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对象:2004年10月1日至2005年9月31日住院正常分娩和剖宫产出的正常新生儿共1699例,男985例,女714例。方法:筛查人员经省医疗机构专门培训并考试合格的固定专职人员。筛查仪器:畸变产物耳声发射(DPOAE),采用美国GRASON STADLES公司生产的GSI70型全自动耳声发射仪作为听力筛查设备。筛查条件:测试在固定的相对安静非隔音室内,环境噪音约40～45dBSPL(A)。测试时新生儿在足量喂奶后1h,处于自然熟睡状态。生后2～4d用DPOAE进行初筛。生后42d对初筛未通过者进行复筛,仍采用DPOAE进行。生后3个月对复筛未通过者转诊到市听力检测中心…     

东莞地区新生儿听力筛查的多中心研究

目的：探索适合于新生儿听力筛查的临床策略,通过多中心合作获得新生儿听力损失发病的基本资料。方法：应用畸变产物耳声发射技术(DPOAE)进行听力普遍筛查。对复筛未通过者,于生后3个月左右采用听性脑干反应(ABR)和40Hz听性相关电位(40HzAERP)技术诊断。结果：该研究实际筛查17360例,筛查率为89.1%。初筛通过15624例(90.0%)。需复筛的1736例,实际复筛1597例,复筛率92%,复筛通过1491例,复筛通过率93.4%。41例复查ABR其中确诊新生儿先天性听力损失39例,减去2例假阳性,该组新生儿先天性听力损失的发生率为2.25‰。结论：新生儿听力普查十分必要。DPOAE是一种快速行之有效的新生儿听力初筛方法,两步筛查法可及早发现听力损失,听力筛查、诊断、康复统筹运作是使聋儿得以康复的重要保证。     

耳声发射在黄疸新生儿听力筛选中的应用

通过瞬态诱发耳声发射 (TEOAE)和畸变产物耳声发射 (DPOAE) ,探讨其在黄疸新生儿听力筛选中的临床意义。应用Celesta 50 3型耳声发射仪对 1 1 5名黄疸新生儿及对照组正常足月新生儿 1 41名 (共 51 2耳 )进行TEOAE和DPOAE听力测定。结果 2 56名新生儿中有 64名双耳听力异常 ,其中正常新生儿占 1 8 44%(2 6/ 1 41 ) ,黄疸新生儿占 3 3 0 4%(3 8/ 1 1 5) ,p <0 0 1 ,有显著意义。生后 3月再次接受OAE复查 ,2例高胆红素患儿听力仍异常。新生儿高胆红素血症是听损伤的高危因素 ,应作听力随访。耳声发射是 1种快而有效的新生儿听力筛选方法之一。     

不同日龄新生儿诱发性耳声发射测试

对20例正常新生儿在出生后第1～5天逐日行瞬态诱发性耳声发射(TEOAE)和畸变产物耳声发射(DPOAE)的测试.了解不同日龄新生儿畸变产物耳声发射(DPOAE)和瞬态诱发性耳声发射(TEOAE)的特征,探讨应用OAE作新生儿听力筛查的最佳时机.随新生儿日龄的增加,其TEOAE和DPOAE检出率和反应幅值逐步提高,新生儿生后第1～2天的检出率和反应幅值显著低于第3～5天,至生后第3～5天,TEOAE和DPOAE检出率接近或达到100%,反应幅值也趋于稳定.结论应用TEOAE和DPOAE进行新生儿听力筛查时,其日龄至少应在3天或3天以上,测试TEOAE和DPOAE快速、方便,不失为普遍性新生儿听力筛查首选方法.     

不同日龄新生儿诱发性耳声发射测试

对20例正常新生儿在出生后第1～5天逐日行瞬态诱发性耳声发射(TEOAE)和畸变产物耳声发射(I)POAE)的测试。了解不同日龄新生儿畸变产物耳声发射(DPOAE)和瞬态诱发性耳声发射(TEOAE)的特征，探讨应用OAE作新生儿听力筛查的最佳时机。随新生儿日龄的增加，其TEOAE和DPOAE检出率和反应幅值逐步提高，新生儿生后第1～2天的检出率和反应幅值显著低于第3～5天，至生后第3～5天．TEOAE和DPOAE检出率接近或达到100％，反应幅值也趋于稳定。结论：应用TEOAE和DPOAE进行新生儿听力筛查时，其日龄至少应在3天或3天以上，测试TEOAE和DPOAE快速、方便，不失为普遍性新生儿听力筛查首选方法。     

