停跳液中加入吗啡对小儿体外循环心肌保护作用的临床研究

目的研究吗啡加入体外循环心脏停搏液中进行心肌灌注后对小儿心肌的保护作用。方法将30例先心病患儿随机分成3组,即吗啡A组、吗啡B组和对照组（C组）。A组于停跳液中加入浓度为1μmol/L的吗啡,B组于停跳液中加入浓度为2μmol/L的吗啡,C组停跳液中未加吗啡。比较各组心脏转流后自动复跳情况,术后拔管时间,ICU停留时间,术后平均住院天数,磷酸肌酸激酶同工酶（CKMb）、心肌肌钙蛋白（cTnl）值的改变。结果吗啡A、B两组心脏自动复跳率高于对照组,吗啡A、B两组在停转流后6h、12h、24h血清CK-MB和cTnl水平较对照组明显降低（P〈0.05）,差异有统计学意义。吗啡B组停转流后12h、24h cTnl水平及停转流后24hCK-Mb水平较吗啡A组低（P〈0.05）,差异有统计学意义。结论停跳液中加入一定浓度吗啡对小儿心肌有保护作用,且这种保护作用与停跳液中吗啡浓度有关。     

自体冷血停搏液对紫绀型先心病婴儿心脏的保护作用

目的：探讨自体冷血停搏液对紫绀型先心病婴儿心脏的保护作用。 方法：96例行体外循环术的紫绀型先心病婴儿随机分为：HTK液组（HTK组,32例）、非自体冷血停搏液组（非自体组,32例）和自体冷血停搏液组（自体组,32例）,阻断前和主动脉开放30 min取右心耳组织,检测ATP的含量及能量储备（EC）;术前和术后即刻取静脉血,测肌酸激酶同工酶（CK-MB）和心肌肌钙蛋白I（cTnI）的浓度;记录术中复跳时间、复跳率、术后 2 h心脏指数（CI）、正性肌力药物依赖情况和左室射血分数、术后24 h心律失常发生率、术后并发症及病死率等。结果：主动脉开放30 min后,3组ATP 和EC含量均显著降低（P<0.05）,其中自体组较HTK组和非自体组高,差异有统计学意义（P<0.05）。3组术后即刻CK MB和cTnI血清浓度均显著增加（P<0.05）,其中自体组较HTK组和非自体组低,差异具有统计学意义（P<0.05）。自体组在复跳时间、术后2 h正性肌力药物依赖及左心射血分数上优于HTK组和非自体组,差异具有统计学意义（P<0.05）。结论：在紫绀型先心病婴儿体外循环下,自体冷血停搏液比非自体冷血停搏液与HTK液能更好地保存心肌细胞能量,减轻心肌损伤,对心脏具有较好的保护作用。     

儿童体外循环围术期内源性一氧化氮浓度的变化

目的 探讨儿童体外循环期间内源性一氧化氮(NO)浓度的变化及意义。方法 将先心病患儿分为非紫绀型与紫绀型组，非紫绀型再分为肺高压与非肺高压组，于转流前、主动脉阻断后、转流结束、术后24h、术后72h和术后7d测定NO浓度。结果 主动脉阻断后略有降低，体外循环结束时显著增高，术后第1d仍处于较高水平，术后第3d恢复正常；在体外循环期间紫绀型与非紫绀型先心病血浆NO浓度的比较有显著意义；非肺高压组NO浓度升高显著，肺高压组则升高较少，两组术后24h及72h的比较有显著意义。结论 本实验显示术后24h、术后72hNO浓度升高显著，可能与体外循环所致的炎性反应有关；肺高压组则升高较少，易发生肺高压危象，这也是术后NO治疗肺高压危象的基础。     

氧疗对紫绀型先天性心脏病患儿血液流变学的影响

采用间歇低流量吸氧的方法，观察16例紫绀型先天性心脏病（CCHD）患儿减疗后血粘度、红细胞变形能力和血小板聚集功能等血液流变学参数的变化。结果显示，氧疗后患儿红细胞压积（HCT）、全血高切粘度、低切粘度、红细胞聚集指数、刚性指数均显著降低（P＜0.01），红细胞变形能力增强（P＜0.01），血小板聚集功能提高（P＜0.01）。说明氧疗是改善CCHD患儿血液流变学特性，提高组织或供的有效方法。     

