弥漫性泛细支气管炎一例及文献综述

目的探讨儿童弥漫性泛细支气管炎的诊断和治疗。方法总结1例弥漫性泛细支气管炎患儿的临床、影像学表现以及病理改变,并复习相关文献。结果患儿临床表现为长期咳嗽、咯痰伴喘息,逐渐出现呼吸困难。双肺闻及较多细湿哕音及喘鸣音,杵状指可疑。胸部X线片：两肺透光度增强,左下肺见多发结节及网状影。胸部高分辨CT：两肺广泛分布小叶中心性细小结节影,左下肺局部小支气管壁增厚,双下叶和右中叶少许支气管轻微扩张。副鼻窦科瓦位提示副鼻窦炎。支气管镜肺活检病理提示支气管上皮破坏,管壁可见大量淋巴细胞、少量泡沫状组织细胞、中性粒细胞浸润,有淋巴滤泡形成,周围肺泡壁有少许淋巴细胞、组织细胞浸润,肺泡壁组织增生不明显,偶见局部纤维化并突入肺泡腔。常规肺功能提示混合性通气功能障碍,支气管舒张试验阳性。PaO2 65mm Hg。根据临床、影像表现、病理改变以及目前弥漫性泛细支气管炎的诊断标准,确诊为弥漫性泛细支气管炎。给予小剂量红霉素[5～10mg／（kg·d）]治疗,患儿病情明显好转。结论弥漫性泛细支气管炎可发生于中国儿童。主要诊断依据为慢性咳嗽、咯痰伴喘息,胸部CT表现为两肺弥漫性分布的小结节影,为细支气管中心性或小叶中心性,伴有副鼻窦炎。小剂量红霉素治疗可控制病情。     

儿童支气管扩张症172例临床研究

目的 探讨儿童支气管扩张症临床表现、 影像学及肺功能方面特点, 分析病因构成, 了解疾病预后。方法 回顾性总结 172 例支气管扩张症患儿的临床表现、 影像学、 肺功能特点及病因, 对部分患儿进行随访。结果 支气管扩张症患儿临床主要表现为咳嗽、咯痰、生长发育受限、杵状指等。胸部X线诊断支气管扩张症的检出阳性率（4.42%）显著低于高分辨率CT（HRCT）的100%。存在免疫缺陷、闭塞性细支气管炎及原发性纤毛运动障碍的患儿在HRCT中弥漫性支气管扩张较其他病因更显著（P＜0.05）。76.92%的患儿肺功能异常。65.70%的患儿可以发现潜在病因,以肺部感染（31.40%）为主。门诊随访患儿FEV1%随病程的延长以每年1.28%的速度下降。其中1例临床症状、HRCT、肺功能均有好转。结论 儿童支气管扩张症临床表现没有明显特异性。大部分患儿可以发现潜在病因,以肺部感染最为常见。对于临床怀疑支气管扩张症的患儿应尽早进行HRCT检查,积极寻找潜在病因。支气管扩张症患儿的肺功能可随着病程的进展缓慢下降。部分患儿在临床症状、HRCT及肺功能上可有好转。     

儿童闭塞性细支气管炎的研究进展

闭塞性细支气管炎是指小气道损伤后炎症及纤维化引起的慢性气流阻塞的临床综合征,儿童表现为重症呼吸道感染后持续咳嗽、喘息及活动不耐受.重症下呼吸道感染、心肺移植、骨髓移植、Stevens-Johnson综合征、结缔组织病、吸入或摄入有毒物质及药物因素等均可导致闭塞性细支气管炎的发生.闭塞性细支气管炎诊断主要依据临床表现、高分辨率CT和肺功能检查,肺组织活检是诊断闭塞性细支气管炎的金标准.目前尚没有公认的闭塞性细支气管炎治疗准则.早期阶段是临床治疗的关键时期.多数采取持续使用糖皮质激素和支气管舒张剂,同时联合其他支持治疗.该文从病因、发病机制、病理、临床表现、辅助检查、诊断、鉴别诊断及治疗方面对儿童闭塞性细支气管炎进行综述.     

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。     

29例小儿间质性肺疾病临床、放射及病理分析

目的提高对小儿间质性肺疾病的认识。方法收集2001年3月至2009年12月住院肺活检的弥漫性肺疾病患儿29例,对其临床表现、肺CT或高分辨CT(HRCT)、肺组织病理特点进行回顾性分析。结果临床表现为咳嗽29例,呼吸困难15例,肺部湿啰音7例,杵状指趾9例。胸CT或HRCT显示:19例以磨玻璃影为主,5例为结节影,5例以多发实变影为主。根据肺病理的改变,结合临床和影像学特点,确诊特发性间质性肺炎18例,包括非特异性间质性肺炎(NSIP)8例、急性间质性肺炎(AIP)3例、隐源性机化性肺炎(COP)3例、淋巴细胞性间质性肺炎(LIP)2例、呼吸性细支气管炎伴间质性肺炎(RBILD)1例、未分型特发性间质性肺炎1例;其他间质性肺疾病11例,包括弥漫性泛细支气管炎2例、过敏性肺炎2例、类脂性肺炎2例、肺泡蛋白沉着症2例、肺泡微石症1例、特发性肺含铁血黄素沉着症1例、结节病1例。其中21例患儿应用糖皮质激素治疗,16例有效。结论小儿间质性肺疾病的主要临床表现为咳嗽和呼吸困难,肺CT表现主要为磨玻璃影、结节影,肺组织病理为小儿间质性肺疾病诊断的主要依据。     

儿童闭塞性细支气管炎的研究进展

闭塞性细支气管炎在儿童中是一种相对少见而严重的慢性阻塞性肺疾病.以感染为最常见原因,主要临床特点是反复或持续咳嗽、气促、喘息,肺部有湿啰音和喘鸣音.目前诊断主要依赖临床表现、肺功能和高分辨率CT检查.目前尚无统一治疗标准,以经验治疗为主.该文主要对儿童闭塞性细支气管炎的辅助检查、诊断、治疗进行综述.     

