双胎贫血-红细胞增多序列征新生儿喂养不耐受3例总结

目的探讨双胎贫血-红细胞增多序列征新生儿喂养情况。方法回顾分析3例喂养不耐受的双胎贫血-红细胞增多序列征新生儿的临床资料。结果 3例生后确诊为双胎贫血-红细胞增多序列征的单绒毛膜双胎早产的供血儿,在喂养过程中都出现了喂养不耐受的临床表现,而3例受血儿的喂养过程顺利。结论单绒毛膜双胎妊娠,若存在宫内慢性双胎输血,产后需密切关注新生儿的喂养问题,尤其双胎供血儿。     

双胎妊娠一胎胎死宫内后存活儿的近期预后

目的 分析双胎妊娠一胎胎死宫内（sIUFD）后存活儿的近期预后。方法 以sIUFD后存活儿52例为病例组,同时选取同期收治的104例胎龄匹配、不伴有sIUFD双胎妊娠分娩的双胎之一为对照组,对其临床资料进行比较分析。结果 52例sIUFD后存活儿中,早产儿42例（80.8%）,合并脑损伤13例（25.0%）,新生儿期死亡3例（5.8%）。sIUFD组的羊水异常（羊水粪染或血性羊水、羊水过多或过少）、脐带异常（脐带扭转或脐带绕颈）及胎盘异常（前置胎盘或胎盘早剥）的发生率、出生窒息率、出生时贫血或红细胞增多症发生率及出生时凝血功能障碍发生率均明显高于对照组（P < 0.05）;sIUFD组住院期间出现院内感染及脑损伤的发生率明显高于对照组（P < 0.05）。结论 双胎妊娠发生sIUFD后会增加存活儿新生儿期并发症的发生率,对于sIUFD存活儿,应加强产前评估,并进行远期预后随访。     

新生儿红细胞增多症47例临床分析

本文报道新生儿红细胞增多症47例,病因:①宫内缺氧红细胞生成素增加(主动型):围产期窒息25例,小于胎龄儿12例,母患糖尿病及21-三体综合征各1例,②血容量增加(被动型):胎～胎输血7例,晚扎脐带1例。有症状的婴儿采用部分换血治疗(15～20ml/kg),尽快使红细胞压积降至0.60以下,本组部分换血22例,另25例经血浆、白蛋白,甚至生理盐水静脉稀释治疗,46例痊愈,仅1例早产儿治疗过晚死亡,病死率为2.1％,     

新生儿红细胞增多症与围产期缺氧因素分析

本文总结了１９９３年３月￣１９９６年２月三年的新生儿红细胞增多症与其胎生有无宫内窘迫的关系其出生。结果表明：在８４例红细胞增多症瘤儿中，分为足月儿和早产儿两组，宫内窘迫急性缺氧若导致红细胞增多以足月儿为多；慢性缺氧则以早产儿为多（Ｐ〈０．００５）。但是，血红蛋白、红细胞压积及红细胞计数与宫内急慢性缺氧和胎龄无密切相关，有待进一步深入了解导致宫内乏氧因素与红细胞增多关系。     

新生儿红细胞增多症与围产期缺氧因素分析

本文总结T1993年3月～1996年2月三年的新生儿红细胞增多症与其胎生有无宫内窘迫的关系其出生。结果表明：在84例红细胞增多症瘤儿中，分为足月儿和早产儿两组，宫内窘迫急性缺氧若导致红细胞增多以足月儿为多；慢性缺氧则以早产儿为多（P＜0．005）。但是，血红蛋白、红细胞压积及红细胞计数与宫内急慢性缺氧和胎龄无密切相关，有待进一步深入了解导致宫内乏氧因素与红细胞增多关系。     

