内源性CO和NO与小儿哮喘关系的研究

目的:探讨小儿哮喘时血浆一氧化碳（CO）和一氧化氮（NO）的含量变化及意义。方法哮喘患儿21例，20名健康儿童为对照，分别在急性发作期和缓解期采静脉血测定血浆CO和NO水平。对测定结果进行统计学分析。结果发作期哮喘患儿血浆CO明显高于正常儿童（分别为1．66±035、1．06±0.12mg／L，t=7．26，P＜0.001），缓解期CO降至正常水平（1．13±0．14mg／L，t=1．56，P＞0．05）；发作期哮喘息地血浆NO2／NO3水平亦明显高于正常儿童（分别为32．8±3．9、29．3±0．28μmol／L，t=3．28，P＜0．01），缓解期NO2／NO3水平亦降至正常水平（29．7±3．0μmol／L，t=0．39，P＞0．05）。且哮喘患儿血浆CO和NO水平呈显著正相关（r=0.485，P＜0．05）。结论小儿哮喘发作期血浆CO和NO水平明显高于缓解期患儿及正常儿童。内源性CO和NO在哮喘发病机制中占有重要地位。     

糖皮质激素受体对急性淋巴细胞性白血病的疗效观察

研究目的探讨ALL患儿LGCR数量与化疗效果及预后的关系。研究方法以受体放射配基结合分析法检测50例ALL患儿及41例正常小儿静脉血淋巴细胞GCR水平，并观察其与化疗效果与预后的关系。研究结果正常小儿静脉血淋巴细胞GCR值为4651±1617结合位点／细胞，其95％正常参考值范围为1482～7800结合位点／细胞。ALL患儿淋巴细胞GCR值（6695±5256结合位点／细胞）明显高于正常小儿（t＝2．50，P＜0．05）。小儿ALL预后好组GCR值明显高于预后差组（t＝4．39，P＜0．001）。结论ALL患儿外周静脉血淋巴细胞GCR值可以作为一项生化指标而应用于判断预后及指导联合化疗。     

人乳蛋白制剂辅助治疗新生儿感染性肺炎

本文观察了由人初乳中提取的蛋白成份对新生儿肺炎的辅助治疗作用，130例新生儿肺炎患儿随机分为母乳组和强化母乳组、人工乳组和强化人工乳级，强化组给予人乳蛋白制剂。四组患儿体重增长分别为43．9±15．8克／天和47．4±14．9克／天（P＞0．05），14．7±19．7克／天和21．8±17．5克／天（P＞0．05）。临床症状减轻时间为4．8±1．9天和3．9±1．6天（P＜0．05），4．3±2．1天和4．1±1．9天（P＞0．05）。强化组在X线表现和血清免疫球蛋白方面也优于非强化组。结果提示人乳蛋白制剂对新生儿感染性肺炎有一定的辅助治疗作用。     

癫痫患儿的T细胞亚群和白细胞介素2的研究

本文检测了23例未经治疗的痛痛（Epilepsy，Ep）患儿和17例正常儿童的T细胞亚群和白细胞介素2（IL-2）水平，以探讨细胞免疫在EP发病中的作用。结果发现EP患儿的CD3，CD4阳性细胞百分率较对照组降低（P＜0．05），CD8升高（P＜0．01），CD4／CD8降低（P＜0．05），而IL-2水平无差异（P＞0．05），提示EP患者存在细胞免疫功能的紊乱。     

