选择性头部亚低温对新生儿缺氧缺血性脑损伤的治疗作用

目的 探讨选择性头部亚低温对足月新生儿窒息后缺氧缺血性脑损伤（ＨＩＢＤ）的神经保护作用及其安全性。方法 将２２例重度窒息足月新生儿随机分为治疗组（１１例）和对照组（１１例）。治疗组采用选择性头部降温方法,维持鼻咽温度为（３４．０±０．２）℃,持续７２ ｈ;对照组不进行降温治疗。两组均于治疗后６４～７２ ｈ测脑脊液神经元特异性烯醇化酶（ＮＳＥ）,血ＣＫ－ＭＢ,尿β２微球蛋白（β２－ＭＧ）等。于治疗前、生后１０ ｄ和３个月进行常规１６导ＥＥＧ检测,并采用新生儿神经行为评分（ＮＢＮＡ）、婴幼儿发育量表（ＣＤＣＣ）进行神经行为发育评价。结果 治疗组脑脊液ＮＳＥ为（１９．５±２．２）μｇ／Ｌ,明显低于对照组[（２４．６±５．３）μｇ／Ｌ］（Ｐ ＜ ０．０１）;生后２８ ｄ治疗组ＮＢＮＡ评分为（３６±３）分,低于对照组[（３２±２）分］（Ｐ＜０．０１）。治疗前两组ＥＥＧ均异常,生后１０ｄ,３个月治疗组ＥＥＧ均正常,对照组２例持续重度异常。两组患儿血ＣＫ－ＭＢ及尿β２－ＭＧ差异无显著性（Ｐ＞０．０５）。结论 选择性头部亚低温对足月新生儿窒息后ＨＩＢＤ可能具有神经保护作用,临床上具有安全性。     

两种尿微量蛋白检测对评估新生儿肾单位发育的价值

为准确评估新生儿时期肾单位发育情况，本文有产联免疫吸附法（ＥＬＩＳＡ）对１４０例中足月及早产儿作尿微量蛋白（ＭＡＬＢ）和尿视黄醇结合蛋白（ＲＢＰ）检测，发现早产儿与足月儿在相同出生阶段相比，ＭＡＬＢ、ＲＢＰ含量显著和蔼同（Ｐ〈０．０１），而早产儿日８－２８天组与足月儿日龄７天内组相比较时ＭＡＬＢ无显著差异Ｐ〉０．０５，ＰＲＢＰ值仍较高，说明在新生儿期早产儿肾单位的发育状况明显落后于足月儿，但其发育     

应用新生儿行为神经测定及脑CT判断新生儿缺氧缺血？…

目的 探讨新生儿行为神经测定及脑ＣＴ影像学改变对新生儿缺氧缺血性脑病预后的关系。方法 采用新生儿行为神经测定（ＮＢＮＡ）对４２例中、重度缺氧缺血性脑病（ＨＩＥ）患儿进行评分及生后（２８￣３０）ｄ脑ＣＴ影像学检查,分别以７月龄发育商ＤＱ９５为标准进行对比。结果 ＮＢＮＡ测定７ｄ〈３５,１４ｄ≤３５分患儿组的ＤＱ低于７ｄ≥３５,１４ｄ〉３５分组,统计学差异有非常显著性意义（Ｐ〈０．０１）;生后（２８￣     

新生儿感染性肺炎血清维生素A水平

研究目的探讨新生儿感染性肺炎患者血清中维生素Ａ水平。研究方法病例对照研究。肺炎组和对照组各２５例，早产儿各７例，足月儿各１８例。采用高效液相色谱仪（ＨＰＬＣ）测定其维生素Ａ含量。结果肺炎组早产儿与对照组早产儿血清中维生素Ａ水平分别为０．３２±０．１２和０．６２±０．３２μｍｏｌ／Ｌ（ｔ＝２．３２２，Ｐ＜０．０５）；肺炎组足月儿与对照组足月儿维生素Ａ水平分别为０．５８±０．１２和０．８３±０．２７μｍｏｌ／Ｌ（ｔ＝２．４６３，Ｐ＜０．０２）。肺炎患者１５例（治疗组）辅以维生素Ａ治疗（１０００ＩＵ／ｄ），另外１０例肺炎患者不用维生素Ａ（对照组），治疗组病程平均为９．７天，对照组为１２．３天（Ｐ＜０．０５）。结论新生儿感染性肺炎患者血清中维生素Ａ水平显著降低，早产儿降低更为明显。辅以维生素Ａ治疗新生儿肺炎效果较好。     

扩张性心肌病儿童的左心舒张功能改变：附21例报告

采用多普勒超声心动图测量了２１例扩张性心肌病（ＤＣＭ）儿童与２０例正常对照儿童的二尖瓣多普勒充盈参数。结果显示,与正常对照组儿童相比,ＤＣＭ组患儿中,１８例（８５．７％）表现为Ｅ峰最大速度（Ｅ）、Ｅ峰与Ａ峰最大速度之比（Ｅ／Ａ）均减低（Ｐ〈０．０５）,Ｅ峰减速时间（ＤＴＥ）延长（Ｐ〈０．０１）,３例（１４．３％）表现为Ｅ增大,Ｅ／Ａ〉２,ＤＴＥ明显缩短,其心功能均为ＮＹＨＡⅢ级,结果表明,ＤＣＭ组     

