急性白血病与血浆纤维结合蛋白的相关性

血浆纤维结合蛋白 (ｆｉｂｒｏｎｅｃｔｉｎ ,简称Ｆｎ)是一族结构相似、免疫原性相同的高分子糖蛋白 ,广泛存在于细胞表面和血浆中 ,血浆中的Ｆｎ是重要的调理蛋白 ,能促进细胞与细胞之间、细胞与基质之间的粘连 ,维护细胞的形状。小儿急性白血病时细胞粘连功能消失。我们检测了小儿急性白血病6 7例 ,并观察了各时期Ｆｎ的不同变化。现报告如下。资料和方法一、对象　 1.患儿组　急性白血病血标本来自本院血液科住院患儿 ,诊断根据临床表现、骨髓涂片瑞氏染色及细胞化学染色 ,按ＦＡＢ分型 ,6 7例中男 40例 ,女 2 7例 ,年龄 6个月～ 14ａ。…     

血清SIL-2R在急性白血病病程中的变化

采用ＥＬＩＳＡＭ抗体夹心法检测４０例不同类型的急性白血病患者ＳＩＬ２Ｒ，并动态观察了在化疗过程中发生各种感染时ＳＩＬ－２Ｒ的变化，以及不同疗效转归患者的ＳＩＬ－２Ｒ的变化情况。结果发现：急性白血病ＳＩＬ－２Ｒ水平显著高于正常人（ｎ＝２５），高危组白血病ＳＩＬ－２Ｒ升高更为显著，在化疗过程中发生感染时ＳＩＬ－２Ｒ进一步升高，感染越严重升高越显著；不同疗效的患者ＳＩＬ－２Ｒ恢复程度不一致，完全缓解者已接近正常水平，部分缓解者虽然有所下降但与完全缓解者的下降有显著差别。这些结果提示ＳＩＬ－２Ｒ监测对判断急性白血病病情、了解预后，有一定临床价值。     

急性白血病儿脑脊液SDS—PAGE检测的应用：附53例观察

本文应用ＳＤＳ－ＰＡＧＥ方法对５３例急性白血病（ＡＬ）患儿８１分脑脊液（ＣＳＦ）蛋白进行测定，发现ＣＳＦ蛋白含量的的变化与白血病的化疗似有密切的关系。即ＣＳＦ蛋白总量随病情缓解而升高，随病情恶化而降低。本研究还发现蛋白总量的改变早于骨髓复发之前，此结果可以比较直观地指导临床医师疗效、监测病情及估计预后。本试验方法简便、快速、材料易得，可用于临床作评判白血病患儿化疗动态反应的指标。     

脑脊液肿瘤坏死因子测定在中枢神经系统白血病的临床意义

本文检测１００例正常人，急性白血病患儿脑脊液中肿瘤坏死因子（ＴＮＦ），并对已发生中枢神经系统白血病（ＣＮＳＬ）的１０例患儿进行动态监测，测得正常儿童脑脊液（ＣＳＦ）中ＴＮＦ为８．８６±０．８０ｎｇ／ｍｌ，无发生ＣＮＳＬ儿童ＣＳＦ中ＴＮＴ为９．３２±１．５２ｎｇ／ｍｌ，而发生ＣＮＳＬ儿童ＣＳＦ中ＴＮＴ显著升高达２０．６１±２．２１ｎｇ／ｍｌ，治疗缓解后下降为９．１６±０．７６ｎｇ／ｍｌ。提示ＴＮＦ检测对临床观察ＣＮＳＬ的发生发展与转归有一定价值。     

Daxx蛋白在儿童急性白血病中的表达及其临床意义

目的：通过检测Daxx蛋白在儿童急性白血病骨髓细胞中的表达，探讨其与急性白血病临床分型和预后的关系，为进一步研究抗白血病治疗新靶点提供思路。方法：应用免疫组织化学链霉素亲和素-生物素-过氧化酶复合物（SABC）方法检测50例儿童急性白血病骨髓细胞Daxx蛋白的表达，对照组为20例非恶性血液病且骨髓正常患儿。结果：在50例急性白血病患儿骨髓细胞中，Daxx蛋白阳性表达率为38.0%，显著高于正常骨髓组织（P<0.05）；Daxx在急性非淋巴细胞白血病患儿骨髓细胞中的阳性表达率为62.5%，显著高于正常骨髓组织（P<0.05）和急性淋巴细胞白血病患儿（P<0.05）；Daxx在急性淋巴细胞白血病患儿骨髓细胞中的阳性表达率为26.5%，与正常骨髓组织比较差异无显著性（P>0.05）； Daxx在高危（HR）、标危（SR）急性淋巴细胞白血病患儿骨髓细胞中的阳性表达率为55.6%和0，两者比较差异有显著性（P<0.05）。结论：Daxx蛋白在儿童急性白血病中异常表达，并且与儿童急性白血病的某些临床特征密切相关，可能在儿童急性白血病的发生发展过程中起重要的作用。 ［中国当代儿科杂志，2007，9（1）：33-36］     

