紫癜性肾炎中尿系列微量蛋白及相关因素分析

目的 了解紫癜性肾炎(HSPN)临床各型尿4项特定蛋白排量高低，以早期发现其各型肾功能受损程度。并分析各型间的临床临床及其之间的相关因素。方法 首先对入选的85例HSPN患儿按照特点表现分为5型：其中Ⅰ型20例、Ⅱ型20例、Ⅲ型15例、Ⅳ型15例、Ⅴ型15例，采用免疫散射速率比浊法，对上述85例患儿及25例健康儿童(正常对照组)分别进行尿微量自蛋白(mALb)、转铁蛋白(uTf)、免疫球蛋白(IgG)、α1-微球蛋白(α1-mG)含量测定，同时检测血肌酐(BCr)和尿素氮(BUN)；最后观察HSPN临床各型尿4项微量蛋白的不同变化。结果 在Ⅰ、Ⅱ型HSPN中，当BUN与BCr含量在正常范围时，尿4项特定微量蛋白排量已出现了不同程度的升高，与对照组比较差异显著；在Ⅲ～Ⅴ型HSPN中。当BUN与BCr有明显升高时，尿微量蛋白排出量升高更为显著，两者呈线性正相关。结论 尿特定蛋白可全面地、敏感地反映肾脏的受损情况，尤其是肾小管的损害，故此检测方法可作为评价肾功能早期损害、判断预后、监测治疗效果的有效指标。     

紫癜性肾炎中尿系列微量蛋白及相关因素分析

目的　了解紫癜性肾炎 (HSPN)临床各型尿 4项特定蛋白排量高低 ,以早期发现其各型肾功能受损程度 ,并分析各型间的临床临床及其之间的相关因素。方法　首先对入选的 85例HSPN患儿按照特点表现分为 5型 :其中Ⅰ型2 0例、Ⅱ型 2 0例、Ⅲ型 15例、Ⅳ型 15例、Ⅴ型 15例 ,采用免疫散射速率比浊法 ,对上述 85例患儿及 2 5例健康儿童 (正常对照组 )分别进行尿微量白蛋白 (mALb)、转铁蛋白 (uTf)、免疫球蛋白 (IgG)、α1 微球蛋白 (α1 mG)含量测定 ,同时检测血肌酐 (BCr)和尿素氮 (BUN) ;最后观察HSPN临床各型尿 4项微量蛋白的不同变化。结果　在Ⅰ、Ⅱ型HSPN中 ,当BUN与BCr含量在正常范围时 ,尿 4项特定微量蛋白排量已出现了不同程度的升高 ,与对照组比较差异显著 ;在Ⅲ～Ⅴ型HSPN中 ,当BUN与BCr有明显升高时 ,尿微量蛋白排出量升高更为显著 ,两者呈线性正相关。结论　尿特定蛋白可全面地、敏感地反映肾脏的受损情况 ,尤其是肾小管的损害 ,故此检测方法可作为评价肾功能早期损害、判断预后、监测治疗效果的有效指标。     

紫癜性肾炎患儿血清基质金属蛋白酶-2、-9及其组织抑制因子-1水平及比值变化的意义

目的探讨基质金属蛋白酶-2、-9(MMP-2、-9)及其组织抑制因子-1(TIMP-1)水平及比值变化在紫癜性肾炎(HSPN)发病机制中作用及与尿微量清蛋白(MA)的相关性。方法采用双抗体夹心ABC-ELISA法对36例单纯紫瘢、16例HSPN患儿进行血清MMP-2、-9和TIMP-1水平检测,免疫散射速率比浊法进行尿MA水平检测。与30例健康儿童对照。结果过敏性紫癜(HSP)患儿急性期血清MMP-2、-9和TIMP-1水平及MMP-2、-9/TIMP-1比值均增高,且HSPN组高于单纯紫癜组,单纯紫癜组高于健康对照组,组间差异具有显著性意义(P<0.01或<0.05)。MMP-2、-9、TIMP-1水平及比值与尿MA水平呈明显正相关(r=0·736,0.726,0·581Pa<0.01)。且尿MA异常组(Ⅱ)较正常组(Ⅰ)增高更明显,组间比较有显著性差异(P<0.01或0.05)。结论MMP-2、-9、TIMP-1水平升高及比值失衡参与HSPN的发病过程,并与尿MA形成呈正相关。     