复方丹参注射液治疗35例新生儿缺氧缺血性脑病的研究

本文通过复方丹参注射液与胞二磷胆碱治疗新生儿ＨＩＥ的对照研究，发现治疗组（３５例）治愈率８２．８６％较对照组（５３．５７％）非常显著增高（ｕ＝２．５８，Ｐ＝０．０１）；新生儿行为神经２０项评分异常率明显降低（ｕ＝２．４９，ｐ〈０．０５）；神经系统后遗症发生率下降；血清ＳＯＤ活性较治疗前显著增高（ｔ＝２．１４７，ｐ〈０．０５）；血清ｃｋ－１３Ｂ、ｃｄ－ＭＢ活性较治疗前显著下降ｃｔ＝２．４，ｐ〈０．０     

大剂量维生素C在新生儿再灌注损伤中的应用

观察了大剂量维生素Ｃ（ＶｉｔＣ）用于新生儿再灌注损伤的疗效。结果ＶｉｔＣ１ｇ／（ｋｇ·ｄ）的疗效明显优于０．５ｇ／（ｋｇ·ｄ）。前者首次用药后患儿血清丙二醛（ＭＤＡ）明显减少，总超氧化物歧化酶（ＳＯＤ）明显增加（Ｐ＜０．００１），血液酸度无明显变化（ｐＨ：Ｐ＞０．１，ＨＣＯ３－：Ｐ＞０．０５，ＢＥ；Ｐ＞０．１）。而后者首次用药后患儿血清ＭＤＡ无明显减少（Ｐ＞０．０５），ＳＯＤ虽明显增加（Ｐ＜０．０１），但增加的幅度明显低于前者，提示ＶｉｔＣ作为自由基清除剂治疗新生儿再灌注损伤时，剂量以１ｇ／（ｋｇ·ｄ）为宜。     

新生儿高胆红素血症及胆红素脑病闪光视觉诱发电位的变化

目的　观察新生儿高胆红素血症及胆红素脑病闪光视觉诱发电位的改变。方法　应用日本产 Ｎ Ｅ Ｕ Ｒ Ｏ Ｐ Ａ Ｃ Ｋ Ｖ 型诱发电位仪对３０ 例高胆红素血症新生儿、９ 例胆红素脑病新生儿及２０ 例正常新生儿进行闪光视觉诱发电位（ Ｆ Ｖ Ｅ Ｐ） 检测。结果　高胆红素组与对照组比较,两组间 Ｆ Ｖ Ｅ Ｐ 变化无显著性差异（ Ｐ＞ ００５） ;胆红素脑病组较对照组 Ｖ 波潜伏期显著延长（ Ｐ＜ ００１） 及ⅢⅤ波间期显著延长（ Ｐ＜ ００５） 。结论　 Ｆ Ｖ Ｅ Ｐ 可较灵敏地反映胆红素脑病中枢神经功能状态的变化,似可作为早期监测胆红素脑病的方法。     

脐血输注在急性髓系白血病患儿化疗后骨髓抑制期中的作用

为探讨脐血对急性髓系白血病（ＡＬＬ）强烈化疗后骨髓抑制期的辅助及支持治疗作用，用密闭式采血法采集ＣＢ，一个胎盘所采集ＣＢ为１个单位。对４２例ＡＭＬ患儿采用ＨＡＥ／ＤＡＥ方案化疗，其中治疗组在强烈化疗后，短期内多个ＣＢ连续输注，平均４￣５个单位ＣＢ。结果治疗组骨髓抑制期天数短于对照组（Ｐ〈０．０１），骨髓抑制程度治疗组较轻，治疗组血小板和中性粒细胞相对较高（Ｐ〈０．０１）。并且治疗组感染和出血发生率     

甲基强的松龙为主治疗急性哮喘重度发作（附27例分析）

目的 探讨甲基强的松龙（ＭＰ）对小儿急性哮喘的重度发作的疗效。方法 在常规治疗基础上，治疗组２７例患儿序贯用ＭＰ５、４、４……ｍｇ／（ｋｇ．ｄ），对照组３１例予琥珀酸氢化可的松１２～２０ｍｇ／（ｋｇ．ｄ），均静脉滴注３～７天。结果 治疗组疗效显著优于对照组（Ｐ〈０．０５），治疗组用药７天临床症状全部消失。结论 患者经济条件许可时，对急性哮喘重度发作可首选ＭＰ。     

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.     

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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     

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.     

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.     

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   

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Overcoming surfactant inhibition with polymers

Inhibition of the function of pulmonary surfactant in the alveolar space is an important element of the pathophysiology of many lung diseases, including meconium aspiration syndrome, pneumonia and acute respiratory distress syndrome. The known mechanisms by which surfactant dysfunction occurs are (a) competitive inhibition of phospholipid entry into the surface monolayer (e.g. by plasma proteins), and (b) infiltration and destabilization of the surface film by extraneous lipids (e.g. meconium-derived free fatty acids). Recent data suggest that addition of non-ionic polymers such as dextran and polyethylene glycol to surfactant mixtures may significantly improve resistance to inhibition. Polymers have been found to neutralize the effects of several different inhibitors, and can produce near-complete restoration of surfactant function. The anti-inhibitory properties of polymers, and their possible role as an adjunct to surfactant therapy, deserve further exploration.     

Understanding infant formula

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and healthcare professionals) will experiment with the infant formula available and then attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.