哮喘患儿血一氧化氮,可溶性白细胞介素2受体及IgE变化

目的探讨哮喘患儿外周血一氧化氮（NO）、可溶性白细胞介素2受体（SIL-ZR）及IgE的变化。方法支气管哮喘急性发作期患者44例，男23例，女21例，对照组为15例健康儿童，男9例，女6例。分别在哮喘急性发作期和缓解期采静脉血，测定NO、SIL-ZR和IgE，并与对照组进行比较。结果支气管哮喘息性发作期患者与缓解期患者（30例）皿中NO分别为107.99±24.96μmol／L和81.36±14.31μmol／L（P＜0.001）;SIL-ZR分别为259.55±58.82kU／L和213.23±33.72kU／L（P＜0.001）;IgE分别为932.61±637.23kU／L和760.80±53644kU／L（P＞0.05）。对照组血中NO为82.26±14.21，与急性发作期哮喘息者相比，有显著性差异（P＜0.001）。结论支气管哮喘急性发作期患儿血中NO和SIL-2R均明显高于缓解期患者和健康儿童。内源性NO在哮喘的发病中可能发挥着重要作用。     

哮喘患儿单个核细胞产生IL—4,IFN—γ与血清IgE的关系

探讨哮喘患儿急性发作期与缓解期单个核细胞（PBMC）产生IL－、IFN－γ水平与IgE的关系。方法用ELISA方法测血清IL-4、IFN-γ和IgG水平，对检测结果统计学处理。结果哮喘急性期和缓解期血清IL-4水平分别为260．1±29．1ng／L和193．7±44．0ng／L（P＜0．001），IFN－γ分别为462．4±69．1ng／L和548．1±78．0ng／L（P＜0．001），Ige分别为2649．36±1413．88U／L和1726．39±1100．22U／L（P＜0．001），IL－4与IgE水平呈明显正相关。结论哮喘患儿急性发作期血清IL－4、IgE水平明显高于缓解期，IFN－γ低于缓解期，IL－4、IFN－γ和IgE在哮喘发病中具有重要作用。     

血浆和尿中内皮素、一氧化氮在神经源性膀胱中的检测及意义

目的探讨内皮素和一氧化氮在神经源性膀胱病人血浆和尿中含量的变化及其临床意义。方法将2003年10月至2004年8月明确诊断的22例患儿作为实验组，均为反射亢进型膀胱源性膀胱。留取空腹血和晨尿，采用酶联免疫方法检测血浆和尿中内皮素（ET—1），一氧化氮（NO）含量，选取同期入院年龄匹配的儿童的血尿标本（腹股沟斜和鞘膜积液30例）设为对照组。结果实验组22例，血浆 ET—1含量（0．487±0．039）fmol／mL，NO 含量（69．57±36．68）μmol／L，尿中 ET —1含量为（0.453±0．029）fmol／mL，NO 含量（392．36±121．1）μmol／L。对照组血浆 ET —1含量（0．494±0.117） fmol／mL，NO 含量（74．56±11．39）μmol／L，尿中 ET—1含量（0．548±0.073）fmol／mL，NO 含量（268．92±88．38）μmol／L。实验组中血浆 ET—1含量高于尿中 ET—1含量（P ＜0．01），尿中 ET—1含量低于对照组（P ＜0．05），尿中 NO 含量高于对照组（P ＜0．05）；血浆中 ET —1、NO 含量较对照组无明显差异（P ＞0.05）。结论反射亢进型神经源性膀胱病人血浆中 ET 和 NO 的含量处于正常水平；而尿中 ET—1和NO 比例失衡。     

内皮素、一氧化氮在毛细支气管炎和哮喘息儿中的变化

目的 探讨血内皮素(ET)和一氧化氮(NO)在毛细支气管炎(毛支)和婴幼儿哮喘(哮喘)中的变化及意义。方法 用放射免疫分析法和分光光度比色法分别检测毛支、哮喘患儿急性期和缓解期血ET和NO，并与正常儿童进行比较。结果 血浆ET在毛支组和哮喘组，急性期均明显高于正常组(P均＜0．01)，缓解期均下降，与正常组比较无显著差别(P均＞0．05)；两组急性期比较无差别(P＞0．05)。与血浆ET一样，血清NO在毛支组和哮喘组，急性期均显著高于正常组(P＜0．01和P＜0．05)；缓解期则下降，与对照组比无显著差别，(P均＞0．05)；两组急性期相比，差异无显著性(P＞0．05)。两组急性期血浆ET与血清NO呈明显正相关(P＜0．05)。结论 在毛支和哮喘中，血ET和NO均升高，二者关系密切，均可能参与毛支及哮喘的发病。     

哮喘患儿血小板计数与平均血小板容积水平变化的临床观察

探讨哮喘患儿血小板计数（Plt)与平均血小板容积（HPV）水平的变化及意义，采用美国Coulter-JT自动血细胞分析仪，检测30例哮喘患儿在喘息发作期及其中22例处于喘息缓解期的Plt及MPV水平，并与20例健康小儿作为对照。结果显示，哮喘患儿于喘息发作期存在Plt增多和MPV减小，与正常对照组比较有非常显著性差异（P＜0．01）；与缓解期比较有显著性差异（P＜0．05）；喘息缓解期Plt及MPV与正常对照组比较无显著性差异（P＞0．05），因而，动态观测哮喘发作时Plt及MPV的变化，可反映哮喘发作中血小板的活化状态，表明血小板参与了哮喘的病理过程；血小板活化可能在哮喘的发病机理中起一定作用，或有助于指导治疗。     

