新生儿缺氧缺血性脑病早期康复干预的临床疗效观察

目的　研究早期康复干预对新生儿缺氧缺血性脑病 (HIE)的临床疗效。对象　临床诊断HIE的足月儿4 1例 ,随机分为干预组 (2 1例 )和非干预组 (2 0例 )。方法　干预组接受早期训练 ,定期随访以CDCC婴幼儿智能发育检查量表进行智能发育评估 ,根据评估的结果进一步指导干预 ,在 12～ 18个月对所有患儿以Gesell发育诊断量表进行智能发育最终评估 ,同时进行常规体格检查和育儿指导。结果　 3月龄、6月龄时干预组CDCC量表的智力发育指数均高于非干预组 (P <0 0 5 ) ;两组心理运动发育指数无显著性差异 (P >0 0 5 )。 1岁时Gesell智能检查干预组总发育商 (DQ)和言语能DQ高于非干预组 (P <0 0 1) ;而两组动作能、应物能、应人能DQ无显著性差异 (P >0 0 5 )。结论　对HIE患儿进行早期干预可改善预后。     

早期综合干预促进缺氧缺血性脑病患儿智力发育临床研究

目的探讨早期综合干预对改善HIE患儿预后的效果,以及通过早期综合干预可达正常的患儿所具备的条件,从而早期预测神经系统后遗症,最大限度地改善患儿的预后。方法将96例中、重度HIE患儿随机分为干预组及对照组。干预组予以早期干预为主,辅以医学干预、营养干预及康复治疗的早期综合干预;对照组出院后常规育儿。3、6、12、24个月均进行智测。结果6、12、24个月时干预组平均发育商比对照组分别高12.4分、15.1分、27.0分,t值分别为3.89、4.75、8.89,差异有显著性,干预组随着干预时间延长,发育商越高。新生儿神经行为测定(NBNA)评分30～34分的患儿,经综合干预2年发育商均可达正常,而≤29分的患儿,CT和临床多呈重度改变,且伴有脑瘫,经2年干预未达正常。干预组发育商达正常(DQ>90)者为84.0%,对照组为34.8%,其中中度干预组患儿100.0%达正常,对照组40.0%达正常,差异极显著。结论早期综合干预能有效地促进HIE患儿的智能发育,7～14dNBNA评分30～34分的患儿干预效果良好;7～14dNBNA评分≤29分、CT和临床呈重度改变的患儿将发生神经系统后遗症,干预效果较差。     

早期干预对改善脑损伤围生儿智能发育的效果

目的探讨早期干预和康复治疗对围生儿脑损伤智能发育的疗效。方法将50例围生期脑损伤患儿随机分为干预组27例和对照组23例,干预组从新生儿期接受早期干预,对照组按常规育儿,两组从新生儿起观察至18个月,并使用Gesell发育量表测查精神运动发育商(DQ)。结果干预组6、12个月DQ显著高于对照组(6个月龄时P<0.05,12个月龄时P<0.01)。干预组后遗症发生率为3.7%(1/27),对照组后遗症发生率为26.08%(6/23),两组比较差异有显著意义(χ2=5.17 P<0.05)。结论对围生儿脑损伤予以正确的早期干预能有效地促进其智能发育,是改善预后、降低后遗症发生率的有效手段。     

综合康复治疗小儿脑性瘫痪242例疗效观察

目的观察小儿脑性瘫痪(简称脑瘫)综合康复治疗的疗效。方法将242例脑瘫患儿分为观察组及对照组,观察组122例患儿进行有针对性的药物治疗、运动治疗、作业治疗、语言矫治训练、引导式教育及中医针灸、按摩等综合康复治疗;对照组120例只采用单一的药物治疗,14 d为1个疗程,共用3~5个疗程。观察并比较两组及观察组不同年龄段患儿的疗效。结果观察组脑瘫患儿经综合康复治疗后,发育指数明显高于对照组,差异有统计学意义(P<0.05,0.01);观察组中0~6个月患儿总有效率为96.77%(30/31),~12个月为94.03%(63/67),~3岁为84.62%(11/13),~5岁为63.64%(7/11),各年龄段间疗效比较差异有统计学意义(P<0.01)。结论综合康复治疗脑瘫患儿疗效显著,早期诊断、早期干预治疗,效果更好。     

新生儿缺氧缺血性脑病早期干预及预后分析

目的探讨早期干预对新生儿缺氧缺血性脑病(HIE)预后的影响.方法对56例中重度HIE患儿从恢复期起随机分成干预组和对照组.所有病例均于出生3,7,12月龄时进行智能发育随访和检测.结果干预组发育商(DQ)明显高于对照组(t=2.2983,2.8303,2.8576,P＜0.05、0.01、0.01),其康复率高,后遗症发生率低(χ2＝4.7640,P＜0.05).结论对HIE患儿早期给予持续的干预,确能有效地促进其智力发育,是改善预后、减低后遗症发生率的有效手段.     

