早产儿D-二聚体和纤溶酶原的动态变化研究

目的观察不同日龄正常早产儿纤溶活性指标栓溶二聚体(D-二聚体,DD)和纤维蛋白溶酶原(纤溶酶原,PLG)生理水平及其变化规律。方法选取日龄1、5、10、20 d的早产儿各40 例及1、5、10,20 d正常足月儿各20例作对照,观察其纤溶指标DD、PLG值的日龄变化规律。结果早产儿和足月儿DD阳性在新生儿期较多见,尤其是在生后1周内,DD阳性率随日龄增长而减低,早产儿减低较慢。早产儿PLG在第1、5 d与足月儿比较差异无显著性(P>0．05),在第10、20 d与足月儿比较差异有显著性(32．00±11．82)％比(41．30±9．74)％,(27．58±9．04)％比(43．65 ±17．38)％,(P<0．05)。早产儿PLG随着日龄增长有下降趋势,而足月儿无明显变化。结论新生儿出生后DD阳性率高和较低活性PLG水平提示纤溶活性的增强,尤其是早产儿更明显。对新生儿特别是早产儿临床出现DD阳性或PLG降低时,对DIC诊断要慎重。     

胎龄和体重及缺氧对新生儿纤溶活性的影响

目的　探讨正常新生儿的纤溶活性状态及胎龄、体重、缺氧对纤溶活性的影响 ,并分析新生儿出血的可能原因及风险因素。方法　对不同胎龄、不同出生体重正常新生儿以及出生窒息足月儿的纤溶酶原活性 (P1g)、组织型纤溶酶原激活物活性 (t PA)、纤溶酶原激活物抑制物活性 (PAI)及D 二聚体含量 (D dimer)进行检测。结果　正常足月新生儿P1g为 (2 84± 0 0 6)IU/ml,t PA为 (0 2 4±0 11)IU/ml,PAI为 (0 64± 0 3 7)AU/ml,PAI/t PA比值为 2 6± 2 0 ,D dimer为 (3 7± 4 7)mg/L ,表现出较宽的取值范围。无合并症早产儿P1g活性为 (2 16± 0 5 2 )IU/ml,D dimer含量为 (1 0± 0 7)mg/L ,PAI/t PA比值为 3 9± 2 6;与正常足月儿相比 ,P1g活性和D dimer含量显著降低 (P <0 0 1) ,PAI/t PA比值显著增高 (P <0 0 5 ) ;窒息足月儿与无合并症早产儿相似 ,P1g活性、D dimer含量为 (1 2 0±0 85 )mg/L ;与足月儿相比 ,P1g活性和D dimer含量亦显著降低 ,而PAI/t PA比值显著增高 ,出生后窒息时间与D dimer含量呈显著正相关 (r=0 5 96,P <0 0 1) ;P1g活性、胎龄、出生体重与D dimer含量均呈显著正相关。结论　正常足月儿纤溶活性有较大个体差异 ;纤溶活性的改变和胎龄、出生体重、缺氧密切相关。纤溶活性     

全身炎症反应综合征患儿凝血纤溶变化与病情危重度的关系

目的 探讨不同程度全身炎症反应综合征(SIRS)患儿凝血纤溶指标的变化及与病情危重程度的关系.方法 按危重症评分将患儿分为SIRS1(≥90分)、SIRS2(80～90分)及SIRS3(≤80分)3组,按预后分为存活组、死亡组;于急性期应用发色底物法测定抗凝血酶(AT-Ⅲ)、纤维蛋白溶酶原(PLG)活性,ELISA法测定D-二聚体(D-dimer,DD)、组织纤溶酶原激活物(t-PA)、纤溶酶原激活物抑制物(PAI-1)含量并与对照组进行比较.结果 SIRS患儿急性期血浆DD及PAI-1含量均增高,AT-Ⅲ、PLG、t-PA含量降低,随着危重症评分的减少,变化程度更显著;死亡组与存活组相比差异有统计学意义.DD及PAI-1含量与危重症评分呈负相关.AT-Ⅲ、PLG、t-PA含量与危重症评分呈正相关.结论 AT-Ⅲ、DD、PLG、t-PA、PAI-1水平可反映SIRS患儿体内凝血纤溶紊乱程度,并与病情的危重程度密切相关.     

