胎儿炎症反应综合征与新生儿疾病

胎儿炎症反应综合征（FIRS）是指胎儿免疫系统被激活释放大量致炎因子而导致的一种亚临床状态,是全身炎症反应综合征（SIRS）在胎儿期的特殊表现形式。宫内感染是导致FIRS的最常见病因。FIRS与早产、早产儿脑白质损伤、支气管肺发育不良、新生儿坏死性小肠结肠炎等多种新生儿疾病的发生密切相关。     

全身炎症反应综合征与脓毒症

全身炎症反应综合征一、定义全身炎症反应综合征(systemic inflammato-ry response syndrome,SIRS)是机体对各种严重损伤,包括感染、创伤、烧伤、缺氧和再灌注等引起的全身反应。机体受到严重打击后引起免疫系统的应激反应,包括多种炎性介质的大量释放,导致抗炎反应与致炎反应系统失衡,若过度的炎症反应继续发展或恶化,可导致急性肺损伤(ALI)、急性呼吸窘迫综合征(ARDS)、多器官障碍综合征(MODS)或MSOF等。SIRS的含义比脓毒症(sepsis)更广泛,更有意义,它不是一种疾病,而是基于对感染、炎症和危重症发生、发展机制深入认识提出的新…     

小儿SIRS凝血系统的变化及临床意义

全身炎症反应综合征(systemic inflammationresponse syndrome,SIRS),是机体对包括感染和非感染等严重刺激所产生的一种失控的全身炎症,这种炎症反应如果继续失控恶化,最终不可避免地引起多器官功能障碍综合征(MODS),炎性介质失控性释放是其主要发生机制。但近年来研究已经初步了解了在SIRS和MODS的炎症反应启动和发展过程中,凝血和抗凝血系统的改变起着关键性作用,而凝血系统激活又可促进SIRS的发展、恶化。国内外学者已对SIRS时部分凝血、纤溶系统的检测指标作过研究,但在小儿全面系统的研究不多。为此,我们对SIRS患儿的凝血…     

中性粒细胞呼吸爆发与肺炎症反应

在肺炎症反应中,中性粒细胞(PMN)是重要的局部反应细胞,PMN参与肺炎症反应的机制主要包括两方面:(1)PMN激活与内皮黏附;(2)激活的PMN通过呼吸爆发和脱颗粒作用,产生大量的氧自由基、多种蛋白水解酶以及细胞因子等炎症介质,引起肺组织损伤。该文就近年来PMN在肺炎症反应机制中的研究进展作一综述,对寻求肺炎症性疾病新的治疗方法将有重要意义。     

败血性休克发病机制的研究进展

败血性休克的发病基础是机体过强的炎症反应。现代研究认为活化的细胞因子相互作用而中介产生的周身性炎症反应综合征充分反映了败血性休克的病理生理与临床表现。周身性炎症反应综合征可分为几个不同的阶段。包括败血症、败血综合征、休克早期、难治性休克、多脏器损害和死亡。参与炎症反应的因子主要有白细胞介素－１、血小板激活因子、肿瘤坏死因子、脂多糖和多形核白细胞等。病原体代谢产物与细胞因子相互作用的结果：激活了补体－凝血系统，激活了激肽释放酶，体内释放β－内啡肽及多形核白细胞在数量、形态与功能上的改变。使机体始终处于高敏的炎症反应中，形成毛细血管通透性增强，内皮受损，血小板凝聚加强和平滑肌收缩。最终造成休克的徽循环障碍。     

体外循环与全身炎症反应综合征

体外循环的临床应用开辟了心脏手术新纪元 ,但因其为非生理状态的血液循环、体温改变、内毒素释放及缺血再灌注损伤等因素可诱导补体活化、黏附分子表达、嗜中性粒细胞激活、细胞因子释放、脂类介质和内皮源性炎症因子产生、大量氧自由基生成。这些炎症介质相互间形成非常复杂的网络 ,引起迅速放大的炎症级联反应 ,触动全身炎症反应综合征 (SIRS)的发生 ,严重者可导致多器官功能不全甚至多器官功能衰竭。该文通过对体外循环时相关致炎因子的产生、触发SIRS及脏器损害的作用机制进行探讨 ,以便临床早期干预SIRS的发生、发展 ,减少术后并发症 ,提高手术成功率     

全身炎症反应综合征/代偿性抗炎症反应综合征与TH1/TH2亚群关系

重症感染时细菌、病毒等或严重创伤均可激活炎症细胞 ,释放多种炎症介　　作者简介 :刘建华 ( 1965 -) ,女 ,河北栾城人 ,主治医师。质 ,并促发炎症介质瀑布样释放 ,导致全身炎症反应综合征 (ＳＩＲＳ)。与此同时 ,机体可释放内源性抗炎症介质 ,以对抗促炎介质 ,防止ＳＩＲＳ引起的自身组织损伤。但内源性抗炎症介质过量释放将导致代偿性抗炎症反应综合征(ＣＡＲＳ) ,导致细胞免疫功能的抑制 ,增加宿主对感染的易感性。ＳＩＲＳ/ＣＡＲＳ之间的平衡决定了机体内环境的稳定性 ,决定了感染、创伤的后果。研究显8181全身炎症反应综合征/代偿性…     

