小儿病毒性心肌炎与Fas/Fasl介导的外周血淋巴细胞凋亡的关系

目的 探讨Fas/Fasl介导的外周血淋巴细胞凋亡在病毒性心肌炎发病中的作用.方法 选择临床诊断病毒性心肌炎患儿和正常小儿各25例,采用Annexin V/PI双参数法经流式细胞仪定量检测两组小儿外周血淋巴细胞凋亡百分率,并用双色流式细胞仪检测外周血淋巴细胞Fas、Fasl蛋白表达结果心肌炎患儿外周血淋巴细胞凋亡率[(0.697 6±0.110 9)%]较正常对照组[(0.128 8±0.106 9)%]明显升高(t=18.459,P＜0.001),且与肌酸激酶同工酶、乳酸脱氢酶、肌酸激酶、羟丁酸脱氢酶升高程度均呈正相关(r=0.817,P＜0.01;r=0.785,P＜0.01;r=0.726,P＜0.01;r=0.712,P＜0.01);心肌炎患儿外周血淋巴细胞Fas、Fasl蛋白表达[(2.8804±0.3020)、(4.4496±0.4836)]较对照组[(2.1300±0.6200)、(0.123 8±0.0384)]明显增加(t=17.321,P＜0.001;t'=44.577,P＜0.001);心肌炎患儿外周血淋巴细胞凋亡百分率与 Fas、Fasl蛋白表达均呈正相关(r=0.972,P＜0.01;r=0.958,P＜0.01)结论病毒性心肌炎患儿外周血淋巴细胞凋亡增加,可能为细胞免疫功能下降的原因之一,且与心肌损害程度相关 Fas、Fasl基因在病毒性心肌炎患儿外周血淋巴细胞凋亡中起着重要作用.     

小儿病毒性心肌炎与Fas／Fas1介导的外周血淋巴细胞凋亡的关系

目的探讨Fas／Fas1介导的外周血淋巴细胞凋亡在病毒性心肌炎发病中的作用。方法选择临床诊断病毒性心肌炎患儿和正常小儿各25例,采用Annexin V／PI双参数法经流式细胞仪定量检测两组小儿外周血淋巴细胞凋亡百分率,并用双色流式细胞仪检测外周血淋巴细胞Fas、Fas1蛋白表达。结果心肌炎患儿外周血淋巴细胞凋亡率[（0．6976±0．1109）％]较正常对照组[（0．1288±0．1069）％]明显升高（t=18．459,P〈0．001）,且与肌酸激酶同工酶、乳酸脱氢酶、肌酸激酶、羟丁酸脱氢酶升高程度均呈正相关（r=0．817,P〈0．01;r=0．785,P〈0．01;r=0．726,P〈0．01;r=0．712,P〈0．01）;心肌炎患儿外周血淋巴细胞Fas、Fas1蛋白表达[（2．8804±0．302O）、（4．4496±0．4836）]较对照组[（2．1300±0．6200）、（0．1238±0．0384）]明显增加（t=17．321,P〈0．001;t’=44．577,P〈0．001）;心肌炎患JLgb周血淋巴细胞凋亡百分率与Fas、Fas1蛋白表达均呈正相关（r：0．972,P〈0．01;r=0．958,P〈0．01）。结论病毒性心肌炎患儿外周血淋巴细胞凋亡增加,可能为细胞免疫功能下降的原因之一,且与心肌损害程度相关。Fas、Fas1基因在病毒性心肌炎患儿外周血淋巴细胞凋亡中起着重要作用。     

病毒性心肌炎与外周血淋巴细胞凋亡的关系

近年来体外实验研究表明 ,许多病毒感染可诱发Ｔ细胞凋亡 ,且Ｆａｓ/ＦａｓＬ径路为Ｔ细胞凋亡的主要方式之一[1] 。为了探索病毒性心肌炎 (ＶＭ)与Ｆａｓ/ＦａｓＬ介导的外周血淋巴细胞凋亡的关系 ,我们检测了ＶＭ患儿外周血淋巴细胞凋亡百分率和ＣＤ 3 细胞Ｆａｓ表达及ＣＤ 8细胞ＦａｓＬ的表达情况 ,以探讨ＶＭ继发性免疫功能低下的原因。对象心肌炎组患儿 15例均为 1997年 10月～ 1998年 6月在我院住院的患儿 ,其中男 9例 ,女 6例 ;年龄 1～ 12岁 (平均8.2岁 )。小儿ＶＭ的诊断标准按照 1994年 5月威海会议修订的小儿ＶＭ诊断标准[2 …     

