闭塞性细支气管炎31例

目的 明确小儿闭塞性细支气管炎(BO)的临床特点、治疗措施及随访结果.方法 对2008年4月- 2011年6月在本科明确诊断为BO患儿的临床资料进行归纳、总结,对其治疗效果进行随访分析.结果 31例患儿反复咳嗽及喘息均＞6周,查体肺部听诊可闻及哮鸣音和(或)粗湿啰音.入院给予雾化吸入支气管扩张剂无反应.31例患儿均存在肺功能异常,24例患儿为阻塞性通气功能障碍,7例患儿肺功能示混合性通气功能障碍;31例肺CT均存在Mosaic灌注征、双肺均可见斑片状密度增高影,肺不张6例,支气管壁增厚5例,支气管扩张2例.31例X线胸片均有过度通气表现,可见斑片状密度增高影,4例呈毛玻璃样改变.患儿均口服泼尼松、阿奇霉素并配合雾化吸入布地奈德及沙丁胺醇.对其中26例患儿进行2个月～3 a随访,规律治疗的6例患儿临床症状明显改善,无明显喘息发作,活动耐力好转,复查肺部高分辨率CT示肺部病变明显改善;10例临床症状略好转,仍有活动后气促,复查肺部高分辨率CT示肺部病变轻微改善;10例患儿依从性差,未按医嘱规律用药及回访,病情经常反复,于呼吸道感染后出现喘息发作,于本院住院3～6次,临床疗效欠佳,复查肺部高分辨率CT示肺部病变无改善,现仍在随访中.结论 BO大多为肺部感染后引起的反复咳嗽、喘息,肺部听诊可闻及明显的呼气相哮鸣音,吸入支气管扩张剂效果不佳,肺功能大多表现为阻塞性通气功能障碍,CT可见肺部典型的Mosaic灌注征.本病预后不佳.     

机械通气患儿舒适性治疗策略及常见急症事件的认识和处理

对正在进行机械通气治疗的患儿予镇静镇痛等治疗,使患儿能耐受气管插管,使机械通气与患儿自主呼吸同步,消除人机对抗,改善患儿的舒适性,从而改善气体交换,目前已成为儿科重症监护病房一个重要的组成部分。在舒适性的治疗策略中,包括镇静剂、镇痛剂、肌松剂及心理治疗等措施。机械通气可挽救危重患儿的生命,但在此过程中却可能引起各种并发症,除与机械通气的原发疾病相关外,部分与机械通气患儿所采取的治疗措施相关,临床医师及时发现并处理常见的呼吸机使用过程中的急症事件。     

Multicenter Study of the Effectiveness of Implantable Cardioverter Defibrillators in Children and Young Adults With Heart Disease

Implantable cardioverter defibrillators (ICDs) are being used with increasing frequency in children and young adults. Our aim was to examine the appropriateness and frequency of ICD discharges in children and young adults, to compare the effectiveness of ICDs when placed for primary or secondary prevention, and to provide time-dependent analysis of ICD discharges. Data were collected from seven institutions on 210 patients <30 years of age who underwent ICD implantation from October 1992 to January 2007. Median age at implant was 15.4 years with a follow-up average of 3.3 years. Heart disease was categorized as electrical (n = 90, 42%), cardiomyopathic (n = 62, 30%), or congenital heart disease (n = 58, 28%). ICDs are increasingly placed for primary prevention. There are increased appropriate ICD discharges for ICDs placed for secondary prevention (52%) versus primary prevention (14%) at 5 years. There is no difference in the risk of inappropriate discharges between primary and secondary prevention indications. There is an increased risk for inappropriate therapy in the congenital heart disease population. An increasing number of ICDs are being placed for primary prevention in young patients, a marked shift in practice during the last two decades. The benefits of ICDs remain greater in secondary than in primary-prevention patients. In both groups, approximately 25% of patients received inappropriate discharges within 5 years of implant. Patients with congenital heart disease are the most affected by inappropriate discharges.     

