原发性高血压患儿硫化氢与同型半胱氨酸的代谢关系及其病理生理意义

目的观察原发性高血压患儿血浆中硫化氢(H2S)与同型半胱氨酸(Hcy)代谢间的关系,探讨其在高血压发病中的病理生理学意义。 方法北京大学第一医院儿科等单位于2005 02随机选取体检时高血压儿童(高血压组)25例,血压正常儿童30例(对照组),对其血压进行测量及体格检查,对家族史进行调查。利用荧光偏振免疫法检测血浆Hcy浓度,应用敏感硫电极法测定血浆H2S浓度。 结果高血压组儿童血浆Hcy为(12.68±9.69)μmol/L,对照组儿童血浆Hcy为(6.62±4.79)μmol/L,差别有显著性意义(P<0.01);高血压组血浆H2S为(51.93±6.01)μmol/L,对照组儿童血浆H2S为(65.70±5.50)μmol/L,差别有显著性意义(P<0.01)。将血浆Hcy与H2S浓度做直线相关分析,结果呈负相关(r=-0.379,P<0.05)。将收缩压与H2S/Hcy比值作直线相关分析,结果二者呈明显负相关(r=-0.687,P<0.05)。 结论原发性高血压儿童存在硫化氢与H2S代谢失衡。     

肺动脉高压新生儿血浆硫化氢变化的意义

目的 探讨肺动脉高压新生儿血浆硫化氢(H2S)的变化及其与肺动脉高压(PH)相关性,为临床有效治疗新生儿PH提供新的思路和理论依据.方法 随机选取2005年3月-2006年3月本院ICU病房住院PH新生儿16例,选取同期住院非PH新生儿16例作为对照组.对全部研究对象均行超声心动图检查,依据简化柏努力方程AP=4V2max计算出压力阶差,估测三尖瓣二侧压力,间接判断其肺动脉压,对二组新生儿于出生3～10 d清晨8:00取静脉血4 mL,迅速分离其血浆,采用敏感硫电极法测定其血浆H2S水平,应用氨基酸分析仪测定其同型半胱氨酸水平.应用SPSS 10.0软件进行t检验.结果 PH新生儿肺动脉收缩压为4.27～9.73(6.49.±1.79)kPa;对照组肺动脉收缩压正常.肺动脉高压组血浆同型半胱氨酸及H:s水平明显高于对照组[(11.94 ±6.65)μmol/Lvs(6.43 ±2.08)μmol/L,t=2.630 P=0.016;(26.99 ±1.33)μmol/Lvs(24.92 ±1.36)μmol/L,t=4.373 P=0].结论 PH新生儿血浆同型半胱氨酸和H2S明显升高,内源性H2S上调可能参与肺动脉压力的调节.H2S可能通过扩张肺动脉降低肺动脉压力而对PH具有保护作用.     

先天性心脏病合并肺动脉高压患儿血浆内皮素、降钙素基因相关肽的临床研究

目的 探讨先天性心脏病(congenital heart disease,CHD)患儿血浆内皮素(endothelin-1,ET-1)、降钙素基因相关肽(calcitonin gene-related peptide,CGRP)浓度的变化以及与肺动脉压力间的关系.方法 选择健康体检儿30例为对照组,无肺动脉高压(pulmonary hypertension,PH)的CHD 30例为无PH组,CHD合并轻度PH患儿20例为轻度PH组,CHD合并中重度肺动脉高压20例为中重度PH组.采用放射免疫法测定血浆ET-1、CGRP浓度.结果 CHD患儿血浆ET-1明显高于对照组(P<0.01),且轻度、中重度PH组明显高于无PH组及对照组(P<0.01);CHD无PH组CGRP浓度低于对照组(P<0.01),且轻度、中重度PH组明显低于无PH组及对照组(P<0.01);血浆ET-1与肺动脉压力呈正相关(r=0.977,P<0.01),CGRP与肺动脉压力呈负相关(r=-0.81,P<0.05);血浆ET-1与血浆CGRP水平呈显著负相关(r=0.843,P<0.01).结论 CHD患儿ET-1升高、血浆CGR降低,两者的变化与肺动脉压有相关性,可能参与了肺血管的重构及PH的形成.     

