儿童感染性心内膜炎35例分析

目的了解儿童感染性心内膜炎(IE)的临床特点、病原谱特征、耐药状况及预后。方法回顾性分析2007年6月1日至2014年12月31日确诊为IE患儿的临床及病原学资料。结果研究年限内共确诊IE 35例,男15例、女20例,平均年龄为(6.56±0.81)岁。原有先天性心脏病23例(65.7%)。赘生物形成24例(68.6%)。57.4%的患儿有长期发热。血培养阳性19例,占54.3%;其中83.3%为革兰阳性球菌,包括凝固酶阴性葡萄球菌、草绿色链球菌和肠球菌。所有阳性球菌均对万古霉素敏感。结论先天性心脏病是IE的易感因素,感染细菌以革兰阳性球菌为主,对万古霉素敏感。     

儿童感染性心内膜炎33例临床特点及疗效分析

目的 探讨儿童感染性心内膜炎的临床特点及影响疗效的因素。方法 回顾性研究2013年11月至2015年11月上海儿童医学中心心内科诊断并治疗的33例感染性心内膜炎患儿的临床资料,分析其病原学特点、心脏基础疾病、赘生物分布特点、治疗方案及预后情况。结果 感染性心内膜炎病原菌的检出率为75.76%。具有先天性心脏基础疾病的比例为93.94%。赘生物主要位于反流的瓣膜,占63.64%,其次为植入的人工材料、缺损处分流及血流冲击处等。手术结合足疗程的抗感染治疗效果较好。结论 感染性心内膜炎的血培养阳性率高,且多发生于具有心脏基础疾病的患儿。早期彻底的手术治疗联合全程抗感染治疗是治疗感染性心内膜炎的关键。     

单中心十年收治感染性心内膜炎患儿临床分析

目的 探讨单中心10年来收治的感染性心内膜炎(infective endocarditis,IE)患儿的临床特点、病原菌分布等情况,为临床诊治提供参考.方法 回顾性分析中国医科大学附属盛京医院2006年10月至2016年10月儿科病房住院治疗的30例确诊 IE 患儿的临床及病原学资料.结果30例IE 患儿中,男18例,女12例,年龄2个月~13岁.IE就诊原因分别为发热21例(70.0%),呼吸系统症状4例(13.3%),神经系统症状4例(13.3%),水肿2例(6.7%),新发心脏杂音1例,心前区不适1例,血三系减少1例.基础疾病所占比例最高的是先天性心脏病17例(56.7%),其中6例为先天性心脏病术后患儿.30例患儿心脏彩超均发现赘生物,血细菌培养阳性15例(50.0%),病原菌分布:金黄色葡萄球菌6例(40.0%),肺炎链球菌4例(26.7%),粪肠球菌2例(13.3%),血链球菌1例,咽颊炎链球菌1例,表皮葡萄球菌1例.入院时C-反应蛋白升高25例(83.3%),其中8例C-反应蛋白>100 mg/L.结论 IE早期临床症状多不典型,发热是IE的常见就诊原因,先天性心脏结构异常易并发IE,革兰阳性球菌是主要致病原,单纯抗感染治疗效果不佳者可联合手术治疗.     

儿童肺炎链球菌感染性心内膜炎3例

对多中心收治的3例肺炎链球菌感染性心内膜炎患儿的临床资料进行回顾性分析。例1为2岁11个月男童,以间断发热1个月余,皮肤发花20 d,发现心脏赘生物1 d入院。超声心动图提示三尖瓣中等团块样回声,血培养结果示肺炎链球菌;例2为3岁10个月女童,以间断发热8 d,腹痛1 d入院。超声心动图提示二尖瓣强回声团,血培养结果示肺炎链球菌。例3为3岁4个月女童,以咳嗽20 d,发热9 d入院。住院期间出现急性左心衰竭。超声心动图提示二尖瓣团块状高回声,血培养结果示肺炎链球菌,菌株血清型23F。3例患儿均有发热表现,2例合并呼吸系统症状。例2出现脑梗死,例3出现急性左心衰竭。3例患儿均通过超声心动图确诊,使用万古霉素治疗后有效,提示本病临床表现多样,万古霉素是重要的抗感染治疗药物之一。     