婴幼儿腹部大手术后细胞因子的变化

新生儿和婴幼儿手术应激后死亡率及并发症的出现远较成人高,确切机制尚不清楚。近年来许多研究表明,在机体对损伤的反应中,细胞因子作为炎症介质起着主要的作用,在这些细胞因子中,肿瘤坏死因子(TNF-α)、白细胞介素1β(IL-1β)和白细胞介素6(IL-6)被认为是炎症和组织损伤后急性期反应的     

SIRS ALI/ARDS的临床相互关系与治疗

目前已认识到一些危重症的病理生理过程 ,与炎性介质的参与有关。炎性介质包括细胞因子、内皮素、自由基、活化的中性粒细胞、活化的补体Ｃ5ａ、组织胺、环氧化酶等。细胞因子是细胞自身分泌产生的一种肽类物质 ,当机体受到感染或损伤后 ,受累的组织和网状内皮系统则产生大量的细胞因子 ,如肿瘤坏死因子 (ＴＮＦ α)、白细胞介素 (ＩＬ)、血小板激活因子 (ＰＡＦ)等 ,引起全身炎症反应综合征(ＳＩＲＳ)。ＴＮＦ α具有广泛的生物活性 ,是机体炎症反应中最重要的炎性介质 ,能诱发多种细胞因子释放 ,引起全身炎症反应。白细胞介素有多种 ,如白…     

2009甲型H1N1流感患儿免疫功能改变初探

目的 探讨2009甲型H1N1流感(以下简称"甲流")患儿免疫功能及可能的免疫发病机制.方法 深圳市儿童医院2009年11月1日-2010年1月10日甲流住院患儿60例,轻症35例(轻症肺炎),重症25例(重症肺炎或甲流相关性脑病,死亡3例),同年龄正常对照组20例.采用real-time PCR、流式细胞术及ELISA检测外周血单个核细胞胞浆模式识别受体(PRRs)维甲酸诱导基因I/黑色素瘤分化相关基因5(RIG/MDA5)、胞膜PRRs Toll样受体(TLRs)分子及其信号途径传导分子、细胞因子/趋化因子及负性调节因子变化;T、B及NK细胞凋亡及凋亡相关基因TRAIL和CASPASE-3表达.结果 (1)甲流患儿RIG/MDA5表达、TLR2、TLR4表达明显高于正常对照组[TLR2(9.69±3.15)×10-2vs.(3.96±0.83)×10-2,t=10.16,P＜0.05;TLR4(10.23±2.85)×10-2vs.(7.46±2.18)×10-2,t=3.76,P＜0.05],以重症甲流患儿增高为著,RIG/MDA5表达增高最为明显;TLRs途径信号传导分子MyD88、TRAM等表达明显高于轻症甲流患儿.(2)甲流患儿CD3+[(1.22±0.38)×109/Lvs.(3.59±1.10)×109/L,t=9.21,P＜0.05]、CD4+、CD8+T细胞及NK细胞绝对计数明显低于正常对照组,B细胞无明显改变.(3)轻症甲流患儿TNF-α、IL-6、IL-1β等炎症细胞因子血浓度或基因高于正常对照组,重症患儿炎症细胞因子TNF-α[(6.42±1.76)×10-2vs.(9.05±2.51)×10-2,t=4.55,P＜0.05]明显低于正常对照组.IFN-α/β表达持续高于正常对照组,尤以重症甲流患儿为著;IFN-I诱导基因IP-10[(20.52±6.09)×10-2vs(1.18±0.34)×10-2,t=18.74,P＜0.05]、RANTES或iNOS轻症患儿表达高于正常对照组,重症患儿表达则趋于减少.(4)甲流患儿CD3+[(32.90±7.66)%vs.(20.21±6.58)%,t=6.21,P＜0.05]、CD4+、CD8+T细胞、NK细胞凋亡高于正常对照组,以重症患儿更为显著.凋亡相关基因TRAIL和CASPASE-3表达明显高于正常对照组.(5)重症患儿PRRs负性凋节因子SOCS1、SOCS3、IRAK-M、TRAF4及FLN29表达明显高于轻症患儿,抗炎细胞因子IL-10及IL-10/TNFα比值随病情加重增高.结论 甲流患儿机体免疫功能紊乱,轻症患儿处于全身免疫激活状态,重症患儿同时存在免疫激活/免疫抑制反应.     

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.     

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.     

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.