新生儿高胆红素血症患儿NBNA与BAEP评价

目的　 探讨新生儿高胆红素血症患儿脑损伤的早期预测方法。 方法 　对 38例高胆患儿分别进行新生儿神经行为评分 (NBNA)和脑干听觉诱发电位 (BAEP)检测。 结果 　高胆组BAEP异常率明显高于对照组 (P <0 0 1) ,Ⅰ、Ⅴ波潜伏期与对照组比较差异显著 (P <0 0 1,P <0 0 5 )。高胆组NBNA评分明显低于对照组 (P <0 0 0 1) ,主要失分项目为行为能力和主动肌张力。在血清胆红素≥ 2 0 4μmol/L时 ,胆红素水平与NBNA评分呈显著负相关 (P <0 0 1) ,血清胆红素浓度≤ 2 5 6 5 μmol/L时 ,部分高胆患儿NBNA评分即有降低 ,BAEP异常率增高 ,黄疸消退后NBNA评分全部恢复正常。 结论 　NBNA和BAEP是早期诊断新生儿胆红素脑病敏感而有效的方法 ,有助于判断病情及指导治疗。     

高胆红素血症对新生儿神经行为及其预后的影响

探讨高胆红素血症对新生儿神经行为及其预后的影响,通过对38例高胆患儿在新生儿期进行神经行为评分(NBNA)检测、婴幼儿期用婴幼儿智力发育量表(CDCC)测定智力发育。结果显示:高胆组NBNA评分明显低于对照组(P<0.001),主要失分项目为行为能力和主动肌张力。在血清胆红素≥204μmol/L时,胆红素水平与NBNA评分呈显著负相关(P<0.01);围产因素、溶血、感染组在黄疸高峰期NB-NA评分低于对照组(P<0.01)。高胆组MDI、PDI与对照组比较差异显著(P<0.001),智能分级比较,对照组优于高胆组(P<0.01,P<0.05)。结果提示,不同程度高胆红素血症无论是不同龄别生理性特点或为不同而更较复杂的病理特异,均可能影响新生儿神经行为能力和智能发育。对新生儿高胆红素血症,无论其程度和病因如何均应积极治疗,尽量减少后遗症的发生。     

高胆红素血症对新生儿神经行为及其预后的影响

探讨高胆红素血症对新生儿神经行为及其预后的影响,通过对38例高胆患儿在新生儿期进行神经行为评分(NBNA)检测、婴幼儿期用婴幼儿智力发育量表(CDCC)测定智力发育.结果显示高胆组NBNA评分明显低于对照组(P＜0.001),主要失分项目为行为能力和主动肌张力.在血清胆红素≥204 μmol/L时,胆红素水平与NBNA评分呈显著负相关(P＜0.01);围产因素、溶血、感染组在黄疸高峰期NB-NA评分低于对照组(P＜0.01).高胆组MDI、PDI与对照组比较差异显著(P＜0.001),智能分级比较,对照组优于高胆组(P＜0.01,P＜0.05).结果提示,不同程度高胆红素血症无论是不同龄别生理性特点或为不同而更较复杂的病理特异,均可能影响新生儿神经行为能力和智能发育.对新生儿高胆红素血症,无论其程度和病因如何均应积极治疗,尽量减少后遗症的发生.     

