一氧化氮合酶mRNA在缺氧性肺动脉高压大鼠肺动脉的表达

目的探讨一氧化氮体系在缺氧性肺动脉高压形成机制中的作用。方法采用地高辛精标记的一氧化氮合酶（ＮＯＳ）ｃＲＮＡ探针对缺氧组大鼠（６只）及对照组大鼠（７只）进行原位杂交。结果缺氧２周后的大鼠肺动脉收缩压（３．８±０．７ｋＰａ）（２８±５ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）、肺动脉平均压（２．８±０．６ｋＰａ）及肺动脉舒张压（１．４±０．４ｋＰａ）与对照组（２．９±０．５ｋＰａ，１．９±０．５ｋＰａ及０．９±０．５ｋＰａ）相比均显著升高。缺氧组大鼠肺动脉内皮细胞中ＮＯＳｍＲＮＡ表达信号为弱阳性（３只）及阴性（３只），平滑肌细胞中表达信号均为阴性；对照组大鼠肺动脉内皮细胞中ＮＯＳｍＲＮＡ表达信号为阳性（７只），平滑肌细胞中表达信号均为阴性。ＮＯＳｍＲＮＡ的表达强度与大鼠肺动脉收缩压、肺动脉平均压及肺动脉舒张压分别呈负相关（ｒｓ＝－０．６７３、－０．５９６及－０．６２１，Ｐ均＜０．０５）。结论缺氧时肺动脉内皮细胞ＮＯＳｍＲＮＡ表达的改变可能参与慢性缺氧性肺动脉高压的形成。     

小儿肺动脉高压的研究进展

小儿肺动脉高压的研究进展杜军保李树政当肺动脉收缩压（ｐｕｌｍｏｎａｒｙａｒｔｅｒｉａｌｓｙｓｔｏｌｉｃｐｒｅｓ－ｓｕｒｅ，ＰＡＳＰ）超过４．００ｋＰａ（３０ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）或肺动脉平均压（ｐｕｌｍｏｎａｒｙａｒｔｅｒｉａｌｍｅａｎ...     

一氧化氮吸入治疗小儿急性呼吸衰竭

为观察一氧化氮（ＮＯ）治疗小儿急性呼吸衰竭的疗效，应用我院自行研制的ＮＯ吸入装置，对１５例急性呼吸窘迫综合征（ＡＲＤＳ）和急性呼吸衰竭（简称急性呼衰）患儿进行ＮＯ吸入治疗。结果：７例有效，ＮＯ吸入前后比较氧分压与吸入氧浓度比值上升４．１±２．３ｋＰａ（３０．５±１７ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）（ｔ＝４．５２，Ｐ＜０．０５），氧合指数降低９±３（ｔ＝４．６３，Ｐ＜０．０５）。对２例肺动脉导管压力监测显示，肺动脉压和肺血管阻力明显下降，体动脉压和心率无显著性变化。结论：ＮＯ吸入疗法对部分急性呼衰患儿有效，宜在急性低氧性呼衰、心功能未受严重损害时应用。     

儿童支气管哮喘肺动脉压力与右心舒张功能的变化

目的　探讨儿童支气管哮喘（哮喘） 患儿肺动脉压力与右心舒张功能的改变。方法　对２０ 例对照组,１４ 例轻、中型哮喘及１７ 例重型哮喘患儿以多普勒超声心动图测定指标：心率校正肺动脉血流加速时间（ＡＴｃ）;右室射血前期时间（ＲＰＥＰ） ;舒张早期快速充盈峰值流速（Ｅ）;舒张晚期快速充盈峰值流速（Ａ） ,估测肺动脉压及右心舒张功能,同时检测血气分析指标。结果　①儿童哮喘存在肺动脉高压,重型哮喘组（ＲＰＥＰ／ＡＴ１ ．２６ ±０．１９） 高于轻、中型组（１．１２ ±０．１５） 及对照组（０．８５±０．１４） Ｐ＜ ０ ．０１ 。②对照组（Ｅ／Ａ１．４１±０．２９） 与患儿右心舒张功能指标有明显差异Ｐ＜ ０．０１;重型哮喘患儿存在低氧血症（ＰａＯ２ ７ ．０２ ±０ ．８０ ｋＰａ） 。结论　哮喘患儿存在肺动脉高压和右心舒张功能下降,且随着病情进展,变化显著。     

肺表面活性物质治疗新生儿胎粪吸入综合征的临床研究

目的探讨肺表面活性物质（ＰＳ）治疗新生儿胎粪吸入综合征（ＭＡＳ）的有效性及临床价值。方法采用气管内滴入ＰＳ治疗８例ＭＡＳ患儿，其中６例接受ＰＳ２剂，２例接受ＰＳ３剂。结果给予首剂ＰＳ后１０分钟患儿青紫迅速消失，皮肤转红润，经皮测定血氧饱和度（ＴｃＳａＯ２）升高。３０分钟后患儿低氧血症迅速改善，动脉血氧分压、动脉血氧分压与吸入氧浓度比值、动脉肺泡血氧分压比值、呼吸机有效指数较治疗前显著增高，分别由原来的５２８±０９８ｋＰａ、８６６±３５２ｋＰａ、０１２±００６ｋＰａ及０１４±００６ｍｌ·ｋＰａ－１·ｋｇ－１增加到８９１±１４３ｋＰａ、１６８１±４１８ｋＰａ、０２１±００５ｋＰａ及０２６±００７ｍｌ·ｋＰａ－１·ｋｇ－１；而吸入氧浓度及平均气道压逐渐降低，由原来的０６８±０１９ｋＰａ及２２０±０４２ｋＰａ降低到０５３±００８ｋＰａ及１９３±０４８ｋＰａ。重复应用ＰＳ后亦有相似效果。结论ＰＳ能有效地改善ＭＡＳ患儿肺顺应性及氧合功能。重复应用ＰＳ可巩固和加强疗效。     

