促红细胞生成素对早产儿脑损伤神经行为的影响

目的 探讨重组人类基因促红细胞生成素(EPO)对早产儿脑损伤保护作用及临床疗效.方法 将42例发生脑室周围白质软化(PVL)脑损伤的早产儿(男25例,女17例)随机分为EPO治疗组(22例)和常规治疗组(20例),治疗组于生后3～5 d予以EPO治疗4周.所有患儿于纠正胎龄40周进行新生儿行为神经检测(NBNA),随诊至6个月,定期行头颅影响学检查、脑电图检查,3、6个月时分别进行发育商(DQ)测定.结果 治疗组患儿NBNA评分及DQ评估均明显高于对照组,差异有显著性(P＜0.05).结论 EPO能促进神经系统损伤修复,对脑损伤的早产儿神经行为发育有促进作用,可改善预后.

Abstract：

Objective To study the clinical curative and protective effects of erythropoietin (EPO)on treating the premature infants with brain damage. Methods Forty-two premature infants with periventricular leukomalacia were divided into the EPO treatment group (n = 22) and control group (n = 20). From postnatal day 3 to 5 ,the EPO treatment group received EPO for 4 weeks. Neonatal behavioral neurological assessments (NBNA) for all infants were performed separately in 40 weeks after correcting gestational age. All infants were followed up until 6 month after birth, MRI and electroencephalogram were performed. The developmental quotient(DQ) was evaluated at the age of 3 and 6 months. Results The NBNA scores of EPO group in 40 weeks and DQ were significantly higher than those of control group (P ＜ 0.05). Conclusion EPO can rehabilitate the damaged neurological system of premature infants with brain damage. Early EPO therapy can promote development of the neurobehavioral and improve prognosis.     

脑室周围白质软化早产儿126例

目的 探讨早产儿脑室周围白质软化(PVL)的治疗措施.方法 PVL早产儿126例按家长意愿分为干预1组(57例)、干预2组(35例)和常规育儿组(34例).干预1组予脑细胞保护剂,接受常规育儿指导及早期干预治疗.干预2组接受常规育儿指导外,进行针灸及按摩传统中医治疗.常规育儿组只接受常规育儿指导.3组患儿均在纠正胎龄后3、9、12、24、30个月行智力检查.纠正胎龄9个月后行神经运动检查.结果 干预1组患儿智力发育及运动发育指数明显高于常规育儿组(Pa<0.05);与干预2组比较,智力发育指数明显提高(Pa<0.05),纠正胎龄9个月后运动发育指数无显著性差异(Pa0.05).3组间脑性瘫痪发病率比较均无显著差异(Pa0.05).结论 早期综合治疗早产儿PVL有一定疗效,可促进智力发育及运动发育,减少认知、行为缺陷,减轻运动障碍,但不能降低PVL脑性瘫痪发生率.     

影响早产儿行为神经测定因素的分析

目的 探讨新生儿行为神经测定(NBNA)在早产儿的应用及影响其评估指标的因素.方法 选择2006年1月至2007年6月在我院新生儿科住院治疗的123例早产儿,分别在纠正胎龄40周时进行NBNA评分,分析胎龄、出生体重、是否机械通气以及机械通气时间、围生期是否有致脑损伤的高危因素对NBNA评分的影响.结果 胎龄<30周、～32周、～34周早产儿及出生体重<1 250 g、～1 500 g、～2 000 g早产儿NBNA评分差异均具有显著性(P<0.01),胎龄越低、出生体重越低,NBNA评分越低;需机械通气的早产儿NBNA评分明显低于未上呼吸机者(P<0.01),机械通气时间＞7 d NBNA评分明显低于＜7 d者(P<0.05);围生期有出血性和缺血性脑损伤、间接胆红素≥256.5 μmol/L、血糖反复<2.6 μmol/L、发生感染者NBNA评分明显降低(P<0.01);臀位、钳产、吸引产早产儿NBNA评分明显低于剖宫产和顺产者(P<0.01);Apgar评分为0～3分者NBNA评分明显低于4～7分和≥8分者(P<0.01).结论 胎龄、出生体重、是否机械通气、机械通气时间、异常的分娩方式、合并重度窒息均影响早产儿的NBNA评分.对于早产儿,特别是胎龄<32周,出生体重<1 500 g,存在致脑损伤高危因素的早产儿,生后3～7 d内应行头颅B超检查,及早发现颅内病变,尽早干预,减少后遗症的发生.     

