新生儿出生第1天凝血指标与危重评分相关性研究

目的 探讨新生儿出生第1天凝血功能指标与危重评分的相关性.方法 对2008年12月至2010年4月出生第1天入住本院的足月儿和早产儿进行危重评分,并检测凝血指标,比较患儿凝血指标与疾病严重程度的关系,以及足月儿和早产儿凝血指标在危重评分各组之间的差异.结果 出生第1天足月儿抗凝血酶Ⅲ(AT-Ⅲ)与疾病严重程度负相关,D-二聚体(D-D)与疾病严重程度呈正相关,回归方程y=1.715-0.005×AT-Ⅲ+0.001×D-D,P=0.000;随病情加重,出生第1天足月儿凝血酶原时间(PT)、部分活化凝血活酶时间(APTT)逐渐延长,AT-Ⅲ水平下降,D-D水平增高,各组间差异有统计学意义(P均＜0.05);出生第1天早产儿AT-Ⅲ水平下降,D-D水平增加,各组间差异有统计学意义(P均＜0.05);相同危重程度各组早产儿D-D值较足月儿增高,极危重组更明显[(1278.2±422.6)μg/L比(321.5±105.2)μg/L,P=0.013].结论 D-D、APTT、AT-Ⅲ和新生儿疾病的严重程度相关,危重新生儿更易发生凝血功能障碍.     

危重新生儿弥散性血管内凝血前期的监测与治疗

目的探讨监测危重新生儿血浆凝血酶原片段F1＋2、凝血酶抗凝血酶复合物（TAT）及D-二聚体等凝血及纤溶因子水平对弥散性血管内凝血前期（pre-DIC）的诊断及治疗意义。方法对NICU收治的96例危重症及36例正常新生儿检测F1＋2、TAT及D-二聚体水平，对30例诊断pre-DIC的患儿予抗凝治疗，并监测治疗后F1＋2、TAT及D-二聚体水平。结果危重症组与对照组相比，F1＋2、TAT、D-二聚体水平均增高，差异有极显著性（P均〈0．01）。TAT对pre-DIC诊断的敏感性较高，F1＋2特异性强；三者在抗凝治疗后均明显下降。F1＋2与TAT、D-二聚体三者之间均呈直线正相关，F1＋2与TAT、D-二聚体的相关系数分别为r=0．70P〈0．01；r=0.42 P〈005；TAT与D-二聚体呈正相关（r=0．35 P〈0．05）；F1＋2、TAT、D-二聚体与危重评分呈直线负相关（r=-0．68、-072、-054P均〈0.01）。结论检测TAT、F1＋2、D-二聚体水平监测危重新生儿的血液高凝状态，有助于pre—DIC的诊断，并可协助判断抗凝治疗的效果。     

全身炎症反应综合征新生儿的凝血功能研究

目的　探讨全身炎症反应综合征 (SIRS)新生儿凝血功能的变化及其临床意义。方法　符合SIRS诊断标准的新生儿 12 6例 ,在入院后 2 4h内进行新生儿危重病例评分 ,并采血测定凝血酶原时间 (PT)、凝血酶时间 (TT)、部分活化凝血活酶时间 (APTT)、D 二聚体 (DD)含量和血小板 (PLT)计数。分别以SIRS符合项数及预后、疾病严重程度分组 ,观察其与凝血功能紊乱间的关系。结果　随着SIRS符合项数的增加 ,SIRS新生儿中危重病例所占百分比及病死率明显增高 ,PT、TT、APTT、DD也增高 ,具有明显的相关性。存活组与死亡组间PT、TT、APTT差异无显著性意义 (P >0 0 5 ) ,死亡组DD显著高于存活组 (P <0 0 1)。危重症组PT、TT、APTT、DD与非危重症组比较 ,差异均具显著性意义 (P<0 0 1)。结论　SIRS新生儿存在凝血机制的活化 ,符合SIRS诊断标准项数越多 ,病情越重 ,凝血功能紊乱越显著 ,病死率越高。     