噬血细胞性淋巴组织细胞增生症前瞻性研究

目的 探讨儿童噬血细胞性淋巴组织细胞增生症(HLH)的临床特点及预后影响因素.方法 2004年1月-2007年5月本院收治HLH患儿64例.男43例,女21例;中位年龄4岁3个月.除诊断所需检测项目外,化疗前常规行头部CT或MRI检测,行脑脊液生化及常规分类、爱泼斯坦病毒(EBV)、巨细胞病毒、单纯疱疹病毒、柯萨奇病毒、埃可病毒抗体水平检测;并定期监测上述指标以评估疗效,对所有HLH采用统一的免疫化疗方案.结果 1.化疗2周后,1例死亡,4例于化疗后8周放弃治疗未评估疗效,余59例均临床有效,其中21例随后因各种原因放弃治疗,32例进入停药观察期,其中3例停药后1个月内复发.2.患儿 64例中EBV相关HLH(EBV-HLH)患儿47例(73.4%),29例疗效差者中24例(82.7%)为EBV-HLH.3.有60例(93.8%)患儿中枢神经系统受累,且影像学改变先于临床症状的出现.结论 1.该组研究中多数患儿更倾向于继发HLH诊断.2.EBV-HLH是我国儿童HLH的主要类型,也是重要的预后不良因素之一.3.HLH患儿中枢神经系统受累的发病率高,预后差,但通过早期积极的治疗是可逆的.4.对HLH患儿确诊后应常规行头颅MRI检查.     

氨茶碱对毛细支气管炎患儿的疗效

目的 探讨应用氮茶碱治疗毛细支气管炎患儿的临床效果.方法 将2005年2月-2006年5月住院治疗的毛细支气管炎患儿128例随机分为试验组和对照组,并根据病情再分为轻症和重症组.对照组患儿按毛细支气管炎常规方案治疗;试验组患儿除常规方案治疗外,同时口服氨茶碱控释片治疗[2 mg/kg,1次/d].入院当天、住院第3、7天分别测定患儿潮气量(VT)、吸呼比(Ti/Te)、达峰时间比(tPTEF/tE)和达峰容积比(vPTEF/vE),同时检测呼吸道α1-酸性糖蛋白、血白细胞介素-8(IL-8)和呼吸道分泌物的细胞计数及分类.结果 治疗后,试验组患儿肺功能及呼吸道分泌物的细胞计数和分类较对照组患儿明显改善(P<0.05.0.01),呼吸道α1-酸性糖蛋白及血清IL-8较对照组患儿明显降低(P<0.05,0.01);试验组患儿VT、Ti/Te、tPTEF/tE、vPTEF/vE与对照组比较显著增加(Pa<0.05,0.01);试验组轻症、重症患儿治愈率与对照组轻症、重症患儿治愈率比较显著增高(P<0.05,0.01).试验组患儿α1-酸性糖蛋白与血清IL-8呈显著正相关(u=0.387 P<0.05);α1-酸性糖蛋白与VT、Ti/Te、tPTEF/tE、vPTEF/VE呈显著负相关(u=-0.412,-0.403,-0.396,-0.385 Pa<0.05).口服氨茶碱控释片不良反应较少,偶见有心率增快.结论 口服氨茶碱能减轻毛细支气管炎患儿呼吸道炎性反应,改善肺功能,减轻小呼吸道阻塞,提高治愈率.     

闭塞性细支气管炎7例

目的分析小儿闭塞性细支气管炎(BO)的临床特点。方法回顾性分析本院呼吸科2004年10月-2009年9月诊断为BO的7例患儿的临床表现、胸部X线及高分辨率CT(HRCT)、病原学、肺功能及治疗和转归等。结果 7例患儿均表现为呼吸道感染后慢性咳喘、气促。病程1.5个月～7 a。查体:双肺均可闻及湿性啰音或喘鸣音。X线胸片:双肺纹理增粗或肺炎表现。肺部HRCT均呈现Mosaic灌注征,其中支气管扩张1例。肺功能:5例均为阻塞性通气功能障碍,舒张试验阴性。病原学:5例发病前患麻疹肺炎,1例患肺炎支原体肺炎,1例患肺炎腺病毒肺炎并真菌感染。支气管镜:2例无特殊表现。治疗及转归:1例有明显支气管扩张放弃治疗,1例因治疗过程中出现支气管扩张而放弃治疗,余5例患儿用激素或加服小剂量阿奇霉素,病情均有好转。结论BO的临床表现以慢性咳嗽、喘息为特点,肺部HRCT呈现特异的Mosaic灌注征,肺炎支原体、腺病毒尤其是麻疹病毒感染后可继发BO,肺功能为阻塞性通气功能障碍。BO的诊断主要依据典型的临床表现、肺部HRCT和肺功能检查。     

