血、尿β_2-MG及尿NAG测定与新生儿窒息后肾功能评价

目的：新生儿窒息可引起全身多脏器功能损害,该文通过观察窒息新生儿血、尿β2微球蛋白(β2-MG)及尿N-乙酰-β-D-氨基葡萄糖苷酶(NAG)含量的变化以评价新生儿窒息后肾功能的改变。方法：采用放射免疫法和对硝基酚比色法对28例窒息新生儿(又分为重度窒息组13例和轻度窒息组15例)和16例正常新生儿血、尿β2-MG及尿NAG同时进行检测。结果：窒息组血、尿β2-MG及尿NAG值［(4.46±1.42) mg/L,(2.69±1.80) mg/L,(13.68±2.01) U/mmol.Cr］明显高于正常组［(2.97±1.24) mg/L,(0.96±0.82) mg/L,(6.12±1.16) U/mmol.Cr］(P<0.01)。重度窒息组血、尿β2-MG及尿NAG值［(4.99±1.28) mg/L,(3.86±1.14) mg/L,(13.94±3.82) U/mmol.Cr］高于轻度窒息组［(4.30±1.21) mg/L,(2.93±0.87) mg/L,(9.68±1.27) U/mmol.Cr］(P<0.05)。窒息新生儿尿β2-MG较血β2-MG升高更明显(P<0.01)。结论：血、尿β2-MG及尿NAG测定对早期评价新生儿窒息后肾功能损害,尤其是对肾小球或肾小管的损害定位、预测损伤程度具有重要意义。     

新生儿窒息后肾损伤监测指标的对比研究

目的 对比研究反映窒息新生儿肾损伤的指标差异。方法 测定48例窒息新生儿(重度25例、轻度23例)血清尿素氮(BUN)、肌酐(Cr)、尿酸(Ua)与尿N-乙酰β-D-氨基葡萄糖苷酶(NAG)、尿γ谷胺酰转肽酶(GGT)、尿β2-微球蛋白(β2MG)和尿常规的变化。结果 重度窒息组各项指标的异常发生率高于轻度窒息组(P<0.05)。重度、轻度窒息组内尿NAG，GGT，β2-MG的异常发生率(83.3%和50.0%，83.3%和50.0%，83.3%和70.0%)高于血清BUN，Cr，Ua异常发生率(33.3%和15.0%，57.1%和40.0%，50.0%和33.3%)。结论 窒息新生儿普遍存在肾功能损害，以肾小管损伤为主为重。早期诊断用尿酶、β2-MG和尿常规反映更为有价值。     

肾病综合征患儿血清抗增殖蛋白水平

目的研究肾病综合征患儿血清抗增殖蛋白(prohibitin,PHB)蛋白水平及其在肾小管间质早期损伤中的意义。方法应用Western blot对36例原发性肾病综合征患儿血清进行PHB蛋白水平检测,同期正常体检儿童30例为对照组。同时检测两组血清肌酐(Scr)、血尿素氮(BUN)、尿微量蛋白系列[白蛋白/肌酐(ALBU/Cr)、N-乙酰β-D-葡萄糖苷酶/肌酐(NAGU/Cr)、免疫球蛋白G/肌酐(IgGU/Cr)、α1微球蛋白/肌酐(α1-MU/Cr)等]。肾小球及肾小管间质损伤评分参照Katafuchi半定量法进行。结果正常对照组儿童血中未检测到PHB蛋白,肾病综合征患儿血PHB蛋白水平不同程度增高(0.203±0.032比0±0,P<0.05)。伴增生性病变肾病综合征患儿血PHB水平明显高于非增生性患儿。血PHB水平与肾小管间质损伤程度以及肾小球损伤程度均呈明显正相关(r=0.868、0.753,P均<0.001);患儿血PHB水平与尿微量蛋白NAG、IgG呈正相关(r=0.586、0.341,P均<0.001)。结论肾脏疾病患儿血PHB表达明显增高,而且血PHB水平与肾小球及肾小管间质损伤程度明显相关...     