新生儿痰真菌生长的临床意义及相关因素探讨

目的探讨新生儿痰真菌生长的临床意义及相关因素。方法回顾性分析149例诊断为新生儿肺炎且痰培养结果阳性住院新生儿的临床资料,根据痰培养结果将患儿分为真菌组、混合组和细菌组,运用Х^2检验及方差分析等统计学方法,比较三组的临床资料。结果（1）真菌组40例、混合组30例和细菌组79例,痰真菌生长占47．0％（70／149）。（2）三组间白细胞数分别为（10．3±3．5）×10^9／L、（11．7±5．2）×10^9／L和（14．4±10．5）×10^9／L,F=3．78,P=0．03;中性粒细胞数分别为（5．1±3．3）×10^9／L、（7．4±4．7）×10^9／L、（9．0±7．4）×10^9／L,F=5．50,P=0．01;下列因素所占比例,三组分别为：早产儿32．5％（13／40）、20％（6／30）和12．7％（10／79）,Х^2=6．68,P=0．04;母产前使用糖皮质激素10．0％（4／40）、6．7％（2／30）和0％（0／79）,P=0．01;使用三联抗生素治疗10．0％（4／40）、16．7％（5／30）和2．5％（2／79）,P=0．02;碳青霉烯类药物治疗32．5％（13／40）、63．3％（19／30）和17．7％（14／79）,Х^2=21．26,P=0．00。上述6个因素三组间差异均有显著统计学意义。（3）以痰真菌生长作为因变量进行Logistic回归分析,共2个变量进入最佳回归方程：碳青霉烯类药物（克倍宁或泰能）治疗（X1）、早产儿（X2）,建立影响痰真菌生长的主效应模型Logistic（SCF）=β0（0．12）＋1．63X1＋1．20X2（Х^2=43．04,P〈0．05）。（4）仅一次痰真菌生长,抗真菌治疗与否未愈率分别为10．0％（2／22）和0％（0／43）,P=0．111;继续住院时间分别为（225．8±7．7）d和（434．1±4．7）d,t=1．095,P=0．278,均无统计学意义。结论（1）在新生儿肺炎中,痰真菌生长比较普遍,以白色念珠菌为主。（2）早产儿、碳青霉烯类抗生素治疗可作为痰真菌生长的独立危险因素。（3）仅一次痰真菌生长只提示需作进一步真菌检查,不能凭此确诊肺部真菌感染或决定是否抗真菌治疗。     

新生鼠窒息后心肌基质金属蛋白酶活性与心肌损害的相关性

目的探讨新生鼠窒息后心肌组织心肌基质金属蛋白酶（MMPs）表达与心肌损害的相关性。方法60只7—10日龄Wistar鼠,随机分成对照组（D组）、窒息组（A、B、C3组,分别为窒息复氧后1、7、14d）,每组15只。窒息组模拟新生鼠常压窒息模型,测定cTnI水平;心肌组织MMPs-3、9活性;心肌病理积分及胶原含量。结果cTnI（ng／m1）A组（0．3680±0．40）明显高于D组（0．0783±0．06）P〈0．05,B组下降（0．1889-i-0．15）P仍〈0．05,C组降至正常（0．1338±0．07）P〉0．05;心肌组织MMPs-3活性A组（0．1847±0．04）,B组最高（O．2780±0．05）,C组（0．2014±0．05）,与D组（0．1213±0．03）比较P均〈0．05;MMPs-9活性A、B、C组逐渐增高（0．1502±0．01、0．1715±0．01、0．1868±0．03、P均〈0．05）;心肌胶原含量A、B、C组逐渐增高（35．59±1．09、38．94±0．67、40．69±0．75ng／ml分别为P〉0．05、P〈0．05、P〈0．05）;MMPs-3、9表达与心肌病理积分呈正相关（r分别为0．669、0．667,P均〈0．05）,与胶原含量呈正相关（r分别为0．482、0．679,P均〈0．05）。结论新生鼠窒息后心肌MMPs-3、9过度激活,继发胶原含量增高,MMPs-3、9活性与心肌病理损害密切相关。提示MMPs参与了窒息后心肌损害,可能是心肌间质重塑的因素之一。     