早期干预对早产儿生存质量影响的研究

目的探讨早期干预对提高早产儿生存质量的作用。方法对128例早产儿采用医务人员和家长相结合的摸式,按照鲍秀兰教授主编的0~3岁教育大纲进行早期干预(教育),设为干预组;90例出院后家长拒绝干预的早产儿设为对照组。对两组患儿定期随访,进行体格发育检查和应用Gesell婴幼儿发育量表测试发育商(DQ)。对脑损害较重的患儿给予新生儿期后的继续治疗。对两组早产儿随访结果进行对比分析。结果干预组一周岁半时,除一例伤残儿外,体重、身长、头围均达正常,而对照组有17例体重或身长低于正常(P<0·01);干预组各个能区的DQ均高于对照组(P均<0·01),一周岁半时平均总DQ(103·1±10·3)明显高于对照组的(88·7±10·7),差异有统计学意义(t=9·61,P<0·01),干预组总DQ<70仅1例(1/128,0·78%),而对照组有6例(6/90,6·67%),差异有统计学意义(χ2=5·9,P<0·05);干预组脑瘫发生率0·78%(1/128),低于对照组的3·33%(3/90)。差异有统计学意义(χ2=7·75,P<0·01)。结论医务人员和家长密切配合,对早产儿早期干预(教育),可促进体格和智能发育,减少伤残,提高生存质量。     

300例语言障碍儿童的智能发育水平分析

目的 了解语言障碍儿童的智能发育特征。方法 对300例语言障碍儿童进行Gesell发育量表评估,了解其运动能、应物能、言语能、应人能4个能区的发育商。结果 300例儿童的运动能平均发育商正常,应物能、言语能及应人能平均发育商均低于正常。运动能、应物能、应人能异常的比例分别为31.0%、49.0%、52.7%。言语能发育商与其他3个能区发育商显著正相关 （r分别为0.506、0.644、0.711,P < 0.01）。结论 语言障碍儿童往往不是单纯的语言落后,而是常伴随运动能力、适应行为及社交行为等方面的落后,应对其进行诊断性的智能发育评估并对伴随的其他发育异常问题进行全面干预。     

睡姿矫正法治疗婴儿姿势性斜头疗效观察

目的研究2个月疗程的睡姿矫正法对8月龄前婴儿姿势性斜头转归的影响。方法选取2015年1月至2016年6月就诊的姿势性斜头患儿73例为研究对象,按家长意愿分成治疗组(n=46)和对照组(n=27)。治疗组采用睡姿矫正法矫正斜头;对照组予安曲婴儿正姿床垫矫正斜头。测量并计算两组患儿治疗前后的经颅斜径和经颅斜径差(CVA),根据CVA对治疗前后两组患儿斜头病情程度进行比较;采用Gesell儿童发育量表对两组患儿治疗前后4大能区发育商(DQ)进行评估。结果治疗前两组斜径A、斜径B、CVA,以及动作能、应物能、语言能、应人能DQ值比较差异均无统计学意义(P0.05);治疗2个月后,治疗组斜径B长于对照组(P0.05),CVA小于对照组(P0.01),各能区DQ值与对照组比较差异均无统计学意义(P0.05)。两组治疗后斜径A、斜径B、CVA、运动能DQ值、应物能DQ值,以及治疗组治疗后应人能DQ值均较治疗前有所改善(P0.01)。两组间斜头病情程度比较在治疗前后差异均无统计学意义(P0.05)。结论应用睡姿矫正法治疗婴儿姿势性斜头,较英国进口的安曲婴儿正姿床垫疗效好,且更简便、经济,值得临床推广。     

婴儿脑性瘫痪的病因与防治探讨(附128例临床分析)

目的：探讨婴儿脑性瘫痪的病因及防治方法。方法：对1998年4月至2000年6月确诊的128例婴儿脑性瘫痪患儿分为干预组30例,未系统干预组35例,门诊转诊的63例为对照组。3组其性别、胎龄、出生体重、病情程度经统计学检验均无显著性差异,采用体格发育、神经系统检查及中国儿童智能发育量表评定发育商。结果：①发病原因依次为缺氧缺血性脑病(HIE)72例(56.2%)及颅内出血(ICH)12例(9.3%),高胆红素血症(高胆血症)32例(25%),感染7例(6.5%),早产儿5例(3.9%)。②干预组的体重、头围明显大于未系统干预组及对照组。③干预、未系统干预及对照3组智力发育指数(MDI)各为(75.6±12.7),(38.4±13.8),(49.7±13.6),(P<0.01);精神运动发育指数(PDI)各为(83.3±15.3),(40.6±17.6),(51.4±14.4),(P<0.01),干预组明显高于未系统干预及对照组。结论：防治的重点应是预防及治疗HIE,ICH,高胆血症,感染及早产。早期干预和新生儿期后治疗以及水疗指针疗法的综合干预可以改善婴儿脑瘫的预后。     

头穴透刺对脑性瘫痪患儿血清白细胞介素-6、肿瘤坏死因子-α水平的影响

目的探讨头穴透刺对脑性瘫痪(CP)患儿血清IL-6、TNF-2水平的影响。方法选择CP患儿160例,随机分为透刺组和西药组。透刺组80例,采用头部腧穴透刺治疗,百会透太阳穴;西药组80例,采用静脉滴注神经营养药物;另选门诊正常体检儿童50例为健康对照组。采用放射免疫分析法检测各组儿童血清IL-6、TNF-α水平。用Gesell发育量表对CP患儿进行治疗前后发育龄各能区(应物能、动作能、言语能、应人能)发育商水平进行动态评估。结果透刺组治疗后显效48例,有效30例,无效2例,总有效率为97.5%;西药组显效25例,有效32例,无效23例,总有效率为71.25%,二组总有效率比较有显著性差异(Z=-7.276P<0.05)。透刺组治疗后发育商DQ值为(68.04±15.54)分,西药组为(57.94±12.84)分,二组比较有显著性差异(P<0.01)。透刺组治疗后IL-6水平为(91.26±32.19)ng/L,西药组为(130.53±31.24)ng/L,二组比较有显著性差异(P<0.01)。透刺组治疗后TNF-α水平为(0.93±0.33)ng/L,西药组为(1.20±0.40)ng/L,二组比较有显著性差异(P<0.01)。结论头穴透刺是促进CP患儿康复的有效治疗方法之一,可调节CP患儿的神经免疫功能。     

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.