先天性心脏病并肺动脉高压患儿血浆一氧化氮和硫化氢的变化

目的研究先天性心脏病(CHD)并肺动脉高压(PH)患者血浆一氧化氮(NO)和硫化氢(H2S)的变化及其与PH相关性,探论PH的形成机制,为临床有效治疗PH提供新的思路和理论依据。方法对全部研究对象行彩色超声心动图检查,明确CHD类型并测定肺动脉收缩压。按彩色超声心动图结果分为4组。正常儿童25例,CHD 75例,其中无PH 25例,轻度PH 25例,中、重度肺PH 25例。取CHD组术前静脉血4 mL,迅速分离血浆,采用分光光度法测定血浆NO水平,采用敏感硫电极法测定H2S水平。结果CHD患儿血浆NO水平明显高于对照组,但血浆NO水平增加到一定程度后不再随肺动脉收缩压增加而增加,肺动脉收缩压与血浆NO水平无相关性。CHD并中重度PH组血浆H2S水平明显低于CHD并轻度CHD组,CHD并轻度PH组血浆H2S水平明显低于对照组,肺动脉收缩压与血浆H2S水平呈负相关。结论CHD致PH形成时NO水平升高,代偿性内源性NO上调可能对缓解PH起一定作用。CHD致PH形成时H2S明显降低,内源性H2S下调可能在PH形成中起重要的作用。     

手足口病患儿血清硫化氢及白细胞介素-6水平改变的研究

目的：研究手足口病(hand,foot and mouth disease,HFMD)患儿血清硫化氢(hydrogen sulfide,H2 S)与白细胞介素(interleukin,IL)-6水平改变的意义。方法选择在我院住院治疗的92例HFMD 患儿为研究对象,根据病情,选择其中48例为重症 HFMD 组,44例为普通 HFMD 组,选择同期健康儿童46例为健康对照组。分别检测其血清 H2 S 及 IL-6水平。结果急性期：重症 HFMD 组与普通 HFMD 组血清 H2 S [(21.72±7.52)μmol /L、(31.86±8.41)μmol /L]水平均明显降低,IL-6[(131.33±17.82)ng /L、(95.48±15.07)ng /L]水平明显升高,与健康对照组[H2 S (55.76±7.80)μmol /L,IL-6(61.31±13.43)ng /L]比较差异有统计学意义(P ﹤0.01);重症 HFMD 组与普通HFMD 组比较差异有统计学意义(P ﹤0.01)。恢复期：重症 HFMD 组血清 H2 S[(34.54±13.21)μmol /L]水平仍低于健康对照组,IL-6[(92.73±15.25)ng /L]水平仍高于健康对照组,差异有统计学意义(P ﹤0.01)。相关性分析显示：重症 HFMD 组与普通 HFMD 组血清 H2 S 与 IL-6水平之间均呈显著负相关(r ＝-0.31,P ﹤0.01;r ＝-0.45,P ﹤0.01)。结论 HFMD 患儿血清 H2 S 和 IL-6水平发生了改变,并且可作为重症 HFMD 患儿早期诊断的指标之一。     

先天性心脏病患儿血清甲状旁腺素水平观察

目的探讨先天性心脏病(CHD)患儿甲状旁腺功能。方法用放射免疫法(IRMA)测定18例学龄前期CHD患儿血清甲状旁腺素(parathyrin,PTH)水平,并测定其血清Ca2 、Mg2 浓度;17例门诊体检的健康儿童作为对照。结果CHD患儿血清PTH水平(14.87±6.02ng/L)较对照组(19.68±5.76ng/L)明显降低,两者比较差异有显著性(P<0.05),CHD组中有8例(44.4%)患儿血清PTH<10ng/L,而对照组均高于10ng/L;CHD患儿血清Ca2 和Mg2 较对照组略低,但无统计学意义(P>0.05)。结论CHD患儿甲状旁腺素分泌不足,存在亚临床甲状旁腺功能低下。     

硫化氢及血红素氧合酶-1在先天性心脏病肺动脉高压中的作用探讨

目的 探讨先天性心脏病(CHD)合并肺动脉高压(PH)患儿手术前后硫化氢(H2S)及血红素氧合酶-1(HO-1)变化的临床意义.方法 将48例CHD患儿按肺动脉收缩压(PASP)分为三组:无PH组(PASP ＜ 30 mmHg＝15例,轻度PH组(PASP 30 ～ 49 mmHg)15例,中、重度PH组(PASP ≥ 50 mmHg)18例.测定三组手术前、手术后1 h及手术后24 h血浆H2S及血清HO-1含量,并比较手术前后的变化;分析两者与PASP间的相互关系.结果 各组患儿术后H2S含量呈上升趋势,但只有术后24 h与术前比较差异有统计学意义(P ＜ 0.05).三组患儿手术前后HO-1活性差异无统计学意义(P ＞ 0.05).三组患儿H2S含量与PASP呈负相关(r ＝ -0.730 ～ -0.458,P均＜ 0.01).三组患儿HO-1活性与PASP无相关性(术前r ＝ -0.031 ～ 0.230,P均＞ 0.05).结论 H2S在PH形成和肺血管重建中发挥重要作用.     

