儿童急性白血病血清白细胞介素24的测定及意义

目的观察急性白血病患儿血清白细胞介素24(IL-24)的水平及意义。方法确诊未治的儿童急性淋巴细胞白血病(ALL)、儿童急性髓细胞白血病(AML)、非白血病患儿及正常儿童(正常对照组)各20例,采用ELISA法测定其血清IL-24的水平并进行比较。结果两组白血病患儿血清IL-24浓度[ALL组:(28.25±2.6)ng/L;AML组:(26.32±3.2)ng/L]均较正常对照组[(113.15±3.7)ng/L]和非白血病患儿[(105.82±4.2)ng/L]明显降低(P值均0.05);但ALL与AML组之间和非白血病组与正常对照组之间差异无统计学意义(P值均0.05)。结论 ALL及AML患儿血清IL-24浓度明显下降。     

急性白血病患儿血清白细胞介素—3的变化

目的　探讨急性白血病 (AL)血清白细胞介素 3(IL 3)的变化及意义。方法　采用双抗体夹心酶联免疫吸附法检测血清IL 3。结果　急性淋巴细胞白血病 (ALL)初治组为 195 .83± 10 8.2 5 pg/ml,急性髓性白血病 (AML)初治组 5 0 3.18± 193.0 1pg/ml,正常对照组 192 .5± 93.2 5pg/ml。ALL初治组与正常对照组比较无显著差异 (P >0 .0 5 ) ,AML初治组显著高于正常组 (P <0 .0 1)和ALL初治组 (P <0 .0 1) ;4周化疗完全缓解(CR)组ALL为 184.6 4± 75 .92 pg/ml,与初治组无差异 (P >0 .0 5 )。CR组AML为 393.0 0± 117.77pg/ml,与初治组无差异 (P >0 .0 5 ) ;ALL长期缓解组为 175 .77± 91.5 6pg/ml,与 4周缓解组及正常对照组比较均无差异(P >0 .0 5 )。结论　血清IL 3可能与ALL的发病无明显相关 ,而参与了AML的病理生理及疾病过程     

c-kit受体在急性白血病患儿中表达的意义

目的 探讨c-kit受体(c-kit R,CD117)在急性白血病(AL)患儿中的表达,了解c-kit R表达在急性非淋巴细胞白血病(ANLL)中的诊断价值及其与ANLL的临床和生物学特征的关系.方法 采用流式细胞术(FCM)分别检测28例急性淋巴细胞白血病(ALL)和37例ANLL患儿骨髓单个核细胞(BMMNC)跨膜蛋白c-kit R 的表达;反转录聚合酶链反应(RT-PCR)检测儿童AL中c kit R mRNA水平,并与非恶性血液病患儿36例BMMNC比较.结果 ALL患儿BMMNC中c-kit R表达率及其mRNA表达水平与对照组比较均无显著性差异(Pa＞0.05),而ANLL患儿的c-kit R表达率及其mRNA表达水平则均显著高于对照组和ALL患儿(x2=40.75,44.20 Pa＜0.01).ANLL患儿c-kit R表达率及其mRNA表达水平与外周血白细胞计数及肝脾大均无关(x2=0.011,0.084Pa>0.05),与其血清LDH水平增高有关(x2=6.257 P＜0.05).结论 c-kit R可作为AL患儿MIC分型诊断的髓系免疫表型依据,可用于鉴别髓系白血病.     

支气管哮喘患儿血清褪黑素水平检测的意义

目的研究不同病情支气管哮喘患儿血清褪黑素水平变化,探讨影响哮喘患儿血清褪黑素水平变化的因素。方法收集哮喘患儿及健康儿童血清样品75例。其中轻度、中度、重度发作期组及临床缓解期组患儿各15例;健康对照组15例。应用酶联免疫吸附法(ELISA)测其血清样品褪黑素水平。结果血清褪黑素水平轻度发作组[(22.76±5.16)ng/L],中度发作组[(16.79±3.35)ng/L],重度发作组[(11.54±1.45)ng/L],缓解期组[(22.06±3.36)ng/L],对照组[(28.72±4.32)ng/L],5组间进行多重比较,除缓解期组与轻度发作组无显著差异外,余各组间均有显著差异(Pa<0.05);随着病情加重,褪黑素的水平渐降低。结论哮喘患儿血清褪黑素水平明显降低,可能与患儿睡眠紊乱、机体处于应激状态时体内高水平皮质激素对松果腺的抑制有关。     

