母乳性黄疸对肾功能的影响及其早期干预

目的探讨母乳性黄疸(BMJ)患儿肾功能变化及早期干预对肾功能的影响。方法测定50例BMJ患儿入院后12 h内血清胆红素和尿β2-徽球蛋白(β2-MG)、α1-MG、清蛋白(Alb)、IgG及出院前尿β2-MG、α1-MG、Alb、IgG,与20例足月新生儿生理性黄疽(对照组)对比。结果BMJ患儿随血清总胆红素升高,尿微量蛋白有升高趋势,血清胆红素205.2-256.5 μmol/ L时尿β2-MG较对照组明显升高(P<0.05);血清胆红素>256.6-342.O μmol/L,β2-MG、α1-MG显著升高(P均<0.01),Alb 较对照组明显升高(P<0.05)。出院前尿β2-MG、α1-MG、Alb恢复正常。结论BMJ可引起暂时肾功能损害,予早期干预治疗后肾功能很快恢复正常。     

单纯性肥胖儿童骨龄与体质量指数及性激素水平的相关性

目的 检测单纯性肥胖儿童的骨龄和性激素水平,分析骨龄与体质量指数(BMI)和性激素水平之间的关系.方法 选取40例单纯性肥胖患儿(肥胖组)及40例体检健康儿童(健康对照组).常规测量二组儿童身高和体质量,并计算BMI.采用G-P图谱法对左手腕指关节正位片进行骨龄评定;采用罗氏E170化学发光免疫分析仪测定二组儿童血清雌二醇、睾酮水平.比较二组儿童骨龄、BMI和性激素水平间的差异,并对骨龄与BMI及性激素水平进行相关性分析.结果 肥胖组骨龄[(11.85±2.76)岁]、BMI[(27.26±5.16) kg·m-2]及雌二醇[(29.20±11.61) pmol·L-1]均显著高于健康对照组[(10.57±2.59)岁,(16.75±4 73) kg·m-2,(22.35±10.87) pmol·L-1](t=10.57、9.50、2.72,P<0.05、0.01、0.01);肥胖组睾酮[(0.07±0.04) μg·L-1]显著低于健康对照组[(0.10±0.05) μg·L-1](t=2.96,P<0.01).肥胖组骨龄与BMI、雌二醇均呈显著正相关(r=0.696、0.773,Pa<0 05);与睾酮呈负相关(r=-0.726,P<0.05).结论 单纯性肥胖儿童的骨龄较同龄健康儿童超前,骨龄超前与体内雌激素水平有关,雌二醇水平的升高可能是导致单纯性肥胖儿童骨龄超前的主要原因.     

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目的 探讨运动、饮食、教育联合干预时单纯性肥胖儿童肾功能的影响.方法 分别对2005年5月至2008年3月浙江省温州市第二人民医院儿科门诊50例单纯性肥胖儿童单纯饮食控制治疗前后和52例单纯性肥胖儿童在饮食控制基础上再制定有计划运动及健康教育联合干预治疗前后肾功能进行测定,并对其疗效进行比较.结果 治疗前单纯治疗组、联合干预组尿白蛋白Alb、IgG、TRF均高于对照组,差异均有统计学意义(P<0.01),而单纯治疗组治疗前与联合干预组治疗前比较差异均无统计学意义(P>0.05).单纯治疗组治疗前后比较:Alb、IgG、TRF差异均有统计学意义(P<0.01).联合干预组治疗前后比较:Alb、IgG、TRF差异均有统计学意义(P<0.01).单纯治疗组治疗后、联合干预组治疗后与对照组比较:单纯治疗组治疗后各项指标差异仍有统计学意义(P<0.01),联合干预组治疗后各项指标差异均无统计学意义(P>0.05).结论 运动、饮食及健康教育联合干预时单纯性肥胖儿童的肾功能改善效果较好,对患儿以后提高生活质量有积极的作用.     

尿N-乙酰-β-D-氨基-葡萄糖苷酶及尿β2微球蛋白在过敏性紫癜肾损害早期诊断中的意义

目的探讨联合检测尿N-乙酰-β-D-氨基-葡萄糖苷酶(NAG)及尿β2微球蛋白(β2-MG)在过敏性紫癜(AP)肾损害早期诊断中的意义。方法以双夹心酶免疫法及放射免疫法检测94例AP患儿尿NAG、β2-MG水平。结果94例AP患儿中15例尿NAG异常,12例尿β2微球蛋白异常,5例尿NAG、β2微球蛋白均异常,尿NAG异常阳性率为15.96%(15/94),尿β2微球蛋白异常阳性率为12.78%(12/94);尿NAG及尿β2-MG异常的联合阳性率为28.72%(27/94),以上阳性率间两两比较:尿NAG与尿β2-MG阳性率无差异(X2=0.39P>0.05),而两者联合阳性率高于尿NAG(X2=4.41P<0.05),也高于尿β2-MG(X2=7.28P<0.05)。结论尿NAG及尿β2-MG均可作为早期诊断AP肾损害的尿标志性蛋白;应用两者联合指标,可提高其早期诊断阳性率。     

