一起暴发宋内志贺茵食物中毒的流行病学及临床特征分析

目的 探讨某小学暴发的宋内志贺菌食物中毒大便培养阳性患儿的临床特征.方法 志贺菌监测、分离、鉴定依照<全国细菌性痢疾监测方案>,药敏试验采用K-B扩散法.共获得245份大便培养阳性病例完整的病例资料,其中男126例,女119例.结果 发热、呕吐、腹痛、寒颤、里急后重是多数患儿的首发症状,男生的腹泻次数与女生比较差异有统计学意义(P<0.05).血白细胞、中性细胞比例,中性细胞绝对值,低血钾,大便常规白细胞构成比、红细胞构成比、脓细胞阳性率、隐血阳性率,性别差异均无统计学意义(P>0.05).女生大便常规中吞噬细胞阳性率高于男生(P<0.05).结论 宋内志贺菌感染多呈不典型发作,部分临床特征有性别差异.     

儿童福氏志贺菌Ⅰ类整合子的检测及其与耐药性的关系

目的了解儿童福氏志贺菌是否存在Ⅰ类整合子及其发生率以及与耐药性的关系。方法对2004年6-11月从本院细菌性腹泻患儿大便培养标本分离到的福氏志贺菌共51株,应用PCR检测Ⅰ类整合子的5′保守区intⅠ1、基因盒区ant（3″）-Ⅰ和3′保守区qacE△1-sull;用K-B（Kriby-Bauer）法测定志贺菌对7种抗菌药物的敏感性。结果51株福氏志贺菌中46株检出intⅠ1,Ⅰ类整合子阳性率90．2％;24株检出qacE△1-sull,阳性率47．1％;intⅠ1、ant（3″）-Ⅰ和qacE△1-sull区段均阳性的为22株,阳性率43．1％;46株intⅠ1阳性福氏志贺菌ant（3″）-Ⅰ基因均阳性;46株intⅠ1阳性福氏志贺菌中qac△1-sull同时阳性的菌株为22株,占47．8％（22／46）、qacE△1-sull阴性的菌株24株,占52．2％（24／46）。Ⅰ类整合子阳性志贺菌对多种抗生素耐药,尤其是对氨苄西林（χ^2=10．13,P〈0．01）和氯霉素（χ^2=19．97,P〈0．01）的耐药性明显高于Ⅰ类整合子阴性志贺菌。结论从福氏志贺菌检出Ⅰ类整合子,携带率为90．2％;携带Ⅰ类整合子阳性志贺菌与抗生素耐药相关。     

伴抗中性粒细胞胞浆抗体阳性狼疮性肾炎患儿临床病理特征分析

目的 分析伴抗中性粒细胞胞浆抗体（ANCA）阳性狼疮性肾炎（LN）患儿的临床及病理特征。方法 回顾性选择2003年1月至2019年12月于中山大学附属第一医院诊断的LN患儿,依据血清ANCA结果分为ANCA阳性组（n=59）和ANCA阴性组（n=454）,比较两组患儿临床表现、组织病理学、治疗缓解与预后情况。结果 ANCA阳性组白细胞降低并伴红细胞沉降率增快（P < 0.05）。两组患儿血肌酐、尿蛋白、尿红细胞差异无统计学意义（P > 0.05）。308例患儿行肾脏穿刺活检,光镜结果提示ANCA阳性组细胞纤维性新月体比例高于ANCA阴性组（P < 0.05）,免疫复合物沉积比例低于ANCA阴性组（P < 0.05）。两组患儿治疗缓解率、生存情况差异无统计学意义（P > 0.05）。结论 ANCA阳性LN患儿肾脏病理损害更重,与临床表现不完全对应平行,及时行肾脏穿刺活检至关重要。     

儿童支气管哮喘与肺炎支原体感染的关系

目的探讨儿童支气管哮喘与肺炎支原体（MP）感染的关系。方法采用MP被动冷凝集法检测哮喘急性发作期103例及对照组非感染性疾病患儿30例血清肺炎支原体抗体（MP-Ab）。MP-Ab阳性患儿随机分为阿奇霉素治疗组和常规治疗组,比较其疗效。结果哮喘组MP-Ab阳性46例,阳性率44.66%;对照组阳性2例,阳性率6.67%,二组比较差异显著（P〈0.05）;MP-Ab阳性阿奇霉素治疗组30例,有效率86.7%;常规治疗组16例,有效率43.8%,二组比较差异显著（P〈0.05）。结论MP感染与儿童哮喘关系密切,对哮喘发作期患儿要考虑MP感染可能,应常规行MP-Ab检查。大环内酯类抗生素疗效确切。     