健康新生儿血二胺氧化酶、D-乳酸测定及意义

目的探讨健康新生儿血二胺氧化酶(DAO)、D-乳酸的正常含量,为新生儿胃肠功能衰竭的早期诊断寻找可靠的检验依据.方法对79例健康新生儿(足月儿59例,早产儿20例)进行血清DAO、D-乳酸含量测定.结果血清DAO为(8.851±3.424)u/ml,血D-乳酸为(0.918±0.715)ug/ml,血清DAO含量在足月儿与早产儿、在性别之间比较无差异,在日龄之间比较＜3天组与～7天及晚期新生儿各组比较差异无显著性,P值均＞0.05,～7天组与晚期新生儿各组比较有显著性差异,P值均＜0.05.D-乳酸含量在性别之间差异无显著性(P＞0.05),但在早产儿与足月儿、＜3 d的早期新生儿与＞14 d的晚期新生儿之间的比较差异有显著性(P＜0.05).结论血DAO不受性别、孕周的影响,血D-乳酸在胎龄、日龄有差别.     

Changes of Fibrinolysis and Their Relationship with Pulmonary Artery Pressures in Neonates with Lung Diseases

目的　研究新生儿肺疾病的纤溶改变及其与肺动脉压力的关系。方法　用发色底物法测定 2 7例新生儿肺疾病患儿 (病例组 )及 2 5例正常新生儿 (对照组 )血浆组织纤溶酶原激活剂 (TPA)和纤溶酶原激活抑制物(PAI)活性变化 ;用超声多普勒方法测定肺动脉血流加速时间 (TPV)与右室射血时间 (RVET)之比值 (TPV/RVET) ,以此估计新生儿肺动脉压力 (TPV/RVET比值与肺动脉压力成反比 )。结果　病例组PAI活性 [(9.3±4 .1)AU× 10 -1/ml]明显高于对照组 [(5 .5± 3.0 )AU× 10 -1/ml],P <0 .0 1,TPV/RVET比值 (0 .2 9± 0 .0 5 )明显低于对照组 (0 .34± 0 .0 8) ,(P <0 .0 5 ) ;发生肺出血者肺动脉压力 [TPV/RVET (0 .2 3± 0 .0 2 ) ]显著升高 ,P <0 .0 5。肺出血恢复期新生儿TPA [(3.7± 1.7)IU× 10 -1/ml]及血小板 [(180± 30 )× 10 9/L]明显高于肺出血发生时 [TPA (1.8± 0 .7)IU× 10 -1/ml,血小板 (98± 39)× 10 9/L],(P <0 .0 1)。结论　新生儿肺疾病时由于肺动脉压力增高 ,肺血管内皮细胞破坏 ,导致TPA释放减少 ,PAI活性增高 ,纤溶活性降低。     

健康新生儿血二胺氧化酶、D-乳酸测定及意义

目的探讨健康新生儿血二胺氧化酶(DAO)、D-乳酸的正常含量,为新生儿胃肠功能衰竭的早期诊断寻找可靠的检验依据。方法对79例健康新生儿(足月儿59例,早产儿20例)进行血清DAO、D-乳酸含量测定。结果血清DAO为(8.851±3.424)u/ml,血D-乳酸为(0.918±0.715)ug/ml,血清DAO含量在足月儿与早产儿、在性别之间比较无差异,在日龄之间比较:<3天组与-7天及晚期新生儿各组比较差异无显著性,P值均>0.05,-7天组与晚期新生儿各组比较有显著性差异,P值均<0.05。D-乳酸含量在性别之间差异无显著性(P>0.05),但在早产儿与足月儿、<3d的早期新生儿与>14d的晚期新生儿之间的比较差异有显著性(P<0.05)。结论血DAO不受性别、孕周的影响,血D-乳酸在胎龄、日龄有差别。     

新生儿呼吸窘迫综合征抗凝及纤溶指标变化的临床意义

目的　探讨新生儿呼吸窘迫综合征 (NRDS)抗凝和纤溶系统变化及其临床意义。方法　采用ELISA法和免疫浊度法分别测定普通早产儿 2 0例、NRDS患儿 2 7例和健康足月儿 15例血浆蛋白C(PC) ,总蛋白S(TPS)、抗凝血酶Ⅲ (AT Ⅲ )、D 二聚体 (D D)、血管性假性血友病因子 (vWF)水平。结果　NRDS患儿血浆PC、TPS、AT Ⅲ低于普通早产儿和健康足月儿 ,D D、vWF明显高于普通早产儿和健康足月儿 ,普通早产儿PC、TPS低于足月儿 ,AT Ⅲ、D D、vWF则无明显差异。结论　NRDS患儿存在抗凝及纤溶系统的激活及血管内皮细胞损伤 ,上述指标是观察NRDS患儿早期DIC敏感指标之一     