A Prospective Study on Non-IgE-Mediated Asthma in Children

ABSTRACT. From 1977 to 1980, seventy-two children were found to have intrinsic (non-IgE-mediated) asthma. Except for five patients, 4 boys and one girl, who apparently grew out of their asthma, the remaining 67 patients were followed for six years. During the study period, 12 patients, 10 boys and 2 girls, developed extrinsic (IgE-mediated) asthma. Nine of them (75%) had a positive family history of atopic disease, which was only the case in 12 (22%) of the patients who did not develop extrinsic disease. Hence, a change from intrinsic into extrinsic asthma is apparently common in boys, but seems to be rare in girls. In addition, in girls with intrinsic asthma, fixed or declining lung function tests followed by signs of pulmonary hyperinflation and lung fibrosis is common. Further, in the present series of children with intrinsic asthma, 5 patients, 4 girls and 1 boy, developed severe bronchiectasis. The majority of patients, who on repeated chest X-rays showed persistent hyperinflation of the lungs associated with fixed FEV₁/FVC levels and a marked increase of serum IgG-concentrations were those at risk to develop severe lung disease.     

High-Frequency Oscillatory Ventilation for Cardiac Surgery Children With Severe Acute Respiratory Distress Syndrome

Acute respiratory distress syndrome (ARDS) in children after open heart surgery, although uncommon, can be a significant source of morbidity. Because high-frequency oscillatory ventilation (HFOV) had been used successfully with pediatric patients who had no congenital heart defects, this therapy was used in our unit. This report aims to describe a single-center experience with HFOV in the management of ARDS after open heart surgery with respect to mortality. This retrospective clinical study was conducted in a pediatric intensive care unit. From October 2008 to August 2012, 64 of 10,843 patients with refractory ARDS who underwent corrective surgery at our institution were ventilated with HFOV. Patients with significant uncorrected residual lesions were not included. No interventions were performed. The patients were followed up until hospital discharge. The main outcome measure was survival to hospital discharge. Severe ARDS was defined as acute-onset pulmonary failure with bilateral pulmonary infiltrates and an oxygenation index (OI) higher than 13 despite maximal ventilator settings. The indication for HFOV was acute severe ARDS unresponsive to optimal conventional treatment. The variables recorded and subjected to multivariate analysis were patient demographics, underlying disease, clinical data, and ventilator parameters and their association with hospital mortality. Nearly 10,843 patients underwent surgery during the study period, and the ARDS incidence rate was 0.76 % (83/10,843), with 64 patients (77 %, 64/83) receiving HFOV. No significant changes in systemic or central venous pressure were associated with initiation and maintenance of HFOV. The complications during HFOV included pneumothorax for 22 patients. The overall in-hospital mortality rate was 39 % (25/64). Multiple regression analyses indicated that pulmonary hypertension and recurrent respiratory tract infections (RRTIs) before surgery were independent predictors of in-hospital mortality. The findings show that HFOV is an effective and safe method for ventilating severe ARDS patients after corrective cardiac surgery. Pulmonary hypertension and RRTIs before surgery were risk factors for in-hospital mortality.     

甲泼尼龙琥珀酸钠治疗婴幼儿毛细支气管炎的疗效

目的 观察甲泼尼龙琥珀酸钠治疗婴幼儿毛细支气管炎的疗效.方法 将65例毛细支气管炎患儿随机分为2组,均采用常规抗感染、对症、吸氧等综合冶疗,治疗组在此基础上加用甲泼尼龙琥珀酸钠注射液,1～2 mg/(次·kg),1次/d;对照组加用地塞米松针[0.5 mg/(kg·次),1次/d]治疗.结果 治疗组患儿在喘憋、肺部哮鸣音和湿啰音消失、住院天数方面,与对照组比较明显缩短,差异有显著性意义(Pa＜0.05).治疗组治愈32例,好转3例,治愈率91.43%;对照组治愈21例,好转9例,治愈率70%,治疗组治愈率显著高于对照组(χ2=4.93 P＜0.05).结论 甲泼尼龙琥珀酸钠治疗毛细支气管炎疗效显著.     

Family-Centered Assessment and Function for Children With Chronic Mechanical Respiratory Support

IntroductionThe purpose of this study was to assess health-related quality of life (HRQL) when managing severe, chronic respiratory insufficiency (CRI) at home.MethodsFamilies enrolled in a comprehensive program for CRI completed the Child Health Ratings Inventories with (a) parent-proxy ratings of the child's HRQL and (b) the parent's own HRQL. Psychometrics and known groups comparisons were reported. Linear regressions models assessed the effect of the parent's HRQL on proxy ratings.ResultsEighty-six parents (67%) completed the measures. The child global HRQL score was low (63.1, SD = 24.9), although ratings of “family life” were higher (73.8, SD = 26.5). The parent global HRQL and emotional functioning (EF) scores were low, despite preserved physical and role function. Having a child with a congenital versus an acquired disability was associated with higher parental EF (p = .004). Higher parental EF correlated with a higher global child HRQL (p < .001).DiscussionFamilies of children with CRI experience reduced HRQL. The Child Health Ratings Inventories is a valid tool for this assessment, identifying emotional distress and potential gaps in support.     