特发性血小板减少性紫癜患儿骨髓巨细胞病毒抗原的检测及临床价值

目的探讨骨髓中人巨细胞病毒（HCMV）感染在小儿特发性血小板减少性紫癜（ITP）中的作用及机制。方法采用二步法免疫组化检测81例ITP患儿骨髓中HCMV早期抗原,并行外周血血小板计数及ELISA法检测血HCMV-IgM。结果ITP患儿骨髓HCMV阳性率（61.73%）明显高于血HCMV-IgM阳性率（20.99%）,P〈0.01;慢性ITP骨髓HCMV阳性率（92.31%）高于急性ITP（55.88%）,P〈0.05;慢性ITP血HCMV-IgM、IgG阳性率与急性ITP相比,差异无统计学意义（P〉0.05）。骨髓HCMV阳性组的平均年龄、治疗前血小板数、治疗后血小板上升值与阴性组相比,差异具有统计学意义（P〈0.05）,骨髓巨核细胞数及产板型巨核细胞数与阴性组相比,差异无统计学意义（P〉0.05）;血HCMV-IgM阳性组的平均年龄、治疗前血小板数、治疗后血小板上升值与阴性组相比,差异无统计学意义（P〉0.05）;血HCMV-IgG阳性组的上述三项指标与阴性组相比,差异亦无统计学意义（P〉0.05）。骨髓HCMV阳性细胞数量分级与治疗前血小板计数之间无相关关系（P〉0.05）。结论HCMV感染在ITP中广泛存在,骨髓HCMV抗原的检测是评价ITP患儿HCMV感染的敏感、特异的指标;骨髓HCMV感染的ITP患儿存在病情加重、疗效不佳及慢性化的趋势;在骨髓HCMV致ITP的机制中,可能主要是影响外周免疫系统从而破坏成熟的血小板,而不是对血细胞生成的抑制。     

儿童非糜烂性反流病与反流性食管炎症状及酸反流比较

目的比较非糜烂性反流病（NERD）与反流性食管炎（RE）临床症状及食管酸暴露特点。方法将127例胃食管反流病（GERD）患儿根据内镜检查结果分为NERD组和RE组,分别对两组的临床症状及食管24hpH值监测结果进行比较。结果127例中NERD79例（62.2%）,RE48例（37.8%）,RE组Ⅰ、Ⅱ、Ⅲ级分别为34、10和4例。NERD组和RE组pH监测阳性者分别为51例（64.6%）和30例（62.5%）,两组阳性率差异无统计学意义（χ^2=0.05,P〉0.05）。NERD组呕吐/反食、腹痛及食管外症状的发生率较RE组高,差异有统计学意义（P〈0.05）,而反酸、烧心/胸骨后痛的发生率两组差异无统计学意义（P〉0.05）。RE组总反流次数及pH值〈4的百分比显著高于NERD组（P〈0.05）;pH值监测阳性的RE组各项酸反流指标均大于pH监测阳性的NERD组,差异均有统计学意义（P〈0.05）;Ⅰ级、Ⅱ级和Ⅲ级RE患儿之间的各项pH监测指标差异无统计学意义（P〉0.05）。结论儿童NERD患者的不典型反流症状和食管外症状发生率更高。GERD的发生与胃酸反流密切相关,酸暴露持续时间延长及反流频率增加可能是导致RE的重要因素,酸反流量与RE的严重程度无明确关系。     

儿童急性白血病survivin、cyclin D1基因的表达

目的探讨基因survivin、cyclin D1的表达与儿童急性白血病（AL）的关系。方法采用RT-PCR方法对42例初发或复发AL患儿和13例完全缓解AL（AL-CR）患儿及15例非恶性疾病患儿骨髓单个核细胞进行基因survivin、cyclinD1表达的检测。结果①AL组survivin、cyclinD1mRNA的阳性率分别为78.6%和42.9%,高于AL-CR组和对照组（P〈0.01）;完全缓解组和对照组差异无统计学意义（P〈0.05）;急性淋巴细胞白血病（ALL）组和急性非淋巴细胞白血病（ANLL）组之间表达差异无统计学意义。AL复发组cyclin D1 mRNA阳性率明显高于初发组。②儿童AL初发者survivin与cyclin D1的阳性表达差异无统计学意义。③cyclin D1 mRNA阳性者缓解率低。结论儿童AL中存在survivin、cyclin D1异常表达,两者阳性表达差异无统计学意义;cyclin D1高表达者可降低AL的化疗敏感性,cyclin D1对AL有判断疗效和预后的价值。     