耳声发射在黄疸新生儿听力筛选中的应用

通过瞬态诱发耳声发射(TEOAE)和畸变产物耳声发射(DPOAE),探讨其在黄疸新生儿听力筛选中的临床意义.应用Celesta 503型耳声发射仪对115名黄疸新生儿及对照组正常足月新生儿141名(共512耳)进行TE0AE和DPOAE听力测定结果256名新生儿中有64名双耳听力异常,其中正常新生儿占18.44%(26/141),黄疸新生儿占33.04%(38/115),p<O.01,有显著意义生后3月再次接受(OAE复查,2例高胆红素患儿听力仍异常.新生儿高胆红素血症是听损伤的高危因素,应作听力随访.耳声发射是1种快而有效的新生儿听力筛选方法之一.     

3～6岁儿童911例听力筛查

目的 了解集体儿童中听力现状。方法对911例3～6岁学龄前幼儿园集体儿童。使用美国Bio—logic耳声发射仪进行测试。结果 911例接受听力筛查儿童中，通过者882例，未通过者29例（3．18％）。1个月后对未通过者进行复筛，其中通过者7例，未通过者22例。对未通过的22例进一步做声阻抗等检查发现：中耳炎17例，耵聍阻塞2例，鼻窦炎2例，服用化疗药物1例。结论 对于有听力损失的儿童，提供最佳的有效干预治疗，以便提高他们的交流技巧、语言能力的发展和行为的调整，进行早期听力筛查并确诊非常必要且非常重要。     

基层医院新生儿听力筛查模式探讨

目的探讨在基层医院开展新生儿听力筛查工作的方法。方法采用瞬态诱发耳声发射(TEOAE)对新生儿进行听力筛查,初筛未通过者于生后42 d左右进行复筛,复筛仍未通过者于出生3个月左右进行听性脑干诱发反应(ABR)等诊断性听力检查,并跟踪随访。结果初筛覆盖率为86.10%,初筛未通过率34.20%,复筛未通过率6.5%,不同程度听力损失婴幼儿占筛查总人数4.69‰。结论基层医院开展新生儿听力普遍筛查很有必要。     

耳声发射在高危新生儿的应用探讨

为探讨某些高危因素对新生儿听力的影响,尽早发现听力障碍,应用丹麦产Madsen Capeila耳声发射仪,对124名高危新生儿测试瞬态耳声发射(TEOAE)和畸变耳声发射(DPOAE),对其中一项不能通过者一月后复查,仍不能通过者3个月复查,如仍不能通过行OAE+脑干诱发电位(ABR)检查,明确听力障碍的原因并行干预治疗.结果显示单耳TEOAE异常者28例次,DPOAE异常者33例次,双耳TEOAE异常者26例次,DPOAE异常者23例次.生后第一次查OAE 124人,未能通过69人,生后一月复查65人,未能通过者26人;生后3个月复查,未能通过者2人,生后6个月复查,1人未通过,异常率0.8%,经ABR检查为单耳轻度听力损害.结论(1)高危儿听力障碍的发生率为0.8%;(2)对第一次不能通过OAE的婴儿,尚不能断定为听力障碍,要定期复查和随访.(3)应对所有出生的新生儿都进行听力筛查,不能只限于高危儿.(4)对巨细胞病毒感染,生后虽OAE正常,但需进一步随访和复查.     

耳声发射在高危新生儿的应用探讨

为探讨某些高危因素对新生儿听力的影响，尽早发现听力障碍，应用丹麦产Madsen Capeila耳声发射仪，对124名高危新生儿测试瞬态耳声发射(TEOAE)和畸变耳声发射(DPOAE)，对其中一项不能通过者一月后复查，仍不能通过者3个月复查，如仍不能通过行OAE 脑干诱发电位(ABR)检查，明确听力障碍的原因并行干预治疗。结果显示：单耳TEOAE异常者28例次，DPOAE异常者33例次，双耳TEOAE异常者26例次，DPOAE异常者23例次。生后第一次查OAE124人，未能通过69人，生后一月复查65人，未能通过者26人；生后3个月复查，未能通过者2人，生后6个月复查，1人未通过，异常率0．8％，经ABR检查为单耳轻度听力损害。结论：(1)高危儿听力障碍的发生率为0．8％；(2)对第一次不能通过OAE的婴儿，尚不能断定为听力障碍，要定期复查和随访。(3)应对所有出生的新生儿都进行听力筛查，不能只限于高危儿。(4)对巨细胞病毒感染，生后虽OAE正常，但需进一步随访和复查。     

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.