儿科肾小球疾病对肾小管功能的前瞻性对照观察

目的 前瞻性观察儿科肾小球疾病的肾小和功能改变。方法 对64例有各种肾小球疾病但肾功能正常者（A组），29例急性肾功能不全者（B组）及73例对照者（C组）3组患儿肾小管功能进行前瞻性观察。将尿β2-微球蛋白（β2-MG）及尿溶液菌酶（LYS）作为近端肾小管功能，24h尿Tamm-Horsfall蛋白（THP）作为远端肾小管功能的评估指标，并与血尿素氮（BUN）、血肌酐（BCr）（肾小球功能）相对照。结果 BUN、BCr及尿β2-MG，LYS，THP在A，C两组间差异无显著性，而与B与A、C组间差异均有显著性。在B组中，血BUN、BCr分别与β2-MG、LYS、THP均无显著相关性。结论 小儿肾小球疾病肾小球功能在正常范围时，肾小管功能无明显改善；一旦肾小球功能受损伤时，则肾小管功能即有不同程度受损，且两者受损程度平行。     

尿系列酶测定对新生儿硬肿症肾功能的评价

目的　探讨新生儿硬肿症尿系列酶的改变作为评价肾小管功能的指标。方法　检测新生儿硬肿症尿系列酶NAG、GAL、GGT、ALP、LDH活性 ,与正常组进行对照 ,同时检测血BUN ,Bcr作为肾小球功能的指标 ,中、重度硬肿者与尿酶值进行相关性分析。结果　尿酶NAG、GAL、ALP、LDH酶活性在新生儿硬肿症组与对照组比较明显增高 ,有显著差异 (P <0 0 1) ,GGT酶无显著差异 (P >0 0 5 ) ;三种不同程度新生儿硬肿症时尿酶NAG、GAL、ALP、LDH均出现统计学差异 ;肾功能血BUN、Bcr值增高与尿酶间存在正的直线相关关系。结论　尿酶NAG、GAL、ALP、LDH活性不同程度升高 ,表明不同程度新生儿硬肿症肾小管功能受累 ,当肾小球功能受损时 ,其与肾小球功能受损成正比     

尿胱抑素C对缺氧缺血性脑病新生儿肾功能的诊断价值

目的 探讨尿胱抑素(Cys C)对围生期HIE新生儿肾小管功能损害的诊断价值.方法 选取HIE新生儿47例为HIE组,按照HIE诊断标准又将其分为轻度HIE组(25例)和中重度HIE组(22例).选取无肾功能损害诱因及HIE临床表现及实验室指标的非HIE新生儿23例为对照组.测定各组新生儿日龄3 d内的尿Cys C及尿视黄醇结合蛋白(RBP)、N-乙酰-β-氨基葡萄糖苷酶(NAG)、β2微球蛋白(β2-MG)等尿酶系列,并计算滤过钠排泄分数(FENa%),应用Kolmogorov-Smimov法分析各组间各指标的差异.结果 HIE组尿Cys C、RBP及FENa%较对照组显著增高,中重度HIE组尿Cys C升高更加明显(Pa<0.05).β2-MG、NAG在对照组、轻度HIE组、中重度HIE组间呈逐渐上升,但除中重度HIE组β2-MG较对照组显著增高(P<0.01)外,其他组组间比较无显著性差异.尿Cys C、RBP、β2-MG、NAG与FENa%均呈显著正相关(r=0.572,0.648,0.402,0.368 Pa<0.05).结论 尿Cys C可作为反映肾小管损伤的诊断指标之一,其诊断性能优于β2-MG,而与尿RBP相似.     