全身炎症反应综合征患儿血浆抗凝血酶Ⅲ、蛋白C的变化及临床意义

目的 观察全身炎症反应综合征（SIRS）患儿综合治疗前后血浆抗凝血酶-Ⅲ（AT-Ⅲ）、蛋白C（PC）的变化，了解SIRS患儿凝血纤溶系统功能紊乱情况，探讨凝血纤溶系统紊乱在SIRS／MODS（多器官功能障碍综合征）中的作用及其与SIRS／MODS严重程度的关系，为临床判断预后和指导治疗提供理论依据。方法 住院SIRS患儿共43例，分为单纯SIRS组（22例）和合并MODS组（21例）。入院24小时内（初期）、综合治疗三天后（恢复期）、各收集血液标本一次，测定血浆AT-Ⅲ（发色底物法）、PC（ELISA法）指标。正常对照组20例。结果 各组患儿在发病初期，血浆AT-Ⅲ、PC水平下降，两组分别与正常对照组比较有显著差异性（P〈0．01），合并MODS组与单纯SIRS组比较也有显著差异性（P〈0．01）。恢复期，各观察指标均明显好转，分别较发病初期均有显著差异（P均〈0．01）。与正常对照组比较无显著差异性（P〉0．05）。结论 SIRS患儿在发病初期，血浆AT-Ⅲ、PC水平下降；恢复期，各观察指标均明显好转，表明SIRS患儿存在凝血纤溶系统的紊乱，并且这些分子标志物的变化以合并MODS组尤为明显，表明SIRS患儿存在凝血纤溶系统功能异常，在SIRS发展成MODS中具有重要作用。通过这些指标的检测，可以更好地了解SIRS／MODS的病理基础，有助于早期DIC的诊断及判断预后，并为临床采取相应治疗措施，改善预后提供了理论依据和客观指标。     

足月危重新生儿凝血功能的改变及临床意义

目的了解足月危重新生儿体内凝血功能的改变。方法对１８例足月危重新生儿,２０例足月非危重新生儿及２０例正常新生儿作前瞻性研究,在病程极期及恢复期分别测定外周血血管性假血友病因子（ＶＷＦ）,Ｄ－二聚体（Ｄ－Ｄ）,血小板α颗粒膜蛋白（ＧＭＰ－１４０）和可溶性纤维蛋白单体复合物（ＳＦＭＣ）含量的变化。结果疾病组患儿在病程的极期血浆ＶＷＦ,Ｄ－Ｄ,ＧＭＰ－１４０的血浓度及ＳＦＭＣ的阳性率均明显高于正常对照组,而危重组又明显高于非危重组;疾病患儿在恢复期以上四项指标均有明显下降,但危重组仍未达到正常对照组水平。结论患病新生儿体内存在不同程度出凝血功能的变化,危重新生儿尤为明显。     

纤维蛋白相关标志物对重症肺炎患儿DIC前状态的诊断价值

目的探讨纤维蛋白单体(FM)、D-二聚体(D-D)、纤维蛋白(原)降解产物(FDP)3种纤维蛋白相关标志物在重症肺炎患儿弥散性血管内凝血前状态(Pre-DIC)中的诊断价值。方法 213例重症肺炎患儿根据其是否合并Pre-DIC分为Pre-DIC组和病例对照组,另选择40例健康儿童作为正常对照组。分析各组的FM、D-D、FDP、凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)、纤维蛋白原(FIB)、血小板计数(PLT)、血栓调节蛋白(TM)水平,应用受试者工作特征曲线对各指标进行比较和评价。结果三组间除FIB外其余各指标差异均有统计学意义(P均0.05),其中FM、D-D、FDP、APTT三组间两两比较差异均有统计学意义(P均0.01),Pre-DIC组最高,病例对照组次之;Pre DIC组的PT明显高于正常对照组与病例对照组(P0.05),而后两组的PT差异无统计学意义(P0.05);Pre-DIC组和病例对照组的TM和PLT均高于正常对照组(P0.01),但前两组的差异均无统计学意义(P0.05)。各指标中FM、D-D、FDP诊断重症肺炎患儿DIC前状态的曲线下面积较大,分别为0.84、0.76、0.64,三者联合诊断时曲线下面积为0.85。结论纤维蛋白相关标志物FM、D-D、FDP可作为重症肺炎患儿Pre-DIC诊断中有价值的标志物,3项联合检测可提高诊断准确性。     