化脓性脑膜炎时地塞米松治疗与脑源性神经营养因子关系的实验研究

为探讨化脓性脑膜炎时地塞米松（DEX）治疗与脑源性神经营养因子（BDNF）的关系，以观察其神经保护作用。用大鼠建立化脓性脑膜炎模型，分别用抗生素及抗生素加DEX治疗，用免疫组化法检测脑组织BDNF的表达。结果：感染后24h在脑组织和渗出到软脑膜、蛛网膜下腔、脑室及脑实质炎性病灶内的炎性细胞高水平表达BDNF蛋白。抗生素治疗后BDNF蛋白表达降到很低水平，与感染后24h组比较差异有显著性（P＜0．01），DEX组BDNF蛋白在大脑组织表达明显增多，与抗生素组比较差异有显著性（P＜0．01），但仍低于感染后24h组（P＜0．05）。提示DEX可能通过上调BDNF蛋白的表达，在化脓性脑膜炎时产生神经保护作用，而这一作用是有限的。     

内源性一氧化碳对培养的肺动脉平滑肌细胞增殖与凋亡的调节

目的 探讨内源性一氧化碳（CO）对低氧性肺血管平滑肌细胞（PASMC）增殖与凋亡的影响及机制。方法 体外培养Wistar大鼠PASMC,进行低氧并予以血红素氧合酶抑制剂卟啉锌－9（zinc protoporphyrin IX,ZnPP-9)处理,分为常氧12h组（N1组）、常氧24h组（N2组）、低氧12h组（H1组）、低氧24h组（H2组）、低氧12h ZnPP组（H1＋ZnPP组）、低氧24h ZnPP组（H2＋ZnPP组）,采用流式细胞术、免疫细胞化学方法检测PASMC增殖与凋亡情况。结果 1．碳氧血红蛋白（HbCO)检测：采用双波长法测定细胞培养液中HbCO的吸光度,用以代替培养液中CO的相对含量。低氧12h培养液中HbCO较常氧时显著增高（P＜0．01）,至24h恢复至常氧水平（P＞0．05）;而低氧同时进行干预,H1＋ZnPP及H2＋ZnPP两组HbCO水平均下降,且明显低于单纯低氧组（P＜0．01）,两组与常氧组相比亦有差异（P＜0．001）。2．流式细胞仪检测PASMC增殖指数（PI）与凋亡指数（AI）：H1组PI较N1组下降（P＜0．01）,H2组则较N2组增高（P＜0．001）;H1＋ZnPP组较H1组PI显著增高（P＜0．001）,H2＋ZnPP组与H2组比则无明显变化（P＞0．05）;H1组AI低于H2组（P＜0．01）,H1＋ZnPP组AI高于H1组（P＜0．001）,H2＋ZnPP组则低于H2组（P＜0．001）。3．PCNA免疫细胞化学染色,H1组及H2组PASMC细胞核阳性反应颗粒分别按N1及N2组明显增加（P分别＜0．01及P＜0．001）,H1＋ZnPP组及H2＋ZnPP组分别较H1及H2组增强（P＜0．01）。4．Caspase 3免疫细胞化学：H2组与H1组比胞浆着色增强（P＜0．05）,ZnPP处理12h及24h分别较H1及H2减弱（P＜0．05）。5．NF－κK免疫细胞化学：H1及H2组核染色分别较N1及N2组增强（P＜0．001）,而H1＋ZnPP组及H2＋ZnPP组则又分别较H1、H2组阳性核表达增多（P＜0．01）。结论 1．低氧12h内源性CO对PASMC增殖抑制作用显著,对凋亡可能无直接影响;低氧24h CO促进PASMC凋亡作用明显,而对增殖的影响不显著。2．NF－κB可能参与了内源性CO抑制PASMC增殖的调控机制。     