小儿肾小球疾病的肾小管功能测定

目的：检测小儿肾小球疾病的肾小管功能改变。方法：对各种肾小球疾病但肾功能正常者64例(A组),急性肾功能不全29例(B组)及75例对照者(C组)3组患儿肾小管功能进行前瞻性观察。将β2-MG及LYS作为近端肾小管功能、THP作为远端肾小管功能的评估指标,并与血的BUN,BCr(肾小球功能)相对照。结果：血BUN,BCr及尿β2-MG,LYS,THP在A,C两组间无显著性差异,而在A与B或B与C组间均有显著性差异。在B组中,血BUN,BCr分别与β2-MG,LYS,THP均有显著相关关系。结论：小儿肾小球疾病肾小球功能在正常范围时,肾小管功能无明显改善;一旦肾小球功能受损伤时,则肾小管功能即有不同程度受损,且两者受损程度平行。     

尿系列酶检测对新生儿肺炎肾功能的评价

目的　 探讨新生儿肺炎时尿系列酶的改变作为评价肾小管功能的指标。 方法 　检测早产、足月新生儿肺炎尿系列酶NAG、GAL、GGT、ALP、LDH活性 ,与正常组进行对照。同时检测血BUN ,SCr作为肾小球功能的指标 ,GAL/NAG比值动态观察作为肾功能恢复的指标。 结果 　早产儿组尿酶NAG、GAL、GGT、ALP、LDH活性与对照组比较明显增高 ,有显著差异 (P <0 0 0 1) ,足月儿组尿酶NAG、ALP、LDH活性与对照组比较明显增高 ,有显著差异 (P <0 0 0 1)。尿酶GAL/NAG比值恢复期与对照组比较有统计学差异 (P <0 0 5 )。 结论 　尿酶NAG、GAL、GGT、ALP、LDH活性增高可作为新生儿肺炎时肾小管功能受累的指标 ,GAL/NAG比值升高提示新生儿肺炎肾功能的恢复     

生物学标志物联合检测在儿童尿路感染定位诊断中的意义

目的 探讨儿童上尿路感染(UUTI)与下尿路感染(LUTI)的生物学标志物水平的差异,为尿路感染(UTI)的定位诊断提供帮助.方法 将2010年5月至2012年1月在天津市儿童医院住院治疗的109例UTI患儿分为UUTI组(18例)和LUTI组(91例).收集患儿的临床资料(包括性别、年龄和起病症状);实验室检查结果,包括血BUN、Cr;生物学标志物包括血清胱抑素C(CysC)、降钙素原(PCT)、血p2-微球蛋白(β2-MG),尿微量清蛋白(mAlb)、转铁蛋白(TF)、α1-微球蛋白(α1-MG)、β2-MG、N-乙酰-β-D-氨基葡萄糖苷酶(NAG).采用SPSS 17.0软件进行数据分析..结果 UUTI组患儿血清CysC、PCT,mAlb、TF、α1-MG、β2-MG、NAG水平均高于LUTI组(P均＜0.05).经受试者工作特征(ROC)曲线分析,上述7项指标的曲线下面积为0.71 ～0.87.采用Logistic多元回归分析,入选和剔除变量的标准为α=0.05,认定CysC、PCT、NAG为影响因素并建立模型.通过这3项指标联合检测,诊断价值更高,诊断灵敏度为90.0％时,特异度可达88.9％.结论 血清CysC、PCT、尿NAG、TF、mAlb、α1-MG、β2-MG均可用于UUTI、LUTI的鉴别诊断,血清CysC、PCT、尿NAG 3项指标联合检测的诊断价值优于各单项指标.     

小儿全身炎症反应综合征早期肾损伤敏感指标的研究

目的探讨小儿全身炎症反应综合征(SIRS)早期肾损伤的敏感指标,为早期干预提供理论依据。方法以2000年12月至2001年11月广州市儿童医院儿科病房45例SIRS患儿及30名健康儿童为研究对象,尿β2微球蛋白(β2-MG)、白蛋白(ALB)采用化学发光酶免疫分析法(CLEIA)测定,尿微量IgG、血、尿视黄醇结合蛋白(RBP)采用酶联免疫吸附比色法(ELISA)测定,尿N-α-乙酰基-β-D-氨基葡萄糖苷酶(NAG)采用比色法测定。结果实验组与对照组比较,尿ALB、β2-MG、RBP、NAG、血β2-MG、血RBP明显升高,有显著性差异。结论尿ALB、β2-MG、RBP、NAG、血β2-MG、血RBP为SIRS患儿早期肾损伤的敏感指标,SIRS患儿早期机体反应较轻时已存在早期肾损害并且病变累及肾小球和肾小管。     

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.