室间隔缺损合并肺动脉高压肺血管床功能状态评估指标的研究

目的探讨室间隔缺损合并肺动脉高压肺血管床功能状态的评估指标。方法对室间隔缺损合并重度肺动脉高压的患儿，于心导管术中应用酚妥拉明，将轻度全肺循环阻力（ＴＰＲ）增加的２７例与重度全肺循环阻力增加的１２例患儿的试验结果进行比较。结果两组患儿的单一肺动脉压降幅（Ｐｐ降幅）分别为２．３ｋＰａ（１７ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）和２．２ｋＰａ，Ｐ＞０．０５，差异无显著意义；而肺动脉压降幅（Ｐｐ降幅）／体循环压降幅（Ｐｓ降幅）的比值，试验前后肺动脉血氧饱和度变化值这二项指标的差异有非常显著意义（Ｐ＜０．０１）。Ｐｐ降幅／Ｐｓ降幅比值与全肺循环阻力的相关性良好［ｒ＝－０．８９９），Ｙ（ＴＰＲ）＝２６６８－１８９２Ｘ（Ｐｐ降幅／Ｐｓ降幅）］。结论用酚妥拉明作扩血管降压试验时，单一的肺动脉压降幅不能完全反映肺血管床的功能状态；而Ｐｐ降幅／Ｐｓ降幅比值，试验前后肺动脉血氧饱和度变化值这二项指标对室间隔缺损合并肺动脉高压患儿的肺血管床功能状态的评估，对手术适应证的选择具有一定指导意义。     

酚妥拉明治疗小儿肺动脉高压症的实验及临床研究

为观察酚妥拉明对肺动脉压的作用，选用１０只家兔，置入右心导管，投入不同剂量酚妥拉明，用换能器监测不同剂量投药前后肺动脉压力的变化。并选择１０例顽固性心力衰竭（简称心衰）合并肺动脉高压患儿，加用酚妥拉明静脉滴注１个疗程，以脉冲多普勒超声监测其治疗效果。结果显示，肺动脉压力（ＡＴ）明显降低，主肺动脉血流加速时间、二尖瓣口舒张早、晚期最大血流速度（ＥＶｍａｘ、ＡＶｍａｘ），左室壁应力（ＥＳδ、ＥＤδ、ＭＥＡＮδ）等指标用药后明显改善。临床观察，其中９例患儿心衰明显减轻。证实酚妥拉明为治疗顽固性心衰有效的血管活性药物。     

婴儿完全性大动脉转位外科根治术前血液动力学的内科调整研究

为探讨球囊房隔造口术（ＢＡＳ）及前列腺素Ｅ１（ＰＧＥ１）对婴儿完全性大动脉转位（ＴＧＡ）外科根治术前血液动力学的调整作用，对３２例年龄２～５６天，平均２６天的ＴＧＡ患儿行ＢＡＳ，对其中１１例于ＢＡＳ前给予ＰＧＥ１，然后进行血液动力学观察。结果：ＢＡＳ后动脉血氧饱和度（ＳａＯ２）由０．５３±０．１４上升至０．７４±０．１１。３０例患儿左右心房压差均＜０．２７ｋＰａ（１ｋＰａ＝７．５ｍｍＨｇ）。单纯ＴＧＡ术后２４个月内左室压力＜５．３０ｋＰａ。ＰＧＥ１应用后ＳａＯ２由术前０．６０±０．１９上升至０．８６±０．０５。心导管检查测得左右室压力比为０．８６。提示，ＢＡＳ和ＰＧＥ１的应用可缓解新生儿ＴＧＡ低氧血症及维持其左右心室良好的压力比值，从而为ＴＧＡ解剖转位术作准备     

取样容积位置对检测肺动脉血流时间间期的影响

在２９例左向右分流的先天性心脏病患儿中,将取样容积（ＳＶ）置于肺动脉中不同位置,记录肺动脉血流频谱,测量心室射血前期（ＰＥＰ）射血时间（ＥＴ）,加速时间（ＡＴ）,加速度（ＡＣＣ）,Ｖ（平均流速）Ｆ（Ｆ＝ＡＣＣ×ＰＥＰ／ＥＴ）等参数,根据肺动脉／主动脉血流时间间期比估测肺动脉压（ＰＡＰ）,结果显示,不同ＳＶ位置的ＰＥＰ,ＰＶ,ＡＴ,ＡＣＣ,ＥＴ,ＰＥＰ／ＡＴ,Ｖ,Ｆ间存在显性差异（Ｐ〈０．０５）,     

婴幼儿肺炎血浆内皮素与肺动脉高压相关研究

婴幼儿肺炎血浆内皮素与肺动脉高压相关研究刘雪芹，李树政，吴希如对４８例（３８例轻症、１０例重症）婴幼儿肺炎患儿，以多普勒超声心动测定的ＲＰＥＰｌＡＴ及ＡＴｃ值为指标估测肺动脉压，应用放射免疫分析方法测定血浆免疫活性内皮素（ｉｒＥＴ）水平。结果表明：肺...     

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.