断标准,提出了高铅血症概念,制 定了铅中毒的干预和治疗方案(试行)[1].     

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.     

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.     

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.