重组人促红细胞生成素对极低出生体重儿神经智能发育的影响

目的 评估早期给予重组人促红细胞生成素（rhEPO）对极低出生体重儿（VLBWI）神经智能发育的临床疗效。方法 选取VLBWI 78例,根据患儿父母的选择分为rhEPO治疗组（n=35）与对照组（n=43）。治疗组生后4~5 h内给予rhEPO（250 IU/kg,每周3次,连用4周）。纠正胎龄40周时行新生儿神经行为检测（NBNA）,纠正胎龄3月、6月、12月时进行Gesell发育量表评估,并比较纠正胎龄6月时脑干诱发电位（ABR）及头颅B超的异常率。结果 治疗组纠正胎龄40周的NBNA评分高于对照组（P<0.05）。治疗组纠正胎龄3月时的适应能力优于对照组,纠正胎龄6月时的大运动、适应能力、社交能力优于对照组,纠正胎龄12月时的大运动、适应能力、精细动作、社交能力、语言明显优于对照组,差异均具有统计学意义（均P<0.05）。治疗组纠正胎龄6月时的ABR异常率、头颅B超异常率明显低于对照组（P<0.05）。结论 早期rhEPO治疗可以促进VLBWI神经系统症状早期恢复,改善患儿的认知、运动及语言能力,对神经系统具有一定的保护作用。     

脑电图在早产儿脑损伤早期诊断中的应用价值

目的 研究早产儿脑电图及头颅CT检查结果的相关性,探讨脑电图在早产儿脑损伤早期诊断中的应用价值.方法 所有患儿在出生24~72h行脑电图检查,根据检查结果分为脑电图正常组,脑电图轻度异常组和脑电图重度异常组.患儿于纠正胎龄40周行头颅CT检查,以了解其脑损伤情况,比较脑电图与头颅CT结果的相关性.结果 对两种检查结果进行行×列表的χ2检验,χ2=9.432,P<0.05,提示早产儿脑电图结果与头颅CT检查结果差异具有统计学意义.结论 脑电图对于早产儿脑损伤的早期诊断具有临床价值,加之脑电图检查方便、经济、实用、无创伤,并且易于重复,值得临床推广.     

早产儿血清促红细胞生成素水平与脑损伤的关系

目的 探讨早产儿血清促红细胞生成素（EPO）水平与脑损伤的关系。方法 选取2014 年10月至2015 年9 月出生胎龄在28~34 周的早产儿304 例作为研究对象。采用颅脑B 超和MRI 检查诊断脑损伤,ELISA 检测血清EPO、S100 蛋白、神经元特异性烯醇化酶（NSE）和髓鞘碱性蛋白（MBP）水平,比较不同血清EPO 水平早产儿脑损伤的发生率,分析血清EPO 水平与各指标的相关性;采用多因素Logistic 回归分析研究血清EPO 水平与脑损伤的关系。结果 304 例早产儿中发生脑损伤125 例（41.1%）;低水平EPO 组缺血性脑损伤发生率明显高于中高水平EPO 组（P P P P 结论 血清EPO 低水平的早产儿脑损伤发生率高,血清EPO 水平与早产儿脑损伤密切相关。     

未足月胎膜早破对早产儿神经发育的影响

目的探讨未足月胎膜早破对早产儿神经发育的影响。方法将早产儿根据产前有无胎膜早破分为未足月胎膜早破组（n=40）和对照组（n=37）。分别对二组患儿在纠正胎龄至40周、纠正胎龄后3个月、纠正胎龄后6个月进行新生儿神经发育评分（NBNA）及婴幼儿智能运动发育检测（CDCC）,比较二组神经发育情况。结果未足月胎膜早破组3个月及6个月运动心理发育指数（PDI）明显低于对照组,经统计学分析有显著性差异（Pa〈0.05）。二组NBNA评分、3个月及6个月智能发育指数（MDI）比较则无明显统计学差异（Pa〉0.05）。结论未足月胎膜早破对早产儿远期运动心理发育有明显的延迟和滞后的影响。     