支气管哮喘患儿血T辅助淋巴细胞的变化

目的探索支气管哮喘儿童细胞免疫状况的变化。方法采用流式细胞仪检测支气管哮喘儿童急性期及正常对照组外周血T辅助淋巴细胞1(Th1)和Th2的百分率、CD4 、CD3、CD8、NK细胞、B细胞百分率及CD4/CD8比值。对10例哮喘缓解期患儿复查淋巴细胞亚群。结果支气管哮喘患儿发作期外周血Th1细胞百分率[(16.28±9.83)%]明显低于正常对照组[(20.77±6.89)%](P<0.05);哮喘组Th2细胞百分率[(5.44±1.96)%]较对照组[(4.21±2.12)%]明显增高(P<0.05)。Th1/Th2比值,哮喘组为3.41±2.56,对照组为5.91±3.24,两组比较有非常显著性差异(P<0.01)。但哮喘组B细胞、NK细胞百分率、CD3、CD4、CD8及CD4/CD8与正常对照组比较均无统计学差异(P均>0.05)。10例哮喘缓解期患儿淋巴细胞亚群结果表明,B细胞百分率显著下降(P<0.05),其他各项指标在发作期与缓解期无明显差异。结论支气管哮喘儿童最重要的免疫异常是Th1/Th2细胞比例和功能失衡,主要表现为Th2细胞应答优势存在,Th1/Th2显著降低。     

病毒性心肌炎小鼠心肌细胞凋亡和坏死的研究

目的： 探讨病毒性心肌炎小鼠心肌细胞的凋亡与坏死之间的相互关系。方法：　用4周龄Balb/c雄性小鼠腹腔接种柯萨奇B3病毒 (CVB3)制成病毒性心肌炎小鼠模型 ,采用膜联蛋白V联合PI染色双参数技术(AnnexinV/PI)在流式细胞仪上定量检测心肌细胞的凋亡和坏死。结果：　病毒性心肌炎小鼠心肌细胞的凋亡百分率和坏死百分率均显著高于正常对照小鼠(P<0.01),并且心肌细胞凋亡百分率及坏死百分率均与心肌病理积分呈正相关(r =0.70 ,P<0.01和r=0.93,P<0.01)。结论：　病毒性心肌炎小鼠同时存在着心肌细胞的凋亡和坏死 ,两者均与心肌病理损害程度密切相关,为病毒性心肌炎的重要病理过程     

心肌炎患儿淋巴细胞凋亡的改变

目的 了解心肌炎患儿的淋巴细胞凋亡的改变,进一步探讨心肌炎的发病机理。方法 荧光法检测41例心肌炎患儿外周血淋巴细胞凋亡的改变,同时测定T淋巴细胞亚群与免疫球蛋白的变化。结果 心肌炎组淋巴细胞凋亡率高于正常组,且T淋巴细胞亚群CD4+,CD4+／CD8+明显下降,CD8+增高(P<0.01)。结论 淋巴细胞凋亡过度导致淋巴细胞总量失衡,T淋巴细胞及体液免疫功能均低下,可能是病毒或支原体感染后引起心肌炎的重要原因之一。     

病毒性心肌炎患儿血清sFas、sFasL的表达水平

目的 探讨可溶性Fas(sFas)及其配体(sFasL)在小儿病毒性心肌炎中的表达以及与细胞凋亡和免 疫反应的关系。方法 采用ELISA法对21例病毒性心肌炎患儿和20例健康儿童进行血清sFas和sFasL的浓度 测定。结果 病毒性心肌炎患儿血清sFas和sFasL表达水平较健康儿童明显增高(P均<0.01);病毒性心肌炎患 儿血清sFas与sFasL表达水平呈正相关(P<0.01)。结论 血清sFas及sFasL表达增高与病毒性心肌炎疾病过 程有关,其作用机制可能通过抑制或减弱激活的T淋巴细胞凋亡造成免疫性心肌损害;病毒性心肌炎患儿血清 sFas表达增高进一步证实其发病与免疫因素有关;血清中sFas和／或sFasL表达增高可望作为T淋巴细胞激活的 标志和病毒性心肌炎的诊断依据之一。     

柯萨奇-腺病毒受体在病毒性心肌炎患儿白细胞中的表达及意义

目的观察柯萨奇-腺病毒受体(CAR)在病毒性心肌炎(VM)外周血白细胞中的表达及意义。方法选择柯萨奇B病毒性心肌炎患儿及同龄健康儿童各20例,采用全血免疫荧光流式细胞术检测白细胞中CAR表达水平,并对VM患儿CAR表达值与心功能(EF值)的变化相关性进行分析。结果流式细胞表明,心肌炎组(VM组)平均荧光强度(MFI)和阳性细胞百分率(PPC)均明显高于正常对照组,差异均有高度显著性(t=6.23、10.87,P均<0.01);其MFI与EF值呈显著负相关(r=-0.757,P<0.05)。结论CAR表达水平有助于判断患儿对柯萨奇B组病毒感染的易感性,对VM的诊断、判断病情和预后有一定的价值。     