支气管哮喘患儿T淋巴细胞及细胞因子的变化

为观察儿童支气管哮喘时Ｔ淋巴细胞亚群分布以及Ｔ细胞活化相关因子及受体的表达状况，应用放射免疫分析等技术，对３４例发作期、２５例缓解期支气管哮喘患儿和１５例正常对照的外周血Ｔ淋巴细胞亚群、血浆及淋巴细胞膜表面白细胞介素－２受体（ＩＬ－２Ｒ）和相关细胞因子等水平进行系统检测。结果：（１）发作期患儿外周血Ｔ细胞亚群ＣＤ３，ＣＤ４及ＣＤ４／ＣＤ８值与缓解期患儿及正常对照比较差异无显著意义，但发作期ＣＤ８高于正常对照（Ｐ＜０．０１）和缓解组（Ｐ＜０．０１）；（２）发作期患儿血浆可溶性ＩＬ－２Ｒ、（ｓＩＬ－２Ｒ）、ＩｇＥ水平明显高于缓解期患儿和正常对照（Ｐ＜０．０１）；（３）免疫电镜观察显示，发作期患儿淋巴细胞膜表面ＩＬ－２Ｒ表达高于正常对照（Ｐ＜０．０５）。提示：（１）哮喘患儿外周Ｔ淋巴细胞亚群的分布发生了变化，哮喘发作期Ｔ淋巴细胞处于激活状态；（２）血浆ｓＩＬ－２Ｒ、ＩｇＥ水平与哮喘病情变化密切相关，可作为临床哮喘病情监测的指标。     

神经激肽A在哮喘患儿血浆含量变化的动态研究

目的　动态研究哮喘患儿血浆神经激肽A(NKA)含量变化规律 ,探讨NKA与小儿哮喘的关系。方法　用酶联免疫方法 ,动态测定 35例不同严重程度哮喘小儿血浆NKA在哮喘发作期及其临床症状缓解期的含量变化。结果　 (1 )小儿哮喘发作期血浆NKA含量 [(2 56± 1 53)ng/L]高于自身症状缓解期 [(70± 66)ng/L]及正常对照组 [(38± 6)ng/L] ,差异有非常显著意义 (q分别为9 497、8 599,P均 <0 0 1 ) ;哮喘症状缓解期血浆NKA含量较正常对照组差异无显著意义 (q =1 2 4 5 ,P >0 0 5)。 (2 )哮喘小儿病情加重 ,血浆NKA含量亦随之增高 ,重度哮喘发作时血浆NKA含量 [(2 96± 1 70 )ng/L]明显高于轻、中度哮喘发作时含量 [(1 90± 99)ng/L] ,差异有显著意义 (q =3 77,P <0 0 5)。结论　小儿哮喘发作期血中NKA含量明显增高 ,病情愈重增高越明显 ,随哮喘症状缓解血中NKA含量下降至正常水平 ;血中NKA含量的变化与小儿哮喘的发作及缓解关系密切     

哮喘缓解期儿童血清白细胞介素-13与免疫球蛋白E变化的意义

目的探讨哮喘患儿IL-13及总IgE变化的意义。方法采用酶联免疫吸附试验（ELISA）方法和Pharmacia UniCAP检测系统检测88例6～14岁的哮喘缓解期和40例正常对照儿童血清IL-13与总IgE水平。结果哮喘缓解期儿童临床症状和肺功能改善；血清IL-13水平高于正常儿童对照组（U=3．93 P〈0.01）；血清总IgE水平仍明显高于正常儿童对照组（U=10．52 P〈0.01）。血清IL-13水平与总IgE水平呈显著正相关（r=0．2685 P〉0．05）。结论哮喘缓解期变态反应性炎症持续存在，血清高质量浓度的IL-13和IgE与哮喘的免疫病理相关。     

心力衰竭时血浆内皮素和血管紧张素Ⅱ变化及药物干预研究

目的探讨心力衰竭患者血浆内皮素（ET）和血管紧张素Ⅱ（AngⅡ）的变化，并观察苯那普利对心衰患儿的疗效。方法40例充血性心力衰竭（CHF）患儿，随机分为苯那普利组（20例）和常规组（20例，其中5例未完成治疗）。用放射免疫法测定血浆ET和AngⅡ，比色法测定血管紧张素转换酶（ACE）水平。常规组用常规抗心衰治疗（洋地黄和利尿剂等），苯那普利组在常规治疗基础上加用苯那普利，治疗4～8周，观察各组治疗效果。结果正常对照组（20人）和40例CHF患儿血浆ET、AngⅡ和ACE水平分别为80．4±19．2ng／L对134．3±47．6ng／L（P＜0．001），96．0±35．5ng／L对223．9±95．5ng／L（P＜0．001），326．8±37．8IU对408．6±67．5IU（P＜0．001）。血浆ET和AngⅡ呈正相关（r＝0．324，P＜0．05）。苯那普利组治疗后ET、AngⅡ和ACE水平均明显下降，而常规组治疗前后均无明显差异。结论CHF患儿血浆ET、AngⅡ和ACE水平均显著高于正常儿童；常规治疗加苯那普利治疗CHF效果明显优于常规治疗。     

血清内分泌激素与小儿哮喘的关系

用放射免疫双抗体法测定74例哮喘息儿的4种血清内分泌激素水平，并与32名健康儿童比较。结果哮喘患儿的血清皮质醇（314．27±207．90nmol／L）、促甲状腺素（1．89±1．26mIU／L）水平均显著低于健康儿（P均＜0．01），生长激素（3．23±2．34μg／L）略降低（P＞0．05），雌二醇（0．231±0.118nmol／L）则显著高于健康儿，表明哮喘患儿存在内分泌功能紊乱。这可能与长期大剂量应用全身性肾上腺皮质激素，导致丘脑-垂体-肾上腺皮质轴的功能受抑有关。     

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.