新生儿20项行为神经测定、脑干诱发电位对诊断胆红素脑病的价值

目的　探讨新生儿 2 0项行为神经测定 (NBNA)和脑干诱发电位 (BAEP)对诊断胆红素脑病的意义。方法　 55例足月黄疸儿按血清胆红素 (TSB)分为 3组 ,检查其NBNA和BAEP。结果　 1 .实验组NBNA评分较对照组低 (P <0 .0 5) ;BAEP除Ⅰ波潜伏期 (PL)外 ,余各波潜伏期及波间期 (IPL)较对照组延长 (P <0 .0 5) ,BAEP异常率高于对照组 (P <0 .0 5)。 2 .NBNA评分、BAEP各波PL、IPL及其异常率均无显著性差异(P >0 .0 5) ;3 .实验组黄疸消退后NBNA评分普遍提高 ;BAEP除Ⅲ～Ⅴ波间期外 ,余各波PL及IPL缩短 (P<0 .0 5) ;4.实验组 60 %以上NBNA评分≤ 37分 ,BAEP异常率明显增高。结论　NBNA和BAEP可用于早期诊断胆红素脑病 ,应该对TSB >1 71 μmol/L、NBNA评分≤ 37分的新生儿检查BAEP ,并对NBNA、BAEP异常者积极干预。     

胰岛素样生长因子-1在新生儿高胆红素血症中的变化及意义

目的：胰岛素样生长因子-1(IGF-1)是神经系统必需的调节因子,目前少有报道其与高胆红素血症之间的关系。该文主要通过测定高胆红素血症（高胆）新生儿血清中IGF-1水平及新生儿神经行为评分(NBNA)来探讨IGF-1与高胆的关系及其临床意义。方法：应用电化学发光分析法检测57例高胆新生儿和 25例正常新生儿血清中IGF-1 浓度,同步测定血清总胆红素(TSB)、未结合胆红素(USB)及白蛋白(ALB)含量,计算USB与ALB比值(B/A),并行新生儿 NBNA 评分。高胆组按血清TSB值221~256 μmol/L,257~342 μmol/L,＞342 μmol/L分为轻、中、重三组;对照组TSB ＜85 μmol/L。结果：轻、中、重高胆患儿血清IGF-1浓度均值分别为39.38±8.42,30.77±4.65,26.34±2.05 ng/L,较对照组50.16±15.73 ng/ L明显降低,在轻、中、重高胆组间IGF-1浓度差异存在显著性(P＜0.01),其值随着胆红素的升高而降低;轻、中、重高胆组NBNA评分均值分别为35.01±2.26,32.45±2.74,26.77±5.02,明显低于对照组38.24±0.78(P＜0.01),高胆各组间差异也有显著性(P＜0.01);血清IGF-1 浓度与NBNA评分呈正相关(r=0.603, P＜0.01),与B/A值呈负相关(r=-0.483, P＜0.01)。结论：高胆患儿血清IGF-1浓度显著降低,降低程度与血清胆红素水平有关;IGF-1可能与新生儿胆红素脑损伤密切相关。［中国当代儿科杂志,2009,11（5）：357-360］     

新生儿行为神经评价与高胆红素血症的关系

目的　 探讨 2 0项新生儿行为神经测定 (NBNA)与高胆红素血症的关系 ,了解高胆红素血症对新生儿行为神经的影响。 方法 　对 62例高胆红素血症患儿运用NBNA。 结果 　①当血清胆红素水平≥ 2 0 5 μmol/L ,对NBNA即有显著影响 (P <0 0 1) ,血清胆红素≥ 3 4 2 μmol/L ,则与NBNA评分呈明显直线负相关关系 (r =-0 841,P <0 0 1) ;②溶血感染所致高胆红素血症NBNA评分均低于对照组 ,差异有显著意义 (P <0 0 5 ) ;母乳性黄疸NBNA评分与对照组无显著差异 ;③高胆红素血症主要影响患儿的视听定向能力。 结论 　提示高胆红素血症影响新生儿行为神经 ,对血清胆红素≥ 3 4 2 μmol/L ,应采取积极有效措施 ,以减少胆红素脑病的发生。     