Incidence of Clostridium difficile in Hospitalized Children A Prospective Study

Abstract. A total of 394 children, aged 0-14 years, referred to a paediatric department were investigated for the occurrence of Clostridium difficile, its cytotoxin in faeces, and its clinical significance in a prospective study over one year. Of the children in the investigation, 337 suffered from gastrointestinal diseases or had been treated with antibiotics prior to the investigation. Twenty-four percent of these children had Cl. difficile in one or more faecal samples and toxin was demonstrated in half of these patients. In 18/148 (12%) of the patients with acute gastroenteritis Cl. difficile was isolated as the only pathogen. In contrast, among 57 control children with no gastrointestinal symptoms and no prior antibiotic treatment significantly fewer harboured Cl. difficile (p<0.01). Cl. difficile was isolated with similar frequency in children with or without prior antibiotic treatment. Isolation of Cl. difficile was significantly higher in patients under one year of age p<0.001). In 78% of the patients with Cl. difficile the bacteria were found in faeces during the initial two days of hospitalization indicating that most of the cases were community acquired. The occurrence of Cl. difficile was not influenced by seasonal variation and the distribution between sexes was equal.     

A Multicenter, Long-Term Study on Arrhythmias in Children with Ebstein Anomaly

To assess the prevalence, history, and treatment of arrhythmias, in particular preexcitation and Wolff–Parkinson–White (WPW) syndrome, in patients with Ebstein anomaly (EA) during childhood and adolescence, we performed a multicenter retrospective study of all consecutive live-born patients with EA, diagnosed, and followed by pediatric cardiologists between 1980 and 2005 in The Netherlands. During a follow-up after EA diagnosis of 13 years 3 months (range: 6 days to 28 years 2 months), 16 (17%) of the 93 pediatric EA patients exhibited rhythm disturbances. Nine patients showed arrhythmic events starting as of the neonatal period. Supraventricular tachycardia was noted in 11 patients. One patient died in the neonatal period due to intractable supraventricular tachycardia resulting in heart failure and one patient died at 5 weeks of age most probably due to an arrhythmic event. The 14 surviving patients all show preexcitation, albeit 4 of them intermittently, and all have a right-sided accessory pathway location. Nine patients underwent catheter ablation of an accessory pathway. Only four patients are currently on antiarrhythmic drugs. The 17% prevalence of rhythm disturbances in pediatric EA patients, most commonly supraventricular arrhythmias, is significantly lower than in adult EA patients. Life-threatening rhythm disturbances are not frequent early in life. Symptomatic patients are well treated with radiofrequency catheter ablation.     

生酮饮食治疗儿童难治性癫痫的前瞻性研究

目的 评估生酮饮食(KD)在难治性癫癎患儿治疗中的疗效和安全性.方法 对KD治疗的难治性癫癎患儿22例进行前瞻性研究,观察期为12周.男16例,女6例.22例患儿中,婴儿痉挛症13例,Lennox-Gastaut综合征4例,Dravet综合征2例,未分类者3例.按照Johns Hopkins方案的改良方法 配制KD.每天监测尿酮体确保酮症状态;记录发作频率和形式以确定起效时间,通过Engel分级评估疗效,KD引入前、引入第4周、引入第12周行血液生化检查并判断KD对难治性癫癎患儿血糖和血脂的影响,监测并处理患儿不良反应.结果 所有病例至少予KD治疗2周,19例至少完成4周,10例至少完成12周.16例患儿出现癫癎缓解,均于3周内(1～15 d)起效,第1周尤为明显.8例发作曾完全控制,均于5周内(1～32 d)发生.达Ⅰ、Ⅱ级疗效者分别占36.4%(8/22例)、22.7%(5/22例).性别、年龄、治疗前病程、病因和不同分型对疗效无明显影响.尽管血糖于KD引入第4周时较基础值明显升高,但仍维持在正常范围;三酰甘油和胆周醇于KD引入第12周时较基础值明显升高,超过或接近正常值高限.不良反应主要为可耐受的消化道症状和代谢紊乱.结论 KD具有起效迅速、有效率高、不良反应轻微等特点,是一种可行的抗癫癎方式.