抗凝血酶和纤溶功能的变化在窒息新生儿中的临床意义

新生儿窒息的病理生理基础和本质为缺氧、酸中毒眼1演,缺氧、酸中毒引起的凝血功能紊乱又加重组织缺氧缺血,严重者可导致感染、休克、多器官功能衰竭甚至死亡眼2,3演,故凝血机制在新生儿窒息发生发展及预后中起重要作用眼4演。本研究拟测定窒息患儿的纤维蛋白原(FIB)、抗凝血酶(A T∶A)、D-二聚体(D蛳D)、Ⅷ因子活性(Ⅷ∶C)、血管性血友病因子(V W F)、纤溶酶原活性(PLG∶A)等指标,以探讨抗凝血酶和纤溶变化与病情严重程度及预后关系。1材料和方法1.1研究对象①随机选取2002～2004年入院的窒息新生儿82例,采用A pgar评分标准,分为轻…     

新生儿胃肠穿孔80例临床特点及预后分析

目的分析新生儿胃肠穿孔的病因、临床特征、治疗及预后。方法回顾性分析2004年1月至2015年12月复旦大学附属儿科医院新生儿科收治的80例新生儿胃肠穿孔患儿的临床资料,根据出生胎龄分为早产儿组与足月儿组,比较两组间的病因、临床表现、治疗及预后。结果 80例新生儿胃肠穿孔中,早产儿62例,足月儿18例。两组病因均以坏死性小肠结肠炎(necrotizing enterocolitis,NEC)为主,临床均以腹胀为主要表现,早产儿发生反应差、休克、弥散性血管内凝血明显多于足月儿(P0.05)。早产儿组平均发病日龄9(1.75,20)d,足月儿组平均发病日龄4.5(1,7.75)d。62例手术治疗,其中胃穿孔8例,肠穿孔54例,18例因未手术穿孔部位不明确。死亡32例,病死率40%,早产儿死亡26例(病死率41.9%),足月儿死亡6例(病死率33.3%)。结论新生儿胃肠穿孔是新生儿期的严重疾病,病死率高。早期诊断、积极治疗和尽早外科干预可能提高患儿的存活率,显著改善患儿的预后。     

不同日龄新生儿凝血指标的临床研究

目的 探讨不同日龄新生儿凝血指标的变化及其临床意义.方法 以我院2005年1至12月129例生后无合并症新生儿为研究对象,按胎龄分为足月儿组和早产儿组,分别于生后1、3、10 d采血测定凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)、纤维蛋白原(Fg)、凝血酶时间(TT)及D-二聚体含量.结果 在出生后1、3、10 d三个不同日龄,早产儿和足月儿两组间的PT、D-二聚体差异均无显著性(P>0.05),但早产儿组的APTT、TT在三个不同的日龄均高于足月儿,Fg低于足月儿,差别均有显著性(P<0.05).同时随着新生儿目龄的增加,PT、APTT,TT、、D-二聚体减少,而Fg增加,三个时间点凝血指标的差别均有显著性(P<0.05).结论 胎龄、日龄对新生儿凝血指标均有影响,为临床诊断和治疗提供了一定的参考依据.     

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   

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.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.     

Psychopathologie du syndrome d’Asperger et « aspérigisation » de la société contemporaine

The author has attempted here to point out, just for a start, the characteristics of Asperger syndrome from the point of view of psychopathology through a rereading of Hans Asperger's original paper (1944). This thesis merits reevaluation, if for no other reason than to fill the gaps in operational diagnostics based on the DSM. It is found by rereading that Asperger's view of the principal disturbances of autistic psychopathy include a “disturbance of natural evidence” or a “crisis of common sense”. This question of natural evidence that he evokes with regard to autistic psychopathy corresponds to W. Blankenburg's natural evidence, which constitutes a key concept for comprehending schizophrenia in the form poor-symptom (“symptomarme Schizophrenie”) that he observes in the speech of his patient Anne Rau. One can deduce from this that in terms of fundamental disturbances, Asperger syndrome and this “symptom-poor” schizophrenia overlap at the level of loss of natural evidence. It is moreover possible to classify Asperger syndrome among the disturbances of spacing in the sense meant by the evolutionary psychiatry of A. Stevens and J. Price. The author then develops our comprehension of Asperger syndrome from the point of view of the perspective proposed by the notion of resilience in people with Asperger syndrome and of the possibility for them, through these mechanisms of adaptation, to find in the organization of the personality of the “as if” type a position of relative equilibrium. They concur or overlap in the creation of crutches, of borrowed personalities secondarily legitimated by the reaction of the socius. This will end up in the production of inventions and œuvres (works). Clearly, one rarely encounters several cases that one could consider pertinently to be “successful” Asperger syndrome. Finally, the author notes that one can find a sort of isomorphism between Asperger syndrome and contemporary society when he proposes the term “asperigisation” to characterize our society, given that the equilibrium between emotion and logic is strongly disturbed in these patients, in whom logic undergoes hypertrophy while emotion is impoverished. From this perspective, the author hopes to suggest reasons for the increase in the number of cases of Asperger syndrome in the clinical setting and in society in general in our contemporary era.     

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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     

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.     

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.