紫癜性肾炎的早期诊断

目的通过对尿分析及24h尿蛋白定量均正常的过敏性紫癜性肾炎(HSPN)患儿61例查尿微量蛋白及行肾活检并行病理分析,探讨早期肾活检的重要性。方法选择初治或复治,伴或不伴有关节及消化道症状,且尿分析及24h尿蛋白定量均正常的HSPN患儿,多次查尿微量蛋白,如尿微量蛋白正常或初检异常但1～2周内尿微量蛋白很快恢复正常者,不予肾活检,而持续尿微量蛋白异常或反复尿微量蛋白异常≥3次者行肾活检,观察病理变化;比较尿Alb的量、尿微量蛋白选择性与病理损害程度的关系,以及肾组织中免疫复合物IgA、IgG、IgM的沉积与病理损害程度之间的关系。采用SPSS11.0软件进行统计处理。结果所有尿分析及24h尿蛋白定量均正常,而持续尿微量蛋白异常或反复尿微量蛋白异常≥3次者,行肾活检,肾脏均有不同程度(Ⅱ～Ⅲ级)损害,其中Ⅱa26例(42.6%),Ⅱb10例(16.4%),Ⅲa21例(34.4%),Ⅲb4例(6.6%);且尿Alb的量、尿微量蛋白选择性与病理损害程度无明显相关性(Pa>0.05);随病理程度加重,肾组织中IgA IgG IgM共同沉积的比例增加(P<0.05)。结论对HSPN尿分析及24h尿蛋白定量均正常的患儿,要密切检测尿微量蛋白,尽早行肾活检,及时治疗,以改善预后。     

尿α1—M和MA检测过敏性紫癜肾损害的临床意义

过敏性紫癜最严重的并发症是紫癜性肾炎,也是影响过敏性紫癜预后的决定因素。以往根据尿常规和肾功能改变确定肾损害,其发生率为30～90％不等。本文报告应用放免法检测尿常规、肾功能均正常的25例过敏性紫癜患儿尿α_1-微球蛋白(α_1-M)和微量白蛋白(MA)的含量,以及23例紫癜性肾炎患儿的尿α_1-M和MA的含量作为对照,探讨其临床意义。 对象和结果 一、观察对象 正常对照组来自本院的轻症上呼吸道感染初诊患儿30例,男17例,女13例,年龄     

实验室系列检查指标在早期诊断过敏性紫癜肾损害的敏感度选择

目的通过检测过敏性紫癜(HSP)患儿微量白蛋白(MA)、α1-微球蛋白(α1-M)和尿N-乙酰-β-D-氨基-葡萄糖胺酶(NAG)含量及相关实验室指标,观察HSP肾损害早期诊断的敏感性.方法采用免疫散射速率比浊法和碱性苦味酸速率法对62例HSP惠儿进行尿MA、α1-M、NAG排量检测,同时做肾功能、肾B超、尿常规、尿12 h Addis计数相关实验室的检查,结合临床及实验室检测指标分析HSP发生肾小菅受损的临床意义.结果1.HSP肾损害占80.60%,尿酶和尿两项特种蛋白、尿12 hAddis计数、尿常规、肾功能、肾B超对判断肾损害的敏感性分别为80.60%、76.00%、52.63%、48.39%、8.06%、7.14%;2.HSP患儿尿常规正常组(第1组)与异常组(第2组)间尿两项特种蛋白比较有显著差异性(P＜0.05),对照组与第1、2组分别比较亦有显著性差异(P＜0.05,P＜0.01),对照组NAG含量与第1组比较有显著性差异(P＜0.05).结论NAG和尿两项特种蛋白可敏感反映HSP肾损害情况,在某些程度上NAG的敏感性更高,可作为早期诊断HSP肾小管损害的判断指标.     

