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。     

肺动脉高压时肺小动脉弹性蛋白的表达变化

研究脉动脉压力对肺小动脉璧原弹性蛋白表达与颁的影响以帮助阐明先天性心脏病（先心病）肺动脉高压（肺高压）肺血管重建的发病机制。将１５例患儿分为对照组（组１）、先心病高肺血流组（组２）及先心病肺高压组（组３）。用免疫组化技术研究弹性蛋白在肺小动脉璧的含量与分布以了解弹性蛋白的合成情况;用原位杂交技术在转录水平研究肺小动脉间性蛋白ｍＲＮＡ的表达分布。结果：各组患儿间年龄比较无差异。组２、组３间肺动脉压力     

内皮素在左向右分流型先天性心脏病肺动脉高压中作用的研究

为探讨内皮素在左向右分流型先天性心脏病（简称先心病）肺动脉高压（简称肺高压）中的作用，采用放射免疫法测定５２例肺高压和１１例非肺高压先心病患儿腔静脉和肺动脉血浆内皮素（ｉｒＥＴ－１）水平，并对其中１０例肺高压患儿进行肺活检免疫细胞化学染色。结果，肺高压患儿肺动脉和腔静脉血浆ｉｒＥＴ－１水平均随肺动脉压力升高而增加，并高于非肺高压患儿（Ｆ＝２．９５；３．３９，Ｐ＜０．０５）。肺高压患儿血浆ｉｒＥＴ－１与肺动脉压力及阻力成正相关（ｒ＝０．６５；０．５８，Ｐ＜０．０１）。肺高压患儿肌性和弹性肺小动脉ｉｒＥＴ－１染色明显加深。研究提示，左向右分流型先心病患儿内皮素产生的增加可导致肺高压患儿肺血管的中膜肥厚和内膜增生，促进了肺高压的发展。     

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。     

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。     

一氧化氮对缺氧和急性肺损伤犬的血液动力学与气体交换的影响

应用化学发光法监测氮氧化物浓度，观察１０只缺氧和急性肺损伤犬在吸入不同浓度一氧化氮（ＮＯ）时血液动力学和气体交换功能的变化。结果显示，５～５０ＰＰＭＮＯ均可降低缺氧犬肺动脉压２５％±３％（Ｐ＜０．０１），降低肺血管阻力３７％±５％（Ｐ＜０．０１），并使急性肺损伤犬的动脉血氧分压／吸入氧浓度（ＰａＯ＿２／ＦｉＯ＿２）比值上升３３．４±２．３（Ｐ＜０．０５），肺内动静脉分流量与总血流量（Ｑ＿ｓ／Ｑ＿Ｔ）比值下降５％±２％（Ｐ＜０．０５）。提示，低浓度ＮＯ（５～２０ＰＰＭ）即可有效降低缺氧性和急性肺损伤犬肺动脉高压并改善其动脉氧合功能。     

小儿扩张型心肌病肺动脉压的多普勒超声估测及其临床意义

目的估测扩张型心肌病肺动脉压并探讨其临床意义。方法用多普勒超声检查２６例扩张型心肌病患儿及２６名配对健康儿。肺动脉压用测三尖瓣最大返流速度及肺动脉血流加速时间（ＡＴ）两种方法估测。结果２６例患儿中２１例有三尖瓣返流，肺动脉收缩压（ＰＡＳＰ）３．２～１０．４ｋＰａ（６．０±１．９ｋＰａ）（２４～７５ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）。其中１８例（８６％）＞４ｋＰａ；对照组１４例有可测三尖瓣返流，ＰＡＳＰ均＜４ｋＰａ。患儿组ＡＴ缩短，ＡＴ与右室射血时间比值降低，也提示肺动脉压增高。ＰＡＳＰ与患儿左室短轴缩短分数、心功能低下持续时间及有无心内超声自发显影有关。随诊显示心功能好转者的ＰＡＳＰ较死亡或病情无改善者低，且后者肺动脉压继续增高。结论扩张型心肌病患儿多存在肺动脉高压，用多普勒超声估测的肺动脉压与患儿的病情及临床预后可能有一定的关系。     

室间隔缺损合并肺动脉高压肺血管床功能状诚评估指标的研究

探讨室间隔缺损合并肺动脉高压肺血和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，将轻工全肺循环阻力增加的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分别为２．３ｋＰａ和２．２ｋｐＡ，Ｐ〉０．０５差异无显著性意义；     

不伴体-肺动脉侧支的婴幼儿肺血减少型复杂先心病肺动脉发育程度的对比观察

目的 分析不伴体-肺动脉侧支的婴幼儿肺血减少型复杂先心病肺细小动脉结构，与左、右肺动脉的发育进行对照。方法 46例不伴体-肺动脉侧支的肺血减少型复杂先心病婴幼儿为病变组。对照组为同年龄组非心、肺源性疾病死亡患儿肺组织标本5例。形态半定量测量肺细小动脉平均中膜厚度百分比（MT％）、平均中膜面积百分比（MS％）和单位面积肺细小动脉数目（APSC）。结果 二组APSC的差异有统计学意义（P=0．007）。心血管造影测量的左、右肺动脉直径之间以及APSC与McGoon比值、肺动脉指数之间呈正相关性。结论 不伴体-肺动脉侧支的肺血减少型复杂先心病婴幼儿肺细小动脉的发育程度与中心肺动脉的发育程度具有良好的相关性。     

韧粘素在先天性心脏病合并肺高压患儿肺血管中的表达

为探讨韧粘素（ＴＮ）在先天性心脏病（先心病）合并肺高压发病机制中的作用及其其调节因素，采用免疫组化方法观察不同肺血清和／或不同肺动脉压力下，先心病患儿肺小动脉中ＴＮ的表达情况。图像分析结果表明，高肺血流伴肺高压时ＴＮ表达阳性面积比例明显高于高肺血流不伴肺高压和对照组（Ｐ均〈０．０１），ＴＮ表达与肺动脉压力升高显著相关（ｒ＝０．７５，Ｐ〈０．０１），与肺血流增多无关（ｒ＝－０．４８，Ｐ〉０．０５）。     

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.