尿微量蛋白对过敏性紫癜患儿肾损害的早期诊断价值

目的 探讨过敏性紫癜(HSP)患儿肾损害早期诊断的实验室指标，为HSP患儿肾损害的早期诊断、早期干预提供依据。方法 采用放射免疫方法测定32例多次尿常规检查均正常的HSP患儿尿4种微量蛋白[β2-微球蛋白(β2-MG)，α1-微球蛋白(α1-MG)，清蛋白(Alb)，免疫球蛋白G(IgG)]。结果 HSP患儿4种蛋白中1项以上异常28例(87．50％)，β2-MG和(或)。α1-MG升高21例(65．63％)，Alb(和)或IgG升高者18例(56．21％)。结论 尿4种微量蛋白能敏感地反映早期肾损害情况，可作为早期肾损害的敏感指标。     

单纯性肥胖男童血清瘦素、性激素水平及其对性发育的影响

目的 探讨单纯性肥胖男性儿童血清瘦素、性激素水平及其对性发育的影响.方法 从8～14岁1 208例小学生中筛选出体质量指数(BMI)≥25 kg/m2的42例男童作为肥胖组,选择BMI 14.1～23.0 kg/m2的健康男童32例作为健康对照组,对所入选儿童用ELISA法测定其血清瘦素,放射免疫分析法测定其血清雌二醇(E2)、睾酮(T)水平,用游标卡尺测量其阴茎长度及睾丸体积.采用SPSS 10.0软件行组间t检验比较二组差异,直线相关分析单纯性肥胖儿童血清瘦素与E2、T的相关性.结果 肥胖组男童血清瘦素为(16.82±11.46)μg/L,较健康对照组(5.43±3.56)μ/L显著增高,二组比较有显著性差异(t=5.419 P<0.01).肥胖组男童血清E2水平为(20.51±16.42)ng/L,较健康对照组[(8.84±4.53)ng/L]显著增高,二组比较有显著性差异(t=3.903 P<0.01).肥胖组男童T水平为(1.64±0.85)μg,/L,低于健康男童[(2.07±0.98)μ/L],二组比较有显著性差异(t=2.018 P<0.05).肥胖组男童阴茎长度为(4.51±1.36)cm,低于健康男童[(5.64±0.99)cm],二组比较有显著性差异(t=3.965 P<0.01).肥胖组男童睾丸体积[(4.21±2.32)cm3]低于健康男童[(7.08±3.76)cm3],二组比较有显著性差异(t=4.043 P<0.01).瘦素与BMI、E2呈正相关(r=0.757,0.266 Pa<0.05),与T呈负相关(r=-0.368 P<0.01).结论 高水平瘦素可能是单纯性肥胖重要的生物学标志之一.单纯性肥胖男童存在性激素代谢紊乱、性发育落后,瘦素可能影响男性肥胖儿童的性发育.     

单纯性肥胖儿童胰岛素抵抗与肿瘤坏死因子-α的关系

目的探讨单纯性肥胖儿童胰岛素抵抗与肿瘤坏死因子-α(TNF-α)的关系。方法单纯性肥胖患儿50例作为观察组(男23例,女27例);选取同期健康儿童30例为对照组(男14例,女16例)。采用发光免疫法、放射免疫法、快速测血糖法分别对两组儿童的血糖、血胰岛素、血脂和TNF-α进行检测。并作对比分析。结果观察组稳态模型胰岛素抵抗指数(HOMA-IR)、TNF-α及舒张血压(DBP),与对照组比较均明显升高(t=3.939,4.938,3.278 P均<0.01);收缩血压(SBP)、空腹三酰甘油(TC)与对照组比较均明显升高(t=2.536,2.573 P均<0.05);胰岛素敏感指数(HOMA-ISI)观察组明显低于对照组(t=-4.75 P<0.01)。观察组TNF-α与体质量指数(BMI)、TC、HOMA-IR均呈显著正相关(r=0.284,0.328,0.361 P均<0.05)。TNF-α与HOMA-ISI呈显著负相关(r=-0.36 P<0.01)。BMI与HOMA-IR呈显著正相关性(r=0.294 P<0.05)。结论单纯性肥胖儿童存在胰岛素抵抗,血清TNF-α与肥胖儿童的BMI、TC、BP、胰岛素抵抗密切相关,TNF-α可能参与胰岛素抵抗病理生理机制。     