新生儿红细胞增多症危险因素病例对照研究

目的：新生儿红细胞增多症发病因素复杂,以往多采用单因素分析法进行研究,该文以多因素分析法分析其独立危险因素,并分析因素间的交互作用,为该病防治提供依据。方法：采用病例对照研究对新生儿红细胞增多症27种危险因素进行调查,对单因素分析中有显著性意义的因素进行非条件logistic回归分析,并以加法模型理论分析因素间的交互作用。结果：单因素分析显示妊高征、窒息、宫内窘迫、小于胎龄儿、胎膜早破、早产、胎盘早剥、多胎妊娠、低出生体重等9个因素有显著性意义(P<0. 05);非条件logistic回归分析显示窒息(OR=7. 8255)、胎膜早破(OR=2. 7007)、妊高征(OR=2. 8313)、早产(OR=7. 7394)、低出生体重(OR=7. 4803)为红细胞增多症的独立危险因素;交互分析显示,低出生体重和小于胎龄儿、低出生体重与宫内窘迫、早产与小于胎龄儿之间存在正交互作用(交互指数大于1)。结论：该病的发生与多种独立危险因素有关,多个危险因素并存亦加大了该病发生的可能性,临床上应加强对相关危险因素的监测和处理。     

红细胞增多症引起的硬肿症12例临床分析

新生儿硬肿症是新生儿常见疾病之一,亦是围产儿死亡的重要原因之一,其发病因素复杂。本文总结了我院1992年1月至1997年10月间因红细胞增多症(NP)引起硬肿症12例,报道如下。临床资料:1.一般资料:12例中男7例,女5例,足月儿10例,早产儿(双胎之一)2例;出生体重最低2050g,最高3300g,平均2850±75g。发生硬肿时间:36～<72h7例,72～96h5例。2.诊断标准:对有宫内缺氧及经胎盘血液灌注过多的高危儿于生后72h内进行Hb及HCT测定,HB≥220g/L,静脉血HCT>65%者,并除外血液浓缩及化验误差,诊断为NP;对其中发生硬肿者,排除其他可能引起硬肿症的因素如…     

新生儿红细胞增多症心脏瓣膜口血液速度变化的观察

用超声脉冲多普勒心动图检测１０例新生儿红细胞增多症心脏各瓣膜口峰值血流速，并与正常对照组进行对比分析，结果表明红细胞增多症组与对照组，红细胞增多症组经治疗后的峰值血流速均明显缓慢，且血流速与红细胞压积的高低呈反比。     

新生儿产前失血的诊治

新生儿产前失血主要由胎胎输血、胎母输血、胎儿胎盘出血及产前的某些诊疗操作所致。产前急性失血可以引起失血性休克,慢性失血可引起新生儿贫血和宫内发育不良,严重者可致死亡,应及时治疗。1　新生儿产前出血的病因及其临床表现1 1　胎胎输血　病因:胎胎输血又称双胎输血综合征(twin twintransfusionsyndrome ,TTTS) ,1 9世纪由奥地利解剖学家Hyrtl和德国妇科学家Schatz首先提出,是单卵单绒毛膜双胎妊娠的一个合并症。胎胎输血发生的解剖学基础是双胎胎盘间有血管吻合,血管吻合可以有各种形式,如动脉到动脉、静脉到静脉、动脉到静脉,或…     

新生儿双胎输血综合征的临床研究

目的　探讨不同分级、不同宫内干预和不同血流供输结果双胎输血综合征(twin-twin transfusion syndrome, TTTS)患儿的临床表现及近期预后。方法　对76例新生儿病房住院的TTTS患儿的住院病案资料进行回顾性研究。将入选对象按以下3种方法分组：(1)按照TTTS分级分为轻度TTTS组(n=38)和重度TTTS组(n=21); (2)按照不同宫内干预方法分为羊水减量组(n=20)、激光治疗组(n=21)和期待治疗组(n=32); (3)按照输血对象分为供血儿组(n=23)和受血儿组(n=30)。结果　重度TTTS组脑损伤、心脏病变、生后窒息、肾功能损害的发生率和住院期间病死率均高于轻度TTTS组, 但差异无统计学意义。激光治疗组脑损伤、心脏病变、肾功能损害的发生率和住院期间病死率有低于羊水减量组和期待治疗组的趋势。受血儿组心脏病变和病理性黄疸的发生率高于供血儿组, 差异有统计学意义(P<0.05)。供血儿组生后窒息和肾功能损害的发生率高于受血儿组, 但差异无统计学意义。结论 TTTS分级高者器官损伤发生率和住院期间病死率相对高。激光治疗较期待治疗和羊水减量治疗的TTTS患儿的预后可能更佳。受血儿更易发生心脏病变、病理性黄疸, 供血儿发生生后窒息和肾脏损害的倾向高于受血儿。     

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.