吸入糖皮质激素对毛细支气管炎患儿肺功能影响的临床观察

目的观察毛细支气管炎患儿吸入糖皮质激素治疗前后的肺功能变化。方法选择60例毛细支气管炎患儿，随机分成治疗组（常规治疗＋吸入糖皮质激素＋β2受体激动剂）、对照组（常规治疗＋吸入β2受体激动剂），检测治疗前后睡眠时的潮式呼吸肺功能。主要参数为：达峰时间比（TPTEF／TE），达峰容积比（VPEF／VE），静态顺应性（Crs）和气道阻力（Rrs）。结果治疗组雾化吸入治疗前后潮式呼吸的各项参数有不同程度的改变TPTEF／TE（t=2．08，P〈0．05），VPEF／VE（t=2．84，P〈0．05），Crs（t=2．33，P〈0．05），Rrs（t=2．98，P〈0．05），经统计学检验差异有显著性。对照组潮式呼吸参数TPTEF／TE及VPEF／VE与治疗前相比差异有显著性（t=2．08，P〈0．05；t=2．35，P〈0．05），Crs与Rrs与治疗前相比差异无显著性（t=1．20，P〉0．05；t=1．26，P〉0．05）。两组治疗后比较，Crs及Rrs发生了明显变化，差异具有显著性（t=2．25，P〈0．05；t=2．09，P〈0．05）。结论吸入糖皮质激素（普米克令舒）治疗毛细支气管炎患儿对肺功能的改善有显著疗效。     

窒息新生儿血清肌钙蛋白I和磷酸肌酸激酶

目的探讨血清肌钙蛋白I（cTnI）和磷酸肌酸激酶同工酶（CK—MB）对窒息新生儿心肌损伤的早期诊断价值。方法选择轻度窒息新生儿29例（轻度组）、重度窒息新生儿18例（重度组）。采用ELISA法和酶动力法检测新生儿血清cTnI水平和CK—MB活性。结果出生d1窒息新生儿血清cTnI和CK-MB水平在轻度组[（2．25±0．54）μg／L、（223．4±23．5）U／L]和重度组[（4．25±0，83）μg／L、（256．3±21．8）U／L]均显著高于对照组（Pα〈0．01）；重度组血清cTnI和CK-MB水平均显著高于轻度组（Pα〈0．01）。治疗后d7窒息新生儿血清cTnI和CK—MB水平均明显下降，轻度组[（0．69±0．18）μg／L、（151．4±18．4）U／L]与对照组均无显著差异（Pα〉0．05），重度组[（1．54±0．72）μg／L、（188．9±21．5）U／L]显著高于轻度组和对照组（Pα〈0．01）。结论窒息新生儿伴心肌损伤时血清cTnI和CK—MB水平升高；动态观察可用于窒息新生儿微小心肌损伤的早期诊断。     

婴幼儿手术应激后瘦素水平变化的临床研究

目的探讨瘦素水平在婴幼儿大手术应激时的变化及意义。方法选择择期手术患儿17例,分先心组(10例)和非先心组(7例),分别于术前、术中、术后2h、术后24h、术后48h采集外周静脉血,离心后,测定血清中瘦素和皮质醇的水平。结果先心组和非先心组瘦素水平在术中均明显下降(术前分别为3．48±0．23、4．50±0．24．术中为2．12+0．14、3．09±0．21,P＜0．05)。术后24h又恢复至基础水平(3．78±0．19、4．89±0．43．P＞0．05)。皮质醇水平先心组术中显著升高(术前为25．39±1．74,术中为50．87±3．59,P＜0．05),非先心组术中升高(术前为28．61±1．69,术中为31．90±2．02,P＞0．05)；术后24h先心组恢复至基础水平(30．95±1．77,P＞0．05),非先心组水平仍高(37．48±1．92,P＜0．05)。结论在手术应激的早期,瘦素的下降和皮质醇的上升是平行的,瘦素在手术应激早期发挥作用。     

584例婴幼儿心—胸腺—胸比值的X线测量

本文收集584例婴幼儿正常胸片进行心－胸腺－胸比值(CTTI)x线测量，结果：新生儿组CTTI为0．36±0．08，－1岁组0．35±0．06，－2岁组0．31±0．05，－3岁组0．29±0．05（F＝51．89，p<0.01)，且随年龄增大CTTI缩小。CTTI能反映胸腺大小，测量方法简单，值得临床采用。     

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.