小儿感染性心内膜炎死亡危险因素及病原学特点分析

目的 分析小儿感染性心内膜炎死亡的危险因素及病原学特点.方法 运用回顾性病例对照研究策略对2000年1月至2007年6月间上海儿童医学中心收治的46例感染性心内膜炎患儿的临床资料进行总结,研究因素包括性别、原发病、感染部位、培养结果 、并发症、治疗措施等16个变量,建立Logistic回归模型.结果 感染性心内膜炎患儿(46例)占同期住院患儿总数的0.78‰,其中40例有心脏疾患,左侧心内膜炎的发病率比右侧高.24例血培养阳性,1例赘生物菌培养阳性,总的细菌培养阳性率为54%.在25例阳性结果 中,革兰阳性菌15例,其中常见病原菌为α-溶血性链球菌;革兰阴性菌7例;真菌3例.46例患儿治愈40例,死亡6例.Logistic回归模型示与死亡有关的因素包括血培养阴性(OR=25.127,95%CI为1.110～363.236)、血液疾病(OR=28.620,95%CI为2.261～354.448)和多器官功能衰竭(OR=19.843,95%CI为0.786～221.754).结论 小儿感染性心内膜炎的病原菌以α-溶血性链球菌最常见,左侧心内膜炎的发病率比右侧高.血培养阴性、血液疾病和多器官功能衰竭是预后不良的危险因素.     

儿童院内及社区获得性肺炎病原菌和药敏试验及分析

目的　了解住院肺炎患儿病原菌分布、药敏情况及临床特点。方法　无菌操作吸取患儿鼻咽深部分泌物或气管插管内合格痰标本 ,接种于 5 %羊血琼脂平皿和巧克力平皿培养 ,用最低抑菌浓度 (MIC)法做药敏试验。结果　院内获得性肺炎 (HAP)组及行气管插管治疗的社区获得性肺炎 (CAP Ⅰ )组病原菌以革兰阴性 (G-)菌为主 ,并以铜绿假单胞菌居首位 (2 5 % ) ;未行气管插管治疗的社区获得性肺炎 (CAP Ⅱ )组革兰阳性 (G+ )球菌相对增多 ,并以肺炎链球菌和表皮葡萄球菌为主 (均占 38 2 % ) ,4 6 2 %的肺炎链球菌分离自 1～ 5岁儿童 ,84 6 %的表皮葡萄球菌分离自 6个月以下婴儿。各种G-菌对环丙沙星较敏感 (总敏感率 77 2 % ) ,而对其他多种抗生素耐药率较高 ;肺炎链球菌对青霉素、氨苄青霉素和万古霉素的敏感率均为 84 6 % ;表皮葡萄球菌对万古霉素 10 0 %敏感 ,对头孢三嗪和阿莫西林 /棒酸的敏感率分别为 76 9%和 6 9 2 % ,对青霉素 10 0 %耐药。HAP组多数预后不佳 ,病死率较高 (8 7% ) ;CAP Ⅰ组 17例重症肺炎中 ,1～ 3个月婴儿 11例 (6 4 7% ) ,其中 2例(18 2 % )死亡。结论　近 5年住院分离的多数病原菌对多种常用抗生素普遍耐药 ;肺炎链球菌感染时仍可考虑首选青霉素治疗 ,但应警惕耐青霉素肺炎链球菌的     

发热、头痛、皮肤紫癜2例

该研究2例学龄期患儿于春季急性起病,表现为发热、头痛、呕吐、意识障碍,以及皮肤紫癜、脑膜刺激征阳性。外周血白细胞及中性粒细胞均增高、血红蛋白及血小板不同程度降低;CRP明显增高;脑脊液白细胞数增高达数百或上千、且以中性粒细胞为主,脑脊液蛋白增高,糖、氯化物正常。其中1例头颅CT示右侧小脑、双侧大脑多发血肿。骨髓细胞学提示感染性骨髓象,血培养、骨髓培养均提示金黄色葡萄球菌(MRSA)。患儿抗感染治疗过程中出现心脏杂音,并且血红蛋白及血小板进行性下降,心脏彩超发现主动脉瓣赘生物形成,确诊为感染性心内膜炎(IE)。根据药敏试验改为万古霉素抗感染治疗,1例6周后痊愈,1例放弃治疗死亡。儿童IE起病隐匿,临床表现多样,建议对于不明原因发热患儿要注意动态观察心脏杂音,及时行心脏彩超排除IE;以化脑为表现的患儿伴有难以用血小板减少解释的皮肤、黏膜出血时,也应警惕IE。     

