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.     

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］     

高胆红素血症新生儿脑脊液神经元特异性烯醇化酶和胆红素水平变化的意义

目的了解足月新生儿胆红素脑损伤时脑脊液（CSF）神经元特异性烯醇化酶（NSE）是否增高，探讨CSF中NSE水平（CSF-NSE）与NBNA评分、CSF中胆红素（CSF-BIL）及血清间接胆红素水平（s-UCB）等的相关性。方法随机选择日龄3～7d的足月新生儿黄 疸39例，根据NBNA评分和核黄疸痉挛期的诊断，分为NBNA评分正常组（17例）、NBNA低分组（15例）和核黄疸痉挛期组（7例）；测定CSF-NSE、CSF-BIL和s—UCB水平。结果3组CSF-NSE、CSF-BIL和s-UCB均有显著性差异（P均〈0．01）；两两比较CSF-NSE均有显著性差异（P均〈0．05）；NBNA评分正常组CSF-BIL与NBNA低分组差异无显著性意义（P=0．259）；NBNA评分正常组s-UCB与NBNA低分组差异无显著性意义（P=0．279）；CSF-NSE和NBNA评分、CSF-NSE、s-UCB相关（r=-0．879，0519，0．661P均〈0．01）。发生胆红素脑损伤时CSF-NSE平均值的95％可信区间〉14．93μg／L。结论测定CSF-NSE对新生儿高胆红索血症脑损伤诊断具有较重要价值；足月高胆红素血症新生儿以NBNA评分可推测CSF-NSE水平；NBNA低分时，可能存在胆红素脑损伤。     

新生儿高胆红素血症对肾功能的影响及血β_2微球蛋白测定的临床意义

目的 了解新生儿高胆红素血症对肾功能的影响以及测定血β2微球蛋白的临床意义.方法 对79例高胆红素血症和43例非高胆红素血症的新生儿进行血尿素氮、肌酐和β2微球蛋白测定.结果 高胆组和非高胆组新生儿血尿素氮、肌酐值均在正常范围,两组比较差异无统计学意义(P均>0.05);高胆组血β2微球蛋白浓度(4.06±0.73)mg/L高于非高胆组(2.01±0.76)mg/L,差异有高度统计学意义(P<0.001);中度高胆组(4.52±0.35)mg/L和轻度高胆组(3.35±0.41)mg/L 比较差异有高度统计学意义(P<0.001).重度高胆组(4.75±0.30)mg/L和中度高胆组比较差异有统计学意义(P<0.05);血胆红素水平与血β2微球蛋白浓度经线形相关分析呈高度直线正相关(r=0.86,P<0.001).结论 新生儿高胆红素血症时β2微球蛋白明显增高,存在肾小球滤过率的下降,并随着血胆红素浓度的增加而加剧,血尿素氮、肌酐不能反映此种变化;对新生儿高胆红素血症应予以积极干预并避免使用肾毒性药物,以免加重肾脏损害.     

新生儿高胆红素血症中胱抑素C的变化及临床意义

目的 了解胱抑素C(CysC)在新生儿高胆红素血症中的变化.方法 104例高胆红素血症新生儿为高胆组,根据其血总胆红素水平分为轻、中、重3组,各28、58、18例;同时选择40例非高胆红素血症新生儿为非高胆组.测定血尿素氮(BUN)、肌酐(Cr)和CysC,并比较高胆组与非高胆组,以及轻、中、重高胆组新生儿之间的差异;分析各指标与血总胆红素的相关性.结果 高胆组和非高胆组新生儿的BUN、Cr均在正常范围,差异无统计学意义(P ＞ 0.05);高胆组新生儿的CysC高于非高胆组,差异有统计学意义(P ＜ 0.05);轻、中、重度高胆组新生儿的CysC逐渐增高,差异有统计学意义(F ＝ 5.39,P ＜ 0.05).新生儿的血总胆红素水平与CysC水平呈直线正相关(r ＝ 0.3,P ＜ 0.05).结论 高胆红素血症时,新生儿的CysC增高并随着血总胆红素水平升高而加剧,可能成为判断高胆红素血症时早期肾功能损害的敏感生物学指标.     

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。     

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。     

尿系列酶测定对高胆红素血症新生儿肾小管功能的评价

目的探讨尿系列酶的改变作为评价高胆红素血症(高胆)新生儿肾小管功能的指标.方法检测38例高胆新生儿尿系列酶(NAG、GAL、GGT、ALP、LDH)活性与50例正常对照;其中32例不同程度高胆患儿进行治疗前后尿系列酶对照分析.结果高胆新生儿尿酶NAG、GAL、ALP、LDH活性与对照组比较明显增高,出现显著性差异(P＜0.01);三组不同程度高胆新生儿NAG、ALP、LDH活性比较均出现统计学差异(P＜0.01),治疗后轻、中度黄疸ANG、ALP、LDH活性与治疗前比较明显减低,出现显著性差异(P均＜0.05),重度黄疸NAG、ALP、LDH与治疗前比较无明显差异,P＞0.05;血清总胆红素水平与NAG、ALP、LDH呈正相关关系.结论尿系列酶改变可以反映高胆新生儿时肾小管功能不同程度的受损;轻、中度高胆新生儿肾小管功能受损是可逆的,重度肾损害恢复慢.     

新生儿高胆红素血症血清神经元特异性烯醇化酶测定的临床价值

目的研究新生儿特异性烯醇化酶(NSE)与新生儿高胆红素血症之间的关系,以探讨高胆红素血症新生儿脑损害早期诊断的客观依据.方法利用放免法对124例高胆红素血症足月新生儿及40例正常足月新生儿进行血清NSE测定,对81例NSE升高的新生儿进行脑干听觉诱发电位(BAEP)测试,并于生后12～18个月进行智能测定.结果NSE水平在胆红素重度升高、中度升高、轻度升高组均增高,与对照组之间差异有显著性,P＜0.01.BAEP异常率、智能发育指数(PDI)及运动发育指数(MDI)下降在轻度升高组与对照组、重度升高组与中度升高组之间差异有显著性,但在轻度升高组与中度升高组之间差异无显著性.结论NSE水平的测定较以BAEP及智能测定来判断新生儿高胆红素脑损害更早、更敏感,NSE可作为判断高胆红素血症新生儿发生脑损害早期预后指标之一.     

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。     

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.