再论儿童社区获得性肺炎

中华医学会儿科学分会呼吸学组和《中华儿科杂志》编辑委员会曾于2006年10月制定了我国首部《儿童社区获得性肺炎管理指南(试行)》[1-2],受到广大儿科呼吸专业工作者的关注和肯定,并起到了指导临床实践的作用,成为《中华儿科杂志》近年被引频次排序名列前茅的文章之一.此后至今已近七载,社区获得性肺炎(community acquired pneumonia,CAP)的诊疗和预防中又涌现出众多新问题、新难点、新认识和新进展,我国儿科工作者也在临床实践中不断加深对CAP的认识.国际上,2011年美国儿科感染病学会联合美国感染病学会推出美国国家层面首部《3个月以上婴儿和儿童CAP管理的临床实践指南》[3].英国胸腔学会在同年也更新了《儿童CAP管理指南》[4],日本同样更新了《儿童呼吸道感染聚焦肺炎的管理指南》[5].从儿科实践出发,结合各国自身特点,循证推荐诊治意见去指导临床,以期降低肺炎的患病率和病死率,这是不同国家指南的共同特点和目标.我国也很有必要与时俱进地更新2006年的儿童CAP管理指南.     

儿童常见肾脏疾病诊治循证指南(二):紫癜性肾炎的诊治循证指南(试行)

一、前言 紫癜性肾炎是儿科常见的继发性肾小球疾病之一,由于诊断标准不统一、观察随访时间差异,因而过敏性紫癜患者中发生肾损害的报告率差别较大,文献报道为20%～100%[1-3].Brogan和Dillon[4]依据临床表现诊断,紫癜性肾炎发牛率为40%～50%.2000年11月中华医学会儿科学分会肾脏病学组在珠海制定了紫癜性肾炎的诊断和治疗方案(草案)[5],2008年中华医学会儿科学分会肾脏病学组在此基础上,按照循证医学的原则制定本指南;适用于有一定儿童肾脏疾病诊治基础或经验的儿科医师.在针对具体患儿时,应充分了解本病的最佳临床证据和现有医疗资源,采纳适合的诊疗方案.     

杜氏肌营养不良呼吸功能评价与管理的研究进展北大核心CSCD

杜氏肌营养不良（Duchenne muscular dystrophy,DMD）是儿童中最常见的肌营养不良[1].临床表现为5岁前起病,对称性肢体无力,伴肌萎缩和腓肠肌肥大,病情进行性发展,20岁左右死于心肺功能衰竭.DMD尚无治愈方法,因此进行规范症状管理十分重要[2].由于呼吸并发症普遍存在,严重影响患者生活质量,同时也是DMD的首要死因[2],2004年美国胸科协会（ATS）制定了DMD呼吸管理指南,规范了呼吸功能监测和干预方法[3].2010年美国疾病预防控制中心建立了DMD多学科协作治疗的管理体系.其中,呼吸并发症管理是预防呼吸衰竭、提高生活质量、延长寿命的重要措施[4-5].国内对呼吸功能评价与管理的研究不多,现将对DMD呼吸功能评价与管理的研究进展进行综述.     

儿童感染性心内膜炎诊断标准建议

感染性心内膜炎(infective endocarditis,IE)是儿科严重的感染性疾病之一.近年IE的发病率有增高的趋势,IE的临床特点也在发生变化,使得IE的诊断及处理面临新的挑战.2001年中华医学会儿科学分会心血管学组、<中华儿科杂志>编辑委员会提出小儿IE的诊断标准(试行)[1].经过临床实践证明,上述试行标准对IE诊断的敏感性明显高于以前的诊断标准[2].近年来国外已有多项新的IE诊断、治疗及预防指南发表[3-7],但内容不尽相同.小儿IE的临床表现及病程,因病原微生物、患者年龄、有无基础心脏病及并发症等而异,临床处理只有多学科(小儿心脏内、外科、感染病科、微生物学及临床药学等)专业人员共同参与,才能进一步提高诊治效果.为了有助于IE的临床诊断及处理,根据国内临床经验,并参考国外IE诊断治疗指南,达成专家共识,制定了"小儿感染性心内膜炎诊断标准建议".     