过敏性紫癜患儿血浆硫化氢、一氧化氮变化的意义

目的 本研究旨在探讨气体信号分子硫化氢(H2S)和一氧化氮(NO)在过敏性紫癜(HSP)患儿发病中的变化及意义.方法 选择2007年1-6月在本院就诊的首次确诊为HSP的住院患儿25例,北京大学第一医院儿科体检健康者19例为健康对照组,分别应用敏感硫电极法及硝酸还原酶法测定其血浆中H2S、NO水平及一氧化氮合酶(NOS)活性,比较HSP患儿与对照组之间的差异及HSP患儿各型之间的差异.结果 HSP患儿血浆H2S水平较健康对照组明显升高[(70.93±14.63)umol/L vs(58.17±7.40)umol/L P<0.01];HSP患者NO水平及NOS活性与健康对照组比较明显升高[(78.32±8.68)umol/Lvs(47.28±6.72)umol/L,(36140±5300)U/L vs(25 640±3 030)U/L P.<0.01].HSP单纯型组血浆H2S、NO水平及NOS活性均较混合型组明显升高,有统计学差异[(74.29±8.52)umol/L vs(62.93±9.14)umol/L,(81.16±9.25)umol/L 138(62.33±11.89)umol/L,(52 270±8 140)U/L vs(31 970±5 990)U/L Pa<0.05].HSP组中心脏受累组与非心脏受累组比较.血浆NO、H2S水平及NOS活性比较无统计学差异[(67.81±7.09) umol/L vs(64.19±8.77)umol/L,(69.24±8.68) umol/L vs(63.20±11.04) umol/L,(40 420±5 950)U/L vs(36 550±5 510)U/L Pa0.05].结论 气体信号分子H2S、NO水平在HSP发病早期显著升高,且在单纯型息儿升高明显.提示气体信号分子H2S、NO可能在HSP发病中发挥重要的保护作用.     

先天性心脏病肺动脉高压患儿左室舒张功能与血浆脑利钠水平的相关性研究

目的：探讨先天性心脏病 (CHD)合并肺动脉高压 (PAH) 患儿脑利钠肽（BNP）水平及与左室舒张功能的关系。方法：对95例CHD继发有PAH的患儿和42例无PHA的CHD患儿（对照组）的多普勒超声心动图资料与其血浆BNP水平进行对比分析。结果：与对照组相比, PAH组的左室舒张末内径（LVDd ）、右室舒张末内径（RVDd） 和肺动脉内径（PAd）明显增大(P<0.05),三尖瓣返流（VTR）速度增快及肺动脉收缩压（PASP）升高(P<0.05)。与对照组比较,PASP组患儿二尖瓣口多普勒血流频谱A峰流速（AV）、A峰流速积分（AVI）和E峰流速积分（EVI）及AV/EV和AVI/EVI比均逐渐明显增大(P<0.01);左室等容舒张时间明显延长(LIVRT)(P<0.05)。血浆BNP水平随着PASP增高而升高,与对照组相比差异有显著性（P<0.01）。PAH组先心病患儿其肺动脉压与二尖瓣口血流频谱AV/EV比值呈正相关（P<0.01）,二尖瓣口血流参数与血浆BNP水平亦呈正相关（P<0.01）。结论：CHD合并PAH患儿左室舒张功能与血浆BNP水平呈正相关;BNP在PAH引起左室舒张功能障碍的发生发展过程中发挥了重要作用。［中国当代儿科杂志,2010,12（1）：13-16］     

一氧化氮治疗小儿急性呼吸衰竭和肺动脉高压

用我科自行研制的NO吸入装置对5例急性肺损伤伴呼吸衰竭(ALI ARF)和5例先天性心脏病伴肺动脉高压(CHD PH)患儿进行NO吸入治疗后,气体交换和血流动力学参数变化的观察,结果显示:ALI ARF组PaO_2/FiO_2上升48±17(54±15%)(T=4.52,P<0.01),氧合指数(OI)下降9±3(26±6%)(T=4.63,P<0.01),3例ARDS的 V_D/V_t下降5±1%(T=5.00,P<0.05);CHD PH组平均肺动脉压(mPAP)下降17±10%(T=5.345,P<0.01),肺血管阻力下降18±11%(T=5.432,P<0.01)。结论:NO吸入可改善ALI ARF患儿的动脉氧合,降低CHD PH患儿的肺动脉高压。     

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.     

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.     

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.