优选静脉免疫球蛋白剂量治疗川崎病临床与免疫学效应研究

目的 探讨１ｇ／ｋｇ与２ｇ／ｋｇ单剂静脉注射免疫球蛋白（ＩＶＩＧ）两种剂量治疗儿童川崎病是否具有同样理想的临床效果与免疫调节效应。方法 对比急性期症状与实验室指标恢复情况及随访半年内冠关动脉病变（ＣＡＤ）发生率,以此作为临床判断指标,观察治疗前后处岣血淋巴细胞凋亡百分率与凋亡细胞ＤＮＡ片段出现等细胞凋亡延迟纠正状况,作为ＩＶＩＧ治疗川崎病发挥免疫调节效应的判断指标。结果 川崎病发病５￣７天内单剂给     

大剂量维生素C在新生儿再灌注损伤中的应用

观察了大剂量维生素Ｃ（ＶｉｔＣ）用于新生儿再灌注损伤的疗效。结果ＶｉｔＣ１ｇ／（ｋｇ·ｄ）的疗效明显优于０．５ｇ／（ｋｇ·ｄ）。前者首次用药后患儿血清丙二醛（ＭＤＡ）明显减少，总超氧化物歧化酶（ＳＯＤ）明显增加（Ｐ＜０．００１），血液酸度无明显变化（ｐＨ：Ｐ＞０．１，ＨＣＯ３－：Ｐ＞０．０５，ＢＥ；Ｐ＞０．１）。而后者首次用药后患儿血清ＭＤＡ无明显减少（Ｐ＞０．０５），ＳＯＤ虽明显增加（Ｐ＜０．０１），但增加的幅度明显低于前者，提示ＶｉｔＣ作为自由基清除剂治疗新生儿再灌注损伤时，剂量以１ｇ／（ｋｇ·ｄ）为宜。     

静脉注射免疫球蛋白治疗川崎病的临床观察

静脉注射免疫球蛋白治疗川崎病的临床观察孙涛李永柏刘淑英陈维金涂文伟杨红育聂荣国谢依群尹秀兰杨锡强川崎病（ＫＤ）是一种原因不明的小儿急性发热性疾病。以前采用阿司匹林（ＡＳＰ）为主的抗凝、抗炎和对症治疗。近年国外报道静脉注射免疫球蛋白（ＩＶＩＧ）可迅速控...     

柯萨奇B组病毒感染与小儿哮喘发作关系的探讨

目的　探讨柯萨奇 Ｂ 组病毒（ Ｃ Ｖ Ｂ） 感染与哮喘发作的关系。方法　观察急性发作期哮喘患儿１０２ 例、上呼吸道感染１８２ 例、非感染性疾病患儿７１ 例、健康儿童３０ 例。采用 Ｅ Ｌ Ｉ Ｓ Ａ 法测定静脉血 Ｃ Ｖ Ｂ 抗原（ Ｃ Ｖ Ｂ－ Ａｇ）和 Ｉｇ Ｍ 抗体（ Ｃ Ｖ Ｂ－ Ｉｇ Ｍ） 、免疫组化法测定 Ｔ 细胞亚群和［３ Ｈ］ － Ｔｄ Ｒ 标记法测定 Ｎ Ｋ 细胞活性。结果　①哮喘患儿 Ｃ Ｖ Ｂ 感染率为５７８ ％ （５９／１０２） ;②哮喘患儿 Ｃ Ｄ８ 显著升高; Ｃ Ｄ３ 、 Ｃ Ｄ４／ Ｃ Ｄ８ 及 Ｎ Ｋ 细胞活性均明显下降（ Ｐ ＜００１） ;而 Ｃ Ｖ Ｂ 阳性哮喘患儿 Ｎ Ｋ 细胞活性又低于非 Ｃ Ｖ Ｂ 阳性哮喘儿（ Ｐ ＜ ００５） , Ｃ Ｄ８ 明显高于非 Ｃ Ｖ Ｂ 阳性哮喘儿（ Ｐ ＜ ００１） 。结论　 Ｃ Ｖ Ｂ 感染与哮喘发作有密切关系。     

32例小儿咳嗽变异性哮喘免疫功能观察

为探讨咳嗽变异性哮喘（ＣＶＡ）发病机理，本文采用病例对照方法，对３２例临床诊断ＣＶＡ患儿进行外周血Ｔ淋巴细胞亚群和血清免疫球蛋白测定，结果表明，观察组ＣＤ３，ＣＤ８明显降低，ＣＤ４／ＣＤ８比值升高，血清ＩｇＥ明显增高。与对照组比较差异非常显著（Ｐ〈０．０１），提示ＣＶＡ患儿存在着免疫功能紊乱，其发病机理可能与支气管哮喘相同。     