动态监测血乳酸与小儿危重病例评分的相关性

目的 探讨动态监测PICU患儿血乳酸与小儿危重病例评分(PCIS)的相关性.方法 对77例入住南通大学附属常州儿童医院儿科重症监护病房的患儿立即进行PCIS,根据评分结果分为极危重组(23例)、危重组(32例)、非危重组(22例).并检测患儿动脉血乳酸水平,每6 h监测1次,并测出乳酸峰值.比较各组间乳酸监测指标(入PICU乳酸水平、乳酸峰值)和PCIS,进行相关性分析,探讨其与患儿预后的相关性.结果 极危重组血乳酸水平:入PICU时乳酸[(5.28±3.69) mmol·L-1]、乳酸峰值[(8.54±4.32) mmol·L-1]明显高于危重组和非危重组(F=3.98,3.12,Pa<0.01),而PCIS[(65.79±2.34) 分]明显低于其他2组(F=4.23,P<0.01);死亡组 PCIS[(62.35±4.22) 分]低于存活组[(89.21±5.36) 分](t=3.15,P<0.01),而血乳酸水平[(5.31±4.05) mmol·L-1]高于存活组[(3.22±2.13) mmol·L-1](t=2.32,P<0.05);PCIS与血乳酸水平呈负相关(r=-0.889,P<0.01).结论 血乳酸升高的PICU危重患儿病情更重、预后更差,PCIS评分可有效评估患儿的病情和预后,并与乳酸水平存在显著负相关.动态监测血乳酸水平是反映危重病患儿病情严重程度和预测患儿转归的较好指标.     

过敏性紫癜患儿急性期淋巴细胞凋亡与血清可溶性Fas和FasL变化的关系

目的 探讨过敏性紫癜(HSP)患儿急性期外周血淋巴细胞凋亡与血清可溶性Fasm(sFas)、可溶性FasL(sFasL)水平变化及其相互关系。方法 选择HSP患儿及健康儿童各33例。分别应用形态法、间接免疫荧光法、双抗夹心ABC-ELISA法检测外周血淋巴细胞凋亡率、CD25^ 细胞百分率及血清sFas及sFasL水平。结果 HSP患儿急性期外周血淋巴细胞培养0、48h凋亡率均显著高于正常对照组(P均＜0．01)；HSP组培养48h CD25^ 细胞百分率显著低于正常对照组(P＜0．01)，HSP患儿外周血淋巴细胞培养48h凋亡率与CD25^ 细胞百分率呈负相关(r＝-0．61 P＜0．05)；血清sFas及sFasL水平较正常对照组明显升高(P均＜0．01)；HSP患儿外周血淋巴细胞培养48h凋亡率与sFasL水平呈正相关(r＝0．69 P＜0．05)，与sFas水平呈负相关(r＝-0．58 P＜0．05)。结论 HSP患儿外周血淋巴细胞凋亡过度和血清sFas、sFasL水平升高，与患者免疫功能紊乱关系密切。血清sFasL升高是HSP患儿淋巴细胞凋亡过度的重要原因之一。     

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.     

How well are we doing? – Metabolic control in patients with diabetes

OBJECTIVE: To compare the present level of metabolic control in children and adolescents with insulin-dependent diabetes mellitus (IDDM) attending Brisbane paediatric diabetes clinics with published overseas data. METHODOLOGY: Blood HbA1c concentrations, population characteristics, current treatment practices and short-term complications were recorded in all patients, aged 19 years and under, attending the diabetes clinics of the two Brisbane Children's Hospitals or the private practice of one of the authors (MJT) in the first quarter of 1998. RESULTS: Two hundred and sixty-eight patients were assessed (M/F 142/126). Ages ranged from 1 to 19 years (mean 11. 2 years); duration of IDDM was 0-16 years (mean 4.4 years); and 141 (53%) were pubertal. Of those aged less than 13 years, only 4% had more than two injections daily. Insulin doses (U/kg/day) rose with increasing age. Larger doses were required in regimens involving more than two injections per day than those involving one to two injections per day. Ketoacidosis or severe hypoglycaemia in the last 3 months were reported in eight (2.7%) and 17 (6.3%) of patients, respectively. Mean HbA1c (+/- SD) was 8.6 +/- 1.4% (range 5.2-14.0%), with 33% of children having a HbA1c concentration < 8%. HbA1c concentrations were significantly related (P < 0.05) to insulin dose and to duration of diabetes, but not to severe hypoglycaemia, ketoacidosis, age, frequency of injections, or number of clinic visits per year. Mean HbA1c concentration was significantly higher (P < 0.05) in those children in puberty (8.7 +/- 1.5%) than in those not in puberty (8.5 +/- 1.2%). CONCLUSION: Only 33% of patients had a HbA1C concentration less than 8% and 6.3% had a severe hypoglycaemic episode in the 3 months. These results are similar to published overseas data.