婴幼儿喘息性疾病尿MA、TRF、IgG、α1-MG的测定及临床意义

目的　 了解婴幼儿喘息性疾病患儿的肾小球和肾小管功能损害情况。 方法 　应用速率免疫散射比浊法测定尿MA、TRF、IgG、α1 MG。尿肌酐测定用碱性苦味酸速率法。 结果 　婴幼儿喘息性疾病尿MA、TRF、IgG、α1 MG排泄率 ,与正常对照组比较均有非常显著性差异 (P <0 0 1)。 结论 　婴幼儿喘息性疾病可能存在肾小球和肾小管功能早期一过性损伤。对临床用药有指导意义。     

各型紫癜性肾炎尿系列酶变化及相关因素分析

目的　通过尿系列酶 [N Z酰 β D氨基葡萄糖苷酶 (NAG)、γ 谷氨酰转移酶 (GGT)、碱性磷酸酶(ALP)、β 半乳糖苷酶 (GAL) ]的检测 ,探讨紫癜性肾炎 (HSPN)临床各型的肾小管受损程度及肾小管与肾小球损害之间的相关性。方法　 1.按照日本田博义对HSPN的分型法将本病分为临床 4型 ,其中Ⅰ型 2 4例、Ⅱ型 2 0例、Ⅲ型 2 0例、Ⅳ型 10例 ;2 .采用碱性苦味酸速率法对 74例HSPN患儿及 2 5例正常对照组患儿行尿系列酶检测 ,同时测定血肌酐 (BCr)及尿素氮 (BUN)做对照 ;3.分析临床各型尿酶变化及其与肾小球功能改变的关系。结果　HSPN临床 4型与对照组相比 ,尿系列酶均有不同程度改变。尿酶排量从高到低排列顺序依次为 :Ⅳ型 >Ⅲ型 >Ⅱ型 >Ⅰ型 >对照组 ;Ⅲ、Ⅳ型出现肾小球功能改变 (BUN、BCr含量增高 )时 ,尿酶排量增高更为明显。结论　尿系列酶可敏感地反映肾小管功能的早期受损。BUN及BCr含量既使在正常范围 ,尿酶含量已有变化 ,提示已有肾小管功能损害 ;当BUN及BCr出现异常改变时 ,尿酶改变更为突出。即BUN、BCr含量与尿酶排量呈正相关关系 ,尿酶排量越高 ,肾小管功能受累越明显 ,肾小球功能破坏也越严重     

肾脏疾病患儿血清免疫球蛋白及其亚类的变化

目的通过对儿童各类肾脏疾病进行血清免疫球蛋白(Ig)及亚类测定,探讨不同肾小球疾病体液免疫功能的变化。方法采用酶联免疫吸附法(ELISA)分别对急性肾炎26例、肾病综合征(NS)30例、紫癜性肾炎(HSPN)28例进行Ig及其亚类测定,20例正常儿童作为对照组。结果NS、急性肾炎、HSPN患儿IgG1和IgG2亚类明显低于正常对照组,差异有显著意义;IgG、IgG3、IgG4降低不明显,而急性肾炎组IgG3明显高于NS组及对照组,差异有显著意义(P<0.05)。结论小儿不同肾小球疾病具有不同的免疫特点,尤其是在总Ig无明显变化的状况下,各亚类的变化在其疾病的发生发展过程中占有重要地位。     

尿α1-微球蛋白与肺炎支原体感染致早期肾损害的相关性

目的分析尿α1-微球蛋白与肺炎支原体(MP)感染致早期肾损害的相关性。方法检测24例血冷凝集试验阳性肺炎患儿的尿常规及尿微量蛋白,包括尿微量清蛋白(Alb)、尿α1-微球蛋白(α1-MG)、尿转铁蛋白(TF)和尿IgG。结果24例中仅1例尿蛋白呈阳性。尿α1-MG在血冷凝集试验<1:256与≥1:256两组及病程≤2周与>2周两组差异均具有显著性(P均<0.05),而其他3项尿微量蛋白组间比较均无显著性差异。结论MP感染致早期肾损害多表现为肾小管重吸收障碍,可能与免疫有关。尿α1-MG可用于MP感染致肾损害的早期诊断。     

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.

---

---

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.     

�¶�֢��ϵ�ϰ���ע��ȱ�ݶද�ϰ������ڵ�ת��

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.