儿科患儿双份血培养实践与效果评价北大核心CSCD

目的评价儿科患者双份血培养对血培养阳性率的影响。方法回顾性分析2013年1月至2015年2月首都医科大学附属北京儿童医院感染内科1 985例住院患儿血培养采集方式、采样前抗生素应用与血培养阳性情况,按照血培养采集方式分3个阶段,血培养单瓶阶段：患儿单送1瓶需氧瓶血培养;血培养双瓶过渡阶段：自相同的皮肤穿刺点抽取2份血液标本同时送检进行需氧瓶培养;双份血培养阶段：自不同的皮肤穿刺点各抽取1份血液标本同时送检进行需氧瓶培养,抽取2份血培养的间隔时间应尽量〈5 min。3个阶段的阳性率比较采用Pearson χ2检验,3个阶段阳性率的变化趋势采用Cochran－Armitage趋势检验。所有检验分析以双侧P〈0.05为差异有统计学意义。结果3个阶段血培养采集之前80%以上患儿都用过抗生素。真阳性率方面,各阶段差异无统计学意义（χ2＝1.343,P〉0.05）,各阶段真阳性率变化趋势差异无统计学意义（P〉0.05）。假阳性菌株常见的是凝固酶阴性葡萄球菌。假阳性率方面,血培养单瓶阶段高于双份血培养阶段,差异有统计学意义（χ2＝6.051,P〈0.05）。结论对于儿童（不包括新生儿）双份血培养可以降低血培养假阳性率,起到鉴别血培养污染的作用。     

儿童急性肾盂肾炎危险因素及耐药分析北大核心CSCD

目的探讨急性肾盂肾炎（APN）患儿的常见致病菌分布特点及耐药现状,并分析发生APN的危险因素。方法回顾性分析2012年3月至2017年3月在天津市儿童医院住院治疗的264例APN患儿中173例尿培养阳性患儿的致病菌分布情况,并对常见致病菌进行药敏试验,以观察其对抗生素的敏感性。与同期住院的200例下尿路感染的患儿分别进行性别、年龄、血、尿指标比较,分析发生APN的危险因素。结果革兰阴性杆菌仍是APN的主要致病菌,占61.3%,其中大肠埃希菌占34.1%;革兰阳性球菌占38.7%,其中屎肠球菌占24.3%。大肠埃希菌对羟氨苄西林耐药率高（89.8%）,而对添加了克拉维酸钾的羟氨苄西林的耐药率（47.5%）明显降低,二者比较差异有统计学意义（P〈0.05）;对头孢哌酮舒巴坦的耐药率均明显低于同类抗生素,差异均有统计学意义（P〈0.01）,对亚胺培南耐药率最低（0.03%）。屎肠球菌对利奈唑胺及万古霉素耐药率低（〈5%）。单因素、多因素分析显示,患儿〈1岁、并泌尿系统基础疾病、血降钙素原（PCT）、尿β2微球蛋白（β2－MG）及中性粒细胞明胶酶相关脂质运载蛋白（NGAL）升高均为APN患儿的危险因素（均P〈0.05）。结论大肠埃希菌是APN的主要致病菌,但肠球菌感染的比例相对较高。这些致病菌耐药率高,应积极行尿培养检查以指导临床用药。对于〈1岁和/或并泌尿系统基础疾病及血PCT、尿β2－MG及NGAL明显升高的泌尿系统感染患儿,应尽早行进一步影像学检查以明确APN诊断。     

抗中性粒细胞抗体在川崎病诊断中的价值

目的：探讨抗中性粒细胞抗体（ANCA)在川崎病诊断中的价值。方法：检测30例典型川崎病患儿和16 例不完全川崎病患儿急性期、恢复期血清ANCA,并随机选取25例健康儿童作为对照组。同时对川崎病患儿急性期行超声心动图(UCG)检查。结果：川崎病患儿急性期血清ANCA阳性率为65%,其中不完全川崎病组和典型川崎病组血清ANCA阳性率分别为69％、63％,均明显高于对照组(P＜0.01）。川崎病患儿恢复期血清ANCA阳性率显著低于急性期（33％ vs 65％,P＜0.05）。川崎病患儿急性期血清ANCA阳性率显著高于UCG的阳性检出率(P＜0.01)。ANCA阳性患儿冠脉病变发生率明显高于ANCA阴性患儿（43％ vs 13％,P＜0.05)。结论：血清ANCA可以作为一项早期诊断川崎病的参考指标和发生冠脉损害的一项实验室预警指标。     

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.