静脉注射免疫球蛋白治疗新生儿缺氧缺血性脑病的临床与免疫学 …

目的 研究静脉注射免疫球蛋白（ＩＶＩＧ）治疗新生儿缺氧缺血性脑病（ＨＩＥ）的临床与免疫学机理。方法 设ＩＶＩＧ治疗组（ｎ＝２）及常规治疗组（ｎ＝２２）。比较两组患儿治疗后ＨＩＥ症状消失时间、住院时间、器官功能障碍发生率及肌酸磷酸激酶（ＣＰＫ）水平，同进检测治疗前后血浆及外周血单个细胞（ＰＢＭＣ）体外产生白细胞介素６（ＩＬ－６）及肿瘤坏死因子α（ＴＮＦ－α）水平。还观察ＩＶＩＣ在体外对患儿ＰＢＭＣ产     

合胞病毒感染儿童淋巴细胞多种表面标记及细胞因子的初步研究

呼吸道合胞病毒（ＲＳＶ）感染发病机理的研究一直受到重视，研究者发现ＲＳＶ感染患儿存在着一系列免疫功能紊乱。本文用ＡＰＡＡＰ技术对ＲＳＶ感染患儿淋巴细胞ＣＤ３、ＣＤ４、ＣＤ８、ＣＤ２３、ＣＤ２５、ＣＤ５７、ＩＦＮｒ、ＨＬＡ－ＡＢＣ、ＨＬＡ－ＤＰ和ＨＬＡ－ＤＲ等表面标记进行检测，并用ＥＬＩＳＡ技术检测血浆中白细胞介素２（ＩＬ－２）、可溶性白细胞介素２受体（ｓＩＬ－２Ｒ）和呼吸道合胞病毒特异性ＩｇＥ（Ｒ     

川崎病血液流变学的初步探讨

为探讨川崎病（ＫＤ）血液流变学改变，采用血液比粘度计检测了７８例ＫＤ患儿急性期与药物治疗１５天后的血粘度。结果：（１）急性期与对照组比较，全血低切粘度、血浆粘度（Ｖｐ）、血沉方程Ｋ值（ＥＳＲ－Ｋ）、红细胞聚集指数、红细胞电泳时间及纤维蛋白原（Ｆｉｂ）明显增高（Ｐ＜０．０１）；红细胞压积（ＨＣＴ）显著降低（Ｐ＜０．０１）。（２）药物治疗１５天后血液流变学指标明显改善，而Ｖｐ、ＥＳＲ－Ｋ仍增高（Ｐ＜０．０１）；ＨＣＴ仍降低（Ｐ＜０．０１）。（３）ＫＤ血粘度增高的主要原因是红细胞聚集性和Ｖｐ增高，后者与Ｆｉｂ、脂质过氧化物及免疫球蛋白呈正相关。研究表明ＫＤ患儿存在高粘、高聚、高凝、低血浓度状态。阿司匹林、复方丹参有抗粘、抗凝疗效，宜较长时间应用。     

静脉注射丙种球蛋白治疗新生儿ABO溶血病22例疗效观察

为探讨静脉注射丙种球蛋白（ＩＶＩＧ）对新生儿ＡＢＯ溶血病的疗效，随机将４２例ＡＢＯ溶血病新生儿分为ＩＶＩＧ治疗组和常规治疗组，两组均于治疗前和治疗后分别查血红蛋白，网织红细胞、血清胆红素及ＩｇＧ．Ａ．Ｍ。结果：ＩＶＩＧ组在皮肤黄疸消退，降低血清胆红素疗效方面均明显优于常规组（Ｐ〈０．０１），ＩｇＧ水平明显提高（Ｐ〈０．０１）ＩｇＡ与Ｉｇｍ水平无变化（Ｐ〉０．０５）；常规组在治疗３－４天后血红蛋白水     

川崎病患儿血清 TNF-α、IL-6 的变化及其与冠状动脉病变之间关系的探讨

为探讨肿瘤坏死因子α（ＴＮＦ－α）和白细胞介素６（ＩＬ－６）在川崎病（ＫＤ）发病中的作用以及其与ＫＤ冠状动脉病变（ＣＡＤ）之间的关系。采用ＥＬＩＳＡ测定ＫＤ患儿血清ＴＮＦ－α和ＩＬ－６水平,与正常对照组进行比较,并将ＫＤ并ＣＡＤ组与无ＣＡＤ组进行比较,结果ＫＤ患儿血清ＴＮＦ－α和ＩＬ－６皆明显高于正常对照组（Ｐ〈０．０１）,ＴＮＦ－α在ＫＤ并ＣＡＤ组患儿亦高于无ＣＡＤ组患儿（Ｐ〈０．０５）,而ＩＬ－６在两组中无显著差别（Ｐ〉０．０５）,提示ＴＮＦ－α和ＩＬ－６皆参与了ＫＤ的发生,而ＴＮＦ－α在ＫＤ患儿ＣＡＤ中,可能起到比ＩＬ－６更为重要的作用。     

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.