IL-13、SccAg与毛细支气管炎关系的研究

目的　探讨白细胞介素 13(IL 13)、鳞状细胞癌抗原 (SccAg)及免疫球蛋白E(IgE)与毛细支气管炎 (简称毛支 )发病机制的关系。方法　用ELISA法检测 36例毛支患儿、2 6例哮喘患儿、4 0例肺炎患儿及 33例正常儿童血清IL 13、SccAg及IgE水平 ,并对结果进行统计学处理。 结果　 (1)毛支患儿发作期血清IL 13(10 4 91± 18 0 5 )ng/L及SccAg(2 4 9± 0 38)ng/ml水平显著高于缓解期(85 15± 17 98)ng/L ,(2 30± 0 34)ng/ml及正常对照组 (77 2 7± 18 16 )ng/L ,(2 2 9± 0 34)ng/ml(P<0 0 5 ) ,而缓解期与正常对照组间无显著性差异 (P >0 0 5 )。 (2 )毛支发作期患儿血清IgE水平(370 91± 6 9 2 6 )kU/L显著高于缓解期 (189 4 6± 70 36 )kU/L(P <0 0 5 ) ,两组均显著高于正常对照组 (15 1 6 6± 70 17)kU/L(P <0 0 5 )。 (3)毛支发作期患儿血清IL 13、SccAg及IgE水平显著低于哮喘发作期 (14 7 0 0± 2 3 78)ng/L ,(3 0 1± 0 37)ng/ml,(6 5 9 5 2± 70 5 1)kU/L(P <0 0 0 1)。 (4 )毛支患儿发作期血清IL 13、SccAg及IgE水平显著高于肺炎组 (80 74± 18 0 8)ng/L ,(2 31± 0 35 )ng/ml,(15 2 87± 6 6 91)kU/L(P <0 0 5 )。 (5 )毛支患儿发作期血清IL 13水平与SccAg、IgE及SccAg与IgE水     

急性白血病患儿血清转铁蛋白受体检测及意义

目的　探讨急性白血病患儿血清转铁蛋白受体 (sTfR)的变化及其临床意义。方法　采用双抗夹心酶联免疫吸附法测定sTfR。结果　急性淋巴细胞白血病 (ALL)初治组 (2 4例 ) 2 4 .73± 1 2 .38nmol/L ,急性髓性白血病 (AML)初治组 (1 1例 ) 2 7.0 9± 1 9.37nmol/L ,正常对照组 (2 0例 ) 30 .49± 9.78nmol/L。三者无显著差异 (P >0 .0 5) ;经 4周化疗ALL完全缓解组 (1 4例 )为 55 .41± 2 2 .0 1nmol/L ,AML(5例 )为 50 .71± 2 0 .99nmol/L ,显著高于初治组及正常组 (P <0 .0 1 ) ;第 8周末ALL(1 0例 )为 33 .0 7± 1 4 .2 7nmol/L ,AML(5例 )为30 .99± 1 2 .90nmol/L ,接近正常水平 (P >0 .0 5) ;ALL长期缓解组 (1 3例 )为 33 .0 7± 1 6 .1 1nmol/L ,与正常对照组无差异 (P >0 .0 5)。结论　sTfR在小儿急性白血病治疗前后有显著变化 ,对诊断及治疗效果的评价有一定参考意义。     

急性白血病患儿血浆内皮素变化及其临床意义的探讨

目的　 探讨AL患儿血浆ET变化的临床意义。方法 　采用放射免疫法检测2 0例健康儿童和 41例急性白血病 (AL)患儿血浆内皮素 (ET)水平。结果 　急性白血病发病组血浆ET平均值为 95 .15ng/L ,明显高于健康儿童对照组 3 9.95ng/L(P <0 .0 1)。经化疗缓解后血浆ET平均值降至正常水平 (3 8.3 0g/L) ,明显低于未缓解组血浆ET平均值 95 .15ng/L(P <0 .0 1)。AL患儿血浆ET值与骨髓白血病细胞数量呈正相关 (r=0 .93 89,P <0 .0 1) ,结论 　动态观察患儿血浆ET水平对判断急性白血病的病情与预后有重要意义。     