临床实践指南更新的若干关键问题

正1临床指南更新的必要性与现状制订严谨、透明和报告充分的临床实践指南(简称临床指南)能够直接为临床医务工作者提供决策依据,提高医务人员的医疗水平、规范医疗行为、提高服务质量、改善患者结局和节约医疗费用~([1])。2011年美国医学研究所更新了临床指南的定义:通过系统地评价和清晰地总结科学证据,并列举出不同干预方案的利与弊,为医生和患者提供最优方案~([2])。此定义强调临床指南在形成推荐意见时须     

晕厥的临床诊断思路

由于晕厥是一种常见病症,而且部分患者具有高度的猝死危险性,因此对于制定适用于晕厥患者诊断程序是众多心脏病专家关注的问题。美国心脏病协会、欧洲心脏病协会两大心脏病专业机构都分别制定了晕厥患者诊断及处理指南[1,2],但是这些指南都没有经过验证其有效性,且也没有一个适用于所有临床遇到的情况。大多数指南都缺乏详细的信息,以期用来指导临床诊断,因此这些指南仅为诊断、处理晕厥提供了基本框架,在临床处理具体患者时应具体分析。其中2001年欧洲心脏病协会颁布的晕厥患者处理(诊断和治疗)指南是目前最新的评价晕厥患者的权威建议[2]…     

基因重组人生长激素儿科临床规范应用的建议

1985年,基因重组人生长激素(recombinant human growth hormone,rhGH)问世,为广大矮身材患儿的治疗带来希望.随后,rhGH在临床得到迅速应用,其治疗的有效性得到广泛验证,关于rhGH的治疗范围、治疗方案、疗效以及安全性的研究日益深入.为规范rhGH的应用及矮身材儿童的诊治,中华医学会儿科学分会内分泌遗传代谢学组于1998年提出《对基因重组人生长激素在临床应用的建议》[1],2008年制定了《矮身材儿童诊治指南》[2].但目前临床上仍存在随意扩大rhGH应用范围、疾病诊断不规范、过度治疗等问题,从而给rhGH治疗带来诸多隐患.     

《中国新生儿病房分级建设与管理指南(建议案)》解读

《中国新生儿病房分级建设与管理指南(建议案)》(简称本指南)由中国医师协会新生儿专业委员会专项工作小组起草,经广泛征求同行意见,已提交管理机关,现处在文件发布之前的程序之中.鉴于很长一段时间以来,我国新生儿病房建设需求巨大,新生儿医学发展势头劲猛,对于本指南的期盼甚切;故经征得有关部门同意,现先用专业委员会的名义以建议案的形式在学术刊物上发布,在适当范围进行必要的介绍,以期能够满足实际工作参照应用的需要,又能起到指南面世前推广作用. 1 目的和意义 新生儿生命脆弱,发病率、病死率高,要求特殊的医疗护理技术服务;而且,新生儿生命健康对降低儿童病死率、提高人口期望寿命和人口素质贡献显著;所以,新生儿病房的规范化建设和管理特别重要.对此,发达国家成功实行的新生儿病房分级制度值得我们借鉴[1-2].根据区域卫生规划的需求,按照一定的标准对新生儿病房分级建设和管理,可以促进其技术条件和能力的科学化、系统化和标准化;保证新生儿医疗护理服务质量和安全;同时可以使其相互间综合业务实力具有科学可比性;有利于构建规范的区域性新生儿重症转运系统,有利于公众知情,有利于多中心临床研究,有利于卫生行政部门科学决策和监督.因此,本指南的发布必将有力促进我国新生儿医疗服务整体水平的提高,并切实保护患者和医务人员的合法权益[3].     

如何把握中枢性性早熟诊断和治疗中的核心问题

<中枢性(真性)性早熟诊治指南>(以下简称指南)发表迄今已近一年半 [1],它对规范中枢性(真性)性早熟的诊治起了一定作用.现分析和解释<指南>中主要问题的制定依据,希望对临床医生诊治工作有所帮助.     

儿科临床研究安全采血限量及相关问题

正儿科临床研究方案设计应严格遵循风险、不适和痛苦最小化原则。血样采集数量、次数以及可能的痛苦和伤害是儿科研究者、家长/监护人、儿童以及伦理委员会最为关心的问题之一。目前关于儿童采血限量的直接证据很少,国内尚未见相关指南报道。作者经查询世界卫生组织(WHO)简报、美国著名儿童医院的伦理审查指南以及美国北岸医疗集团(North ShoreLIJ Health System)人体受试者保护指南等资料[1-10],     

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.     

�¶�֢��ϵ�ϰ���ע��ȱ�ݶද�ϰ������ڵ�ת��

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.