出血性休克与脑病综合征患儿脑功能损害的临床特点

目的　探讨出血性休克与脑病综合征的脑功能损害的临床特点。方法　对 12例出血性休克与脑病综合征患儿的临床资料进行分析总结。结果　 12例依据脑功能损害的临床特点可分为暴发型 7例 :发病后迅速进入昏迷 ,频繁抽搐 ,肌张力显著降低 ,伴有多脏器功能衰竭 ;脑功能障碍型 5例 :入院后病情有所缓解 ,但 3～ 4d后又反复抽搐 ,持续 2d ,在此期间检查可有重度脑电图异常和CT示脑水肿。本文 7例暴发型均死亡 ,5例脑功能障碍型 ,4例留有严重的运动和语言障碍 ,1例目前判断神经功能正常。结论　出血性休克与脑病综合征可分为暴发型和脑功能障碍型 ,各有其临床特点。     

出血性休克与脑病综合征

出血性休克与脑病综合征(HSES)于1983年由Levin首先报道并加以命名。HSES发病迅速,病死率高,生存者易留有严重的神经系统后遗症,其临床特点是突发的抽搐和昏迷、休克、弥漫性血管内凝血、水样腹泻、代谢性酸中毒、肝肾功能障碍。该文对HSES的临床研究进展作一综述,以提高对该病的认识和治疗水平。     

出血性休克与脑病综合征

出血性休克与脑病综合征(HSES)于1983年由Levin首先报道并加以命名。HSES发病迅速，病死率高，生存者易留有严重的神经系统后遗症，其临床特点是突发的抽搐和昏迷、休克、弥漫性血管内凝血、水样腹泻、代谢性酸中毒、肝肾功能障碍。该文对HSES的临床研究进展作一综述，以提高对该病的认识和治疗水平。     

儿童致死性病毒性脑病7例

目的探讨致死性病毒性脑病的临床和实验检查特点。方法回顾性分析7例致死性病毒性脑病患儿临床过程及预后,肝酶学、脑脊液常规改变,头颅CT改变。结果7例患儿均呈骤然高热、严重抽搐和迅速进入昏迷、肝酶显著升高。4例死亡,3例留有严重神经系统后遗症;血氨均正常;头颅CT均显示显著脑肿胀,可见对称性基底核低密度病变。结论高热、迅速发生抽搐和昏迷、肝功能损害的患儿,应警惕致死性病毒性脑病发生。     

出血性休克和脑病综合征

出血性休克和脑病综合征(Heamorrhagic Shock and Eecephalopathy syndrom, HSE)起病突然,发生于婴儿期,以高热、脑病、严重休克和DIC、水样或血水样腹泻伴肝肾功能不全为特征。Levin等于1983年报道10例,并首次将本病命名为HSE。     

出血性休克与脑病综合征的干预探讨

目的　 探讨出血性休克与脑病综合征 (HSES)有效的急救措施。 方法 　回顾性分析 12例出血性休克与脑病综合征的临床过程 ,干预措施及对干预的反应。 结果 　HSES的主要危害是脑功能的损害。 结论 　改善和保护脑功能是治疗的重点 ,关键是迅速恢复氧通气、脑灌注 ,即迅速稳定呼吸和循环功能。休克纠正越快 ,预后越好 ;扩容应迅速而大胆。可尽早气管插管和正压通气 ,纠正低氧血症。正压通气、纠酸、抗DIC、抑制炎症反应均有利于循环功能的改善 ,应尽早进行。降颅压并不能改善神经系统功能的预后     

出血性休克和脑病综合征

去年,有10例婴儿因患以往未被认识的新的疾病而住院。此病以脑病的急性发作、发烧、休克、水样泻、严重的弥漫性血管内凝血(DIC)和肾脏、肝脏功能障碍为特征。这些婴儿中7例死亡。尚未发现特异性病原,但初步研究提示此病的病理生理学改变可能包括蛋白分解酶(如胰蛋白酶)释放入血循环并伴有微循环障碍。作者称此病为出血性休克和脑病综合征(HSE)。     

出血性休克与脑病综合征的干预探讨

目的 探讨出血性休克与脑病综合征(HSES)有效的急救措施。方法 回顾性分析12例出血性休克与脑病综合征的临床过程，干预措施及对干预的反应。结果 HSES的主要危害是脑功能的损害。结论 改善和保护脑功能是治疗的重点，关键是迅速恢复氧通气、脑灌注，即迅速稳定呼吸和循环功能。休克纠正越快，预后越好；扩容应迅速而大胆。可尽早气管插管和正压通气，纠正低氧血症。正压通气、纠酸、抗DIC、抑制炎症反应均有利于循环功能的改善，应尽早进行。降颅压并不能改善神经系统功能的预后。     

出血性休克并脑病综合征

出血性休克并脑病综合征是 Levin 等于1983年首次提出的一种见于婴儿期的综合征。临床表现主要为突发高热、休克、脑病、弥漫性血管内凝血、肝肾功能损害。病死率高达60%以上,存活者亦留有严重的神经系统后遗症。其发病机制尚不清楚,认为可能与婴儿体温调节中枢不完善或病毒感染、特发性胰腺炎有关。病理表现为多脏器点状出血、坏死或炎性改变。目前,尚无特效治疗方法,主要以早期采取对症、支持治疗为主。     

2007年第4期《中国小儿急救医学》国家级继续医学教育园地答案

1．脑炎、瑞氏综合征、出血性休克脑病综合征和急性坏死性脑病 2．儿童，发热、意识改变、惊厥发作 3．发病年龄小、重度弥漫性脑水肿、血小板明显下降、血清谷草转氨酶明显升高[第一段]     

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.