儿童急性白血病患者血浆止凝血分子标志物检测的临床意义

目的　了解三种止凝血分子标志物在不同类型 ,不同出血程度的儿童急性白血病 (AL)患者中的变化并探讨其临床意义。方法　用ElISA方法检测了 56例初诊AL患儿血浆中的凝血酶调节蛋白 (TM) ,可溶性纤维蛋白单体复合物 (SFMC) ,D-二聚体 (D -D)含量 ,并用常规方法检测了 56例AL患儿的血小板 (BPC)、血浆凝血酶原时间 (PT)、活化部分凝血活酶时间 (APTT)、凝血酶时间 (TT)、鱼精蛋白副凝试验( 3P)及纤维蛋白原定量 (Fg)。结果　 56例AL患儿的SFMC、D -D均值高于正常对照组儿童的均值 ,两组比较有显著差异 (P <0 0 5)。急性非淋巴细胞白血病 (ANLL)与急性淋巴细胞白血病 (ALL)比较 ,ANLL患儿组的SFMC、D -D的血浆含量显著高于ALL组。高白细胞性急性白血病 (HLAL)与其它初诊时白细胞数低于 1 0 0× 1 0 9/L的AL患儿比较 ,TM、SFMC、D -D均有显著差异 (P <0 0 5)。无出血组与正常对照比较 ,SFMC、D -D及BPC有显著差异 (P <0 0 5) ,其它项目无显著差异 ,轻度出血组与无出血组比较 ,BPC减低有显著差异 (P <0 0 5) ,而其它项目无显著差异 ,重度出血组与轻度出血组相比较 ,TM、SFMC、D -D、3P试验、Fg均有显著差异。结论　 1、儿童AL患儿存在不同程度凝血、纤溶紊乱 ,以HLAL患儿最严重。 2、血管内皮细胞受损 ,?     

P糖蛋白表达在儿童急性白血病中的意义

为探讨儿童急性白血病 (AL)P糖蛋白 (P gp)表达与临床、免疫分型及预后。运用单克隆抗体UIC2 及流式细胞仪 (FCM)间接免疫荧光法对不同时期儿童AL的P gp表达进行检测。结果 :4 5例初治急性淋巴细胞性白血病 (ALL)P gp表达率8 89% ,1 2例复发ALL阳性率为 2 5% ,完全缓解 (CR)组为 0。 32例初治急性非淋巴细胞白血病 (ANLL)P gp表达率为 1 8 75% ,6例复发ANLL 33 3% ,CR组为 0。初治ANLL患儿P gp表达与CD34 、CD7高度相关 ,初治ALL与CD抗原无明显相关性。P gp+ANLL的CR率 2 0 % ,明显低于P gp ANLL( 83 3% ) ,P gp+ALL的CR率 ( 75% )与P gp ALL的CR率 ( 92 86 % )相近 (P >0 0 5) ,但复发率高。结果表明 :P gp高表达是导致儿童AL化疗耐药的重要机制 ,检测P gp是判断预后 ,指导治疗的有用指标。     

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目的探讨细胞周期蛋白D3、E的表达与儿童急性白血病(AL)的关系。方法采用免疫组织化学法,对广西医科大学第一附属医院儿科2002年1月至2004年5月收治的50例初发或复发儿童AL,其中急性淋巴细胞白血病(ALL)30例(高危ALL12例,标危ALL18例),急性非淋巴细胞白血病(ANLL)20例,和26例完全缓解AL(AL CR)患儿,以及23例同期住院的非恶性疾病患儿(对照组),检测其骨髓细胞中细胞周期蛋白D3、E的表达情况。结果AL组中细胞周期蛋白D3、E阳性率分别为54%和46%,高于AL CR组和对照组(P<0.01);在ALL和ANLL间差异无显著性(P>0.05)。高危ALL细胞周期蛋白D3阳性率明显高于标危ALL(P<0.01)。化疗前细胞周期蛋白D3、E表达阳性率明显高于化疗缓解后(P<0.01)。细胞周期蛋白D3、E在AL组中的表达呈正相关(r=0.5298,P<0.01)。结论细胞周期蛋白D3、E与儿童AL发病有关,它们的表达存在相关性,可作为疗效观察的指标;细胞周期蛋白D3可能与患儿不良预后有关。     

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.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

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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.