促红细胞生成素通过早产儿血脑脊液屏障的观察

目的研究外源性重组人促红细胞生成素(rhu—EPO)能否通过早产儿血脑脊液屏障。方法将胎龄28-35周、体质量<2500g的早产儿随机分为治疗组20例,对照组16例。治疗组给予rhu-EPO750IU/(kg·周).3次/周,隔日1次,疗程2周;对照组按早产儿常规治疗。用酶联免疫法测两组早产儿治疗前后血清、脑脊液EPO浓度。结果1.治疗组治疗2周后血清、脑脊液EPO浓度均明显高于对照组(P均<0.01)。2.治疗组治疗前后血清、脑脊液EPO浓度相比,治疗后明显高于治疗前(P<0.01)。3.对照组血清、脑脊液EPO浓度在2周后同出生时相比均有所降低,但无显著差异(P>0.05)。结论外源性应用rhu—EPO能通过早产儿血脑脊液屏障。     

MRI和DTI评价早产儿脑白质髓鞘发育

目的 应用磁共振(MRI)、磁共振弥散张量成像(DTI)研究早产儿脑白质髓鞘发育的特点。方法 胎龄≤32周、出生体重<1 500 g的31例早产儿根据头部MRI检查分为早产脑损伤组(12例)和早产无脑损伤组(19例)。选取24例足月儿作为对照组。均于胎龄或纠正胎龄37~40周之间完成头部MRI及DTI检查。测定3组相同感兴趣区的部分各向异性参数(FA)和表观扩散系数(ADC)。结果 早产脑损伤组内囊后肢FA值小于早产无脑损伤组和足月对照组 (P < 0.05);早产脑损伤组和早产无脑损伤组的额叶白质和豆状核的FA值小于足月对照组 (P < 0.05);3组间枕叶白质的FA值差异无显著性 (P > 0.05)。早产脑损伤组和早产无脑损伤组内囊后肢、豆状核、枕叶白质、额叶白质的ADC值高于足月对照组 (P < 0.05)。结论 早产儿脑损伤容易出现内囊后肢深部脑白质髓鞘化障碍或延迟。早产儿至纠正胎龄足月时,无论有无脑损伤,脑周围白质及灰质成熟度均低于足月儿。     

产前应用单疗程及多疗程地塞米松对早产儿脑发育的影响

目的 探讨产前应用单疗程及多疗程地塞米松对早产儿脑发育的影响.方法 选择本院2005年8月-2007年3月NICU收治的28～34周的早产儿118例,按母亲产前应用地塞米松的情况分为对照组、单疗程组、多疗程组.对照组未用地塞米松;单疗程应用地塞米松10 mg,1次/d,肌肉注射,3 d为1个疗程,仅用1个疗程;多疗程组应用地塞米松2个疗程以上.3组早产儿出生后均予对症支持治疗.纠正胎龄至预产期后行新生儿20项行为神经测定(NBNA),纠正胎龄至出生3、6、12个月时采用中国儿童发展中心(CDCC)制定的婴幼儿智力发育指数(MDI)和运动发育指数(PDI)量表进行检测.采用SPSS 11.0软件进行统计学分析.结果 纠正胎龄达预产期时及达预产期后7 d单疗程组NBNA评分明显高于对照组及多疗程组(Pa<0.05).纠正胎龄至出生3、6个月时行CDCC评分,单疗程组MDI和PDI均明显高于对照组及多疗程组(Pa<0.05);12个月多疗程组MDI和PDI明显低于单疗程组及对照组(Pa<0.05),单疗程组显著高于对照组(Pa<0.05).结论 产前单疗程应用地塞米松能明显提高早产儿近、远期预后;多疗程GCS对早产儿神经系统近、远期发育均有一定的不良影响.     

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.