急性白血病患儿血清肿瘤坏死因子a的活性变化及临床意义

用放射免疫方法测定了２０例正常儿童及４０例急性白血病（ＡＬ）患儿化疗前后血清肿瘤坏死因子（ＴＮＦ－ａ）浓度。结果：在ＡＬ诊断时，ＴＮＦ－ａ水平显著升高；当患儿获得部分缓解至完全缓解时，ＴＮＦ－ａ水平呈梯形降低，其最低值仍高于健康儿童（Ｐ〈０．０１￣０．０５）；２２例急性淋巴细胞白血病（ＡＬＬ）患儿ＴＮＦ－ａ水平在任何阶段都比１８例急性非淋巴细胞白血病（ＡＮＬＬ）患儿低（Ｐ〈０．０１）；ＴＮＦ－ａ水     

血清SIL—2R在急性白血病病程中的变化

采用ＥＬＩＳＡ双抗体夹心法检测４０例不同类型的急性白血病患者ＳＩＬ２Ｒ，并动态观察了在化疗过程中发生各种感染时ＳＩＬ－２Ｒ的变化，以及不同疗效转归患者的ＳＩＬ－２Ｒ的变化情况。结果发现：急性白血病ＳＩＬ－２Ｒ水平显著高于正常人（ｎ＝２５），高危组白血病ＳＩＬ－２Ｒ升高更为显著，在化疗过程中发生感染时ＳＩＬ－２Ｒ进一步升高，感染越严重升高越显著；不同疗效的患者ＳＩＬ－２Ｒ恢复程度不一致，完全缓解者已     

中枢神经系统白血病脑脊液纤维结合蛋白和中分子物质的变化

中枢神经系统白血病脑脊液纤维结合蛋白和中分子物质的变化福建医学院附属协和医院儿科（３５０００１）李建，郭瑞官，沈建箴对２０例中枢神经系统白血病（ＣＮＳＬ）患儿的脑脊液进行纤维结合蛋白（ＦＮ）和中分子物质（ＭＭＳ）检测，并动态观察治疗后的含量变化，以探...     

儿童白血病脑脊液P^53蛋白和ras癌基因蛋白表达及临床意义探讨

为探讨Ｐ＾５３蛋白和ｒａｓ癌基因蛋白在白血病儿童脑脊液（ＣＳＦ）中的表达水平及与白血病化疗的关系，应用Ｗｅｓｔｅｒｎ斑点印迹法检测５４例儿童急性白血病ＣＳＦ中Ｐ＾５３蛋白和ｒａｓ癌基因蛋白表达水平。结果显示，白血病儿童危象期ＣＳＦ中Ｐ＾５３蛋同于非肿瘤对照水平。缓解期Ｐ＾５３蛋白和ｒａｓ癌基因蛋白与危象期比有增高趋势，同时高于非肿瘤对照水平，白血病儿童ＣＳＦ治疗有增高趋势，同时高于非肿瘤对照水平，     

脑脊液肿瘤坏死因子测定在中枢神经系统白血病的临床意义

本文检测１００例正常人，急性白血病患儿脑脊液中肿瘤坏死因子（ＴＮＦ），并对已发生中枢神经系统白血病（ＣＮＳＬ）的１０例患儿进行动态监测，测得正常儿童脑脊液（ＣＳＦ）中ＴＮＦ为８．８６±０．８０ｎｇ／ｍｌ，无发生ＣＮＳＬ儿童ＣＳＦ中ＴＮＴ为９．３２±１．５２ｎｇ／ｍｌ，而发生ＣＮＳＬ儿童ＣＳＦ中ＴＮＴ显著升高达２０．６１±２．２１ｎｇ／ｍｌ，治疗缓解后下降为９．１６±０．７６ｎｇ／ｍｌ。提示ＴＮＦ检     

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.