V3通用引物联合多重PCR检测感染性心内膜炎病原菌

目的 了解2007年至2010年21例在上海儿童医学中心接受手术治疗的急性感染性心内膜炎(infective endocarditis,IE)患儿的病原菌分布.方法 21例IE患儿均行血培养、赘生物培养及赘生物PCR检测(以16SrRNA基因的保守区序列V3为靶基因);对葡萄球菌进行多重PCR扩增检测耐甲氧西林葡萄球菌.结果 21例IE患儿赘生物PCR阳性检出率95.2％,血培养阳性检出率57.1％,赘生物阳性检出率9.5％,3种方法阳性检出率的差别有统计学意义(P ＜0.0001).其中1例心内膜赘生物的PCR结果与血培养结果不一致,赘生物PCR结果为放射菌,血培养结果为溶血巴斯德菌,利用血培养得到的菌落进行PCR检测,与赘生物PCR的结果一致,均为伴放线放线杆菌;其余11例心内膜标本的PCR结果与血培养结果一致.多重PCR技术检测mecA基因能快速、敏感、准确地检测耐甲氧西林葡萄球菌,与femA基因联合检测能有效检测耐甲氧西林金黄色葡萄球菌,及时提示临床使用糖肽类抗生素.21例患儿均痊愈出院,术后随访无感染复发.结论 V3通用引物联合多重PCR法可提高IE患儿赘生物病原菌检出率,且受抗菌药物的影响小,适用于符合手术指征且血培养阴性或鉴别困难的病原学诊断,有利于手术后抗菌药物的选用及提高IE的最终治愈率.     

116例细菌性脑膜炎儿童脑脊液病原菌分布及耐药性分析

目的：了解小儿细菌性脑膜炎病原菌分布特点及其耐药状况,为临床进行及时有效治疗提供依据。方法：回顾分析2008年1月至2012年6月昆明市儿童医院5097例小儿脑脊液中的病原菌分离培养结果及其药敏试验情况。采用K-B纸片法对病原菌进行常用抗生素的敏感性检测并进行分析。结果：5年来共从5097例脑脊液标本中检出病原菌116株,其中革兰阳性菌77株（66.4％）,革兰阴性菌30株（25.9％）,真菌9株（7.8％）,阳性率为2.28%（116/5097）。排名前6 位的病原菌依次为表皮葡萄球菌32株(27.6%),肺炎链球菌15株(12.9%),大肠埃希菌15株(12.9%),溶血葡萄球菌9株(7.8%),新型隐球菌8株(6.9%),金黄色葡萄球菌6株(5.2%)。新生儿及婴儿早期的病原菌主要是凝固酶阴性葡萄球菌,其对青霉素、红霉素、克林霉素敏感率均低于40%;肺炎链球菌对青霉素敏感率仅为13.4%,而对红霉素和克林霉素敏感率达到60.0%;但未见万古霉素耐药的葡萄球菌和肺炎链球菌。革兰阴性杆菌对亚胺培南、美洛培南、头孢哌酮/舒巴坦及头孢吡肟敏感率较高。结论：近5年来小儿细菌性脑膜炎患者病原菌以革兰阳性球菌为主,检出病原菌对常用抗生素耐药性较高;另外,新型隐球菌检出占有一定的比例,应高度注意新型隐球菌性脑膜炎,防止误诊和漏诊。     

二维超声心动图对感染性心内膜炎诊断价值

近年来,采用二维超声心动图(2DE)检查诊断感染性心内膜炎(infective endocarditis,IE)普遍受到重视。它既可显示心脏内结构,又能够检查心内膜赘生物额外回波及IE瓣膜的功能状态。我科自1986年12月至1997年12月对11例小儿感染性心内膜炎的患儿进行了2DE检查,现报告如下。     

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.     

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.     

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.