高压氧对小儿难治性硬膜下积液术后脑复张疗效分析北大核心CSCD

目的探讨高压氧治疗对小儿难治性硬膜下积液行钻孔引流手术后脑复张的影响。方法我科收治的6O例因化脓性脑膜炎并发硬膜下积液患儿根据硬膜下积液厚度采取分层区组随机分为2组。治疗组3（）例在术后常规药物治疗的基础上行高压氧治疗,对照组3O例术后常规药物治疗。根据复查头颅CT硬膜下残腔最大层面的厚度（T）,将脑复张的程度分为4级（I级：T〈0．5cm,Ⅱ级：0．5cm≤T〈1cm,Ⅲ级：1cm≤T〈1．5cm,Ⅳ级：T≥1．5cm）。分别比较两组术后1、3、6个月时患儿脑复张程度。结果术后1个月,治疗组复查头颅CT统计硬膜下残腔厚度为（O．6±0．2）cm,脑复张程度l级（8例）,Ⅱ级（22例）;对照组为（0．7±0．2）cm,脑复张程度I级（5例）,Ⅱ级（25例）;两组比较,差异无统计学意义（P〉0．05）。术后3个月时,治疗组复查头颅CT硬膜下残腔为（（）．4±0．1）cm,恼复张程度I级（20例）,Ⅱ级（10例）;对照组为（0．6±0．2）cm,脑复张程度I级（9例）,Ⅱ级（21例）;两组比较,差异有统计学意义（P〈0．05）。术后6个月时,治疗组复查头颅CT硬膜下残腔为（0．2±0．1）cm,脑复张程度I级（30例）,Ⅱ级无;对照组为（0．5±0．1）cm,脑复张程度I级（18例）,Ⅱ级（12例）;两组比较,差异有统计学意义（P〈0．05）。结论高压氧能有效促进难治性硬膜下积液术后脑复张,可作为难治性硬膜下积液术后常规辅助治疗措施。     

婴幼儿手术应激后瘦素水平变化的临床研究

目的探讨瘦素水平在婴幼儿大手术应激时的变化及意义。方法选择择期手术患儿17例,分先心组(10例)和非先心组(7例),分别于术前、术中、术后2h、术后24h、术后48h采集外周静脉血,离心后,测定血清中瘦素和皮质醇的水平。结果先心组和非先心组瘦素水平在术中均明显下降(术前分别为3．48±0．23、4．50±0．24．术中为2．12+0．14、3．09±0．21,P＜0．05)。术后24h又恢复至基础水平(3．78±0．19、4．89±0．43．P＞0．05)。皮质醇水平先心组术中显著升高(术前为25．39±1．74,术中为50．87±3．59,P＜0．05),非先心组术中升高(术前为28．61±1．69,术中为31．90±2．02,P＞0．05)；术后24h先心组恢复至基础水平(30．95±1．77,P＞0．05),非先心组水平仍高(37．48±1．92,P＜0．05)。结论在手术应激的早期,瘦素的下降和皮质醇的上升是平行的,瘦素在手术应激早期发挥作用。     

体外循环手术患儿血清S-100b蛋白及脑电图变化的研究

目的观察常温（35℃）及浅低温（32℃～34℃）体外循环（CPB）下,心内直视手术患儿围手术期血浆S-100b蛋白的含量以及手术前后脑电图（EEG）的变化。方法体外循环下行室间隔缺损修补术的患儿40例,分为常温（35℃）组（n1=20）和浅低温（32℃～34℃）组（n2=20）。所有患儿均在麻醉诱导前（a点）、CPB开始后15min（b点）、CPB结束后1h（c点）、术后24h（d点）、术后48h（e点）以及术后7d（f点）采取血标本,运用酶联免疫吸附法（ELISA法）测定血浆S-100b蛋白的含量。同时于术前和术后7d对全部患儿进行脑电图检查,对所得结果进行统计学分析。结果CPB结束后以及术后24h,两组血浆S-100b蛋白较术前明显升高（P〈0．01）,且持续到术后48h仍高于术前水平（P〈0．05）,至术后第7天基本降至术前水平;比较两组各对应时间点血浆S-100b蛋白浓度,差异无统计学意义（P〉0．05）。计算两组EEG异常率,术后EEG结果较术前明显变差（P〈0．01）,但两组术后第7天EEG异常率比较,差异无统计学意义（P〉0．05）。术后第7天时,EEG的异常变化滞后于血浆S-100b蛋白的改变。结论体外循环心内直视手术患儿术后脑功能有不同程度损害,术后血浆S-100b蛋白浓度和EEG检查对脑损伤的评价有重要意义。本研究未发现常温（35℃）体外循环明显增加心内直视术后的神经损伤。     

右腋下小切口在小儿心内直视手术中的应用研究

目的比较小儿右腋下小切口与胸骨正中切13行心内直视手术的临床资料,探讨右腋下小切口先心病手术的临床效果。方法我们自2012年12月至2013年12月实施右腋下小切口小儿心内直视手术102例,与同期胸骨正中切口手术主要诊断类似患儿102例比较,进行回顾性分析。结果两组主动脉阻断时间、体外循环时间及手术时间比较,差异无统计学意义（P〉0．05）。呼吸机辅助通气时间、术后24h引流量以及术后住院时间比较,右腋下小切口优于胸骨正中切口,差异有统计学意义（P〈0．01）。结论采用右腋下小切口能安全有效地完成先心病心内直视手术,治疗效果好,术后恢复快,切口美观。     

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.