新生儿高间接胆红素血症血清NSE含量变化及意义

目的 探讨高间接胆红素血症(高间胆)新生儿血清神经元特异性烯醇化酶(s-NSE)的变化及其与新生儿行为神经评分(NBNA)的关系及意义.方法 应用ELISA法分别测定71例高问胆新生儿和20例正常足月新生儿s-NSE含量,并对所有对象于生后第7天进行NBNA评分;按胆红素数值大小分为轻、中、重3组,按NBNA评分值分为＞36分、35～36分、＜35分3组.结果 高间胆新生儿s-NSE浓度明显高于对照组(P＜0.01),重度组高于轻度组(P＜0.01),轻度组与中度组、中度组与重度组之间差异均无统计学意义(P＞0.05);高间胆组新生儿NBNA评分显著低于对照组(P＜0.001);经直线相关分析,s-NSE含量与NBNA评分旱显著负相关(r=0.8843,P＜0.001),血清间接胆红素(s-UCB)与NBNA评分相关性较差(r=0.2756,P＜0.05),s-UCB与s-NSE无直线相关性.结论 s-NSE测定可以反映高胆红素血症新生儿脑损伤;临床以s-NSE含量为依据对新生儿高胆红素血症进行干预更合理.     

新生儿高胆红素血症患儿脑干听觉诱发电位及血清神经元特异性烯醇化酶变化及临床意义

目的通过对新生儿高胆红素血症患儿脑干听觉诱发电位(BAEP)及血清神经元特异性烯醇化酶(NSE)检测,评估高胆红素血症对新生儿听神经损伤。方法 56例血清胆红素大于 220．5μmol／L的新生儿为高胆红素血症组(简称高胆组),血清胆红素小于220．5μmol／L的49例足月儿为对照组,分别进行BAEP、NSE检测。结果高胆组新生儿BAEP的Ⅰ、Ⅲ、Ⅴ波绝对潜伏期(PL)、峰间潜伏期(IPL)均明显延长,与对照组比较,差异有显著性意义(P<0．01);高胆组新生儿NSE明显高于对照组,差异有显著性意义(P<0．01);NSE水平与BAEP的Ⅴ波反应阈值呈正相关(r=0．65,P<0．01)。结论高胆红素血症可导致新生儿听神经损伤;BAEP和NSE对其神经损伤有较高的敏感性,可作为监测指标。     

新生儿高胆红素血症行为神经测定及婴幼儿期智能发育随访

目的探讨高胆红素血症对新生儿神经行为能力及婴幼儿期智能发育的影响。方法对43例新生儿高胆红素血症（NHB）患儿，根据血清胆红素水平，将所有观察病例分为3组：NHBⅠ组（205．5～256．5μmol／L）15例，NHBⅡ组（256．5～341．5μmol／L）21例，NHBⅢ组（342．5μmol／L）7例。健康新生儿39例作为健康对照组。采用新生儿神经行为评分（NBNA）方法分别在生后3～7d（黄疸高峰期）、15～25d（黄疸消退后）进行检测，并在生后18个月时分别对二组受试婴儿进行智力评定[Gesell量表（中国标准化）]。结果NHB组NBNA明显低于健康对照组（P〈0．001），主要表现在行为能力和肌张力方面。NHB组婴幼儿期智能发育与健康对照组差异显著（P〈0．01），且早期新生儿NHB（〈7d）比晚期NHB对智能发育影响更大（P〈0．05）。结论NHB对新生儿神经行为及婴幼儿期智能发育有一定影响。     

脐血胆红素浓度测定对早期预测新生儿高胆血症的研究

目的　 早期预测新生儿高胆血症的发生。 方法 　测定 10 0例足月新生儿脐血胆红素浓度 ,同时用经皮胆红素测定仪 (TCBM )动态观察胆红素变化。 结果 　高胆组脐血胆红素浓度显著高于非高胆组 (P <0 0 0 1) ,脐血≥ 40 μmol/L ,黄疸发生率 61 5 %。 2 4hTCB读数≥ 16,高胆血症发生率为 72 2 % ,当脐血胆红素浓度≥ 40 μmol/L ,其特异性及阳性预测值提高到 90 %以上。 结论 　脐血胆红素浓度测定对早期预测新生儿高胆血症提供了可靠依据。     

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.     

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.     

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.