尿α1-微球蛋白与肺炎支原体感染致早期肾损害的相关性

目的分析尿α1-微球蛋白与肺炎支原体(MP)感染致早期肾损害的相关性。方法检测24例血冷凝集试验阳性肺炎患儿的尿常规及尿微量蛋白,包括尿微量清蛋白(Alb)、尿α1-微球蛋白(α1-MG)、尿转铁蛋白(TF)和尿IgG。结果24例中仅1例尿蛋白呈阳性。尿α1-MG在血冷凝集试验<1:256与≥1:256两组及病程≤2周与>2周两组差异均具有显著性(P均<0.05),而其他3项尿微量蛋白组间比较均无显著性差异。结论MP感染致早期肾损害多表现为肾小管重吸收障碍,可能与免疫有关。尿α1-MG可用于MP感染致肾损害的早期诊断。     

尿微量系列蛋白测定对肥胖相关肾病早期诊断的意义

目的探讨尿微量系列蛋白检测对早期发现单纯性肥胖患儿肾损害的临床意义。方法用ELISA方法对40例单纯性肥胖患儿尿微量系列蛋白进行检测,并对50例健康儿童测尿微量系列蛋白作为对照。同时测血脂及作肾功能检查。结果单纯性肥胖患儿40例尿微量系列蛋白检测发现8例异常,5例清蛋白(Alb)增高,其中1例转铁蛋白(TRF)、尿视黄醇结合蛋白(RBP)同时增高,3例TRF增高。对其中1例行肾组织活检,结果提示异常。正常儿童50例尿微量系列蛋白检测均正常。结论尿微量系列蛋白检测可作为早期诊断儿童相关肾病的敏感指标。     

缺氧缺血性脑病新生儿尿大分子碱性磷酸酶的研究

目的研究缺氧缺血性脑病（HIE）新生儿尿大分子碱性磷酸酶（HMAP）水平的变化及其早期诊断肾功能损害的价值。方法HIE组包括轻度脑病52例和中重度脑病40例。利用抗HMAP的单克隆抗体，用免疫催化方法检测92例HIE患儿治疗前后及30例正常新生儿（对照组）尿HMAP水平，并测定两组尿＆．微球蛋白（β2-MG）水平。结果HIE组治疗前尿HMAP、β2-MG水平高于治疗后（t=28．15、t=16．12，P〈0．01）和对照组（t=11．89、t=12．34，P〈0．01），且中重度HIE组尿HMAP高于轻度组（t：2．35，P〈0．05）；HIE组尿HMAP的异常发生率（85．9％）显著高于β2-MG（65．2％）（Y2=10．62，P〈0．01）；HIE组尿HMAP与β2-MG呈直线正相关（r=0．56，P〈0．01）。结论HIE新生儿大多存在肾功能损害，尿HMAP比＆．MG更灵敏地反映HIE时肾损害的程度，可早期发现HIE新生儿肾损害。     

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.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

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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     

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.     

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.     

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.     

alpha -SMOOTH MUSCLE ACTIN DISTRIBUTION IN THE PULMONARY VASCULATURE COMPARING HYPOPLASTIC AND NORMAL FETAL LUNGS

We investigated the intra-acinar pulmonary vascular muscularization in the developing human fetal lung between the 17th and 24th gestational weeks, that is, during the canalicular phase of lung development. Fifteen hypoplastic and 25 normal developed lungs were included in this study using monoclonal alpha -smooth muscle (sm) actin antibodies for smooth muscle detection. Computer-aided image analysis was performed for morphometrical measurements and statistical evaluation. Alphasm-actin-immunoreactive intra-acinar vessels down to a luminal diameter of less than 10 mu m were detected in hypoplastic as well as in normally developed lungs. Crucial differences presented as follows: significantly higher density of intra-acinar vessels, especially due to alpha -sm-actin-negative vessels less than 30 mu m in luminal diameter, in the control group; significantly higher alpha -sm-actin immunoreactivity per section unit as well as per vessel in the hypoplastic lung group. As suggested by others, alpha-sm-actin-positive cells of the intra-acinar vessel wall in the developing human lung were demonstrated to be smooth muscle cells, their immediate precursors, and pericytes. We conclude that the increased alpha -sm-actin immunoreactivity represents muscularization of the vessel wall in functional terms and may be regarded as one structural cause among others for the establishment of persistent fetal circulation in hypoplastic lungs.