力欣奇继续医学教育园地——思考病例系列(9)答案

(病例见本刊2011年第9期彩页)该患儿活化部分凝血活酶时间(APTT)时间延长,APTT主要反映内源性凝血是否正常,活化部分凝血活酶:(1)血浆因子Ⅷ,因子Ⅸ和因子XI水平减低,如血友病A、血友病B及因子XI缺乏症;(2)严重的凝血酶原(因子Ⅱ),因子Ⅴ、因子Ⅹ和纤维蛋白原缺乏,如阻塞性黄疽、肝脏疾病、新生儿出血症、肠道灭菌综合征、吸收不良综合征、口服抗凝剂及低(无)纤维蛋白血症等;(3)纤维蛋白溶解活力增强,如继发性、原发性纤维蛋白溶解功能亢进等;(4)     

血浆D-乳酸对小儿胃肠功能障碍评价的研究

目的探讨小儿危重症血浆D-乳酸的变化规律，评价D-乳酸对小儿危重症胃肠功能障碍的诊断价值。方法检测不同危重程度，胃肠功能障碍或衰竭时血浆D-乳酸的水平以及动态监测小儿危重症急性期和恢复期D-乳酸的变化。结果随着危重程度增加，血浆D-乳酸显著上升，不同危重程度组血浆D-乳酸水平相比差异有统计学意义（P〈0．01）；胃肠功能障碍或衰竭时患儿血浆D-乳酸水平与正常对照组及非胃肠功能障碍组比较差异有显著性（P〈0．01）；危重组及极危重组患儿恢复期血浆D-乳酸水平较急性期明显下降，差异有统计学意义（P〈0．01，P〈0．01）。结论血浆D-乳酸可作为小儿危重症胃肠功能障碍或衰竭的诊断指标以及胃肠功能恢复的指标。     

急性感染患儿危重病例评分与凝血功能紊乱的关系

目的 探讨急性感染患儿病情严重度与凝血功能紊乱的关系.方法 将收住人ICU的88例急性感染患儿进行危重病例评分后分为极危重组、危重组和非危重组,所有患儿检测血小板(PLT)、凝血酶原时间(PT)、部分凝血活酶时间(APTT),分析比较三组患儿主要凝血指标的差异.结果 危重组PT、APlT较非危重组延长,极危重组PT、APTT延长更明显(P<0.01).极危重组PLT较非危重组低,而危重组PLT较非危重组高(P<0.01).结论 急性感染患儿危重病例评分与凝血功能紊乱密切相关,随着病情加重,凝血功能紊乱更明显.     

全身炎症反应综合征患儿凝血功能改变及其临床意义的探讨

目的 研究全身炎症反应综合征(SIRS)患儿出凝血系统的功能改变和临床意义及其对预后的影响.方法 采用前瞻性病例对照设计,按照小儿/新生儿SIRS新定义将收住ICU的患儿分为SIRS组(24例)、非SIRS组(21例),另设正常对照组(28例).SIRS组按预后再分为死亡组(10例)和生存组(14例),监测血小板计数(PLT)、凝血酶原时间(PT)、部分凝血酶原时间(APTT)、纤维蛋白原(FBG)、凝血酶-抗凝血酶复合物(TAT)、抗凝血酶Ⅲ(AT-Ⅲ)、蛋白C(PC)、血栓调节蛋白(TM)、D-二聚体(DD)、组织型纤溶酶原激活物(TPA)共10项反映凝血系统功能的指标.结果 (1)SIRS组中PT、APTT、TAT、TM、DD、TPA水平均较非SIRS组及对照组升高(P<0.05);AT-Ⅲ、PC水平均较非SIRS组及对照组降低(P<0.05),非SIRS组PC水平较对照组降低(P<0.05);PLT、FBG水平在SIRS组、非SIRS组及对照组之间比较差异无显著性(P>0.05);(2)SIRS患儿中,死亡组与生存组各出凝血指标间比较差异无显著性(P>0.05).结论 (1)SIRS患儿存在凝血功能异常,主要表现为凝血活化、抗凝活性的降低和纤溶系统的活化;(2)出凝血分子标志物是疾病早期针对性反映患儿凝血系统所处状态的良好指标;(3)分子标志物对预后的意义尚需大样本进行评估.     

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.