儿童闭塞性细支气管炎28例临床分析

目的：总结儿童闭塞性细支气管炎（BO）的临床特点,以期提高临床医师对儿童BO的认识。方法：回顾性分析2007年7月至2012年4月经临床诊断为BO的28例住院患儿的临床资料,并随访其预后。结果：所有患儿均表现有反复喘息及咳嗽。感染后闭塞性细支气管炎（PIBO）23例,其中腺病毒肺炎12例,麻疹病毒肺炎和流感病毒A肺炎各2例,肺炎支原体肺炎、腺病毒并肺炎支原体肺炎、呼吸道合胞病毒并副流感病毒3肺炎、肺结核各1例,病原不明3例;非感染性因素导致BO 3例,即Steven-Johnson 综合征、副肿瘤天疱疮、造血干细胞移植后发生BO各1例;病因不明2例。28例胸部HRCT结果示充气不均25例,马赛克灌注征21例,支气管壁增厚15例,支气管扩张12例,气体潴留征6例。21例行肺功能检查,均提示有阻塞性通气功能障碍。18例行舒张试验,17例阴性。所有患儿均使用糖皮质激素治疗,24例口服小剂量阿奇霉素治疗。住院期间死亡1例,18例随访4个月~?4年7个月,临床表现好转12例,无好转5例,死亡1例。结论：下呼吸道感染是儿童BO最常见病因,腺病毒是主要感染病原;BO临床上主要表现为反复喘息、咳嗽,但无特异性;胸部HRCT可显示BO较特征性征象,是BO诊断和病情随访的重要指标;BO肺功能特异性地表现为不可逆的阻塞性通气功能障碍;糖皮质激素、阿奇霉素是可能有效的治疗药物;儿童BO总体预后不好,早期诊断治疗、避免反复呼吸道感染有望改善儿童BO预后。     

闭塞性毛细支气管炎7例临床分析

目的 了解小儿闭塞性毛细支气管炎(BO)的临床特点.方法 回顾性分析2005年9月至2008年5月吉林大学第一医院临床诊断为BO的7例惠儿的临床表现、X线胸片及高分辨CT(HRCT)、肺功能检查、治疗及随访.结果 7例均表现为呼吸道感染后慢性咳嗽、喘息、气促,2例病程6周以上,其余5例病程均在8～16周以上.查体均有呼吸急促,双肺闻及湿哆音、喘鸣音.X线胸片7例均有过度通气,2例有斑片状影,2例呈毛玻璃样改变.HRCT:斑片影7例,Mosaic灌注6例,支气管壁增厚2例,支气管扩张2例,肺不张1例.肺功能均为阻塞性通气功能障碍.血气分析显示不同程度的低氧血症.3例行纤维支气管镜检查无特异性.7例惠儿均口服糖皮质激素治疗,3例配合应用吸入激素(普米克令舒).4例随访3～12个月,其中2例病情有改善,呼吸急促及运动不耐受明显减轻,肺内啰音明显减少或消失,肺CT有所改善;2例仍有活动后气促,肺CT无明显改善.结论 BO肺功能为不可逆性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.     

儿童闭塞性细支气管炎42例临床分析

目的 探讨儿童闭塞性细支气管炎的临床特点.方法对北京儿童医院2001年4月-2007年8月诊断闭塞性细支气管炎的42例患儿进行分析,内容包括患儿年龄、性别、临床表现、病因、肺功能改变、影像学改变、治疗及随访预后等方面.结果 42例患儿(男31例,女11例)诊断时年龄最小的7个月,最大的12岁2个月,均以持续咳嗽、喘息为主要表现.肺部体征有喘鸣音及湿性啰音.病因为感染后32例(76.2%),其中考虑为腺病毒肺炎者8例(占感染后闭塞性细支气管炎的25%),考虑为麻疹肺炎者7例(占感染后闭塞性细支气管炎的21.9%),考虑为呼吸道合胞病毒感染者2例(占感染后闭塞性细支气管炎的6.2%).病因为Steven-Johnson综合征的4例(9.5%).骨髓移植后1例(2.4%).肺功能表现为小气道阻塞性通气功能障碍35例(89.7%),混合性通气功能障碍4例(10.3%).肺CT有典型的马赛克灌注征者34例(81.0%),支气管扩张14例(33.3%),支气管壁增厚14例(33.3%),肺不张4例(9.5%),合并Swyer-James综合征2例(4.8%).所有患儿均予皮质醇激素、小剂量红霉素或阿奇霉素口服治疗,辅以糖皮质激素和支气管扩张剂吸入,或白三烯受体拮抗剂口服治疗.随访1个月至5年,除2例临床表现及影像检查、肺功能有一定程度好转外,其余患儿均有不同程度的加重,死亡1例.结论儿童闭塞性细支气管炎多为感染后起病,其特征性的临床、影像学和肺功能表现基本可以确定诊断.该病预后不佳.     

儿童特发性肺间质纤维化临床表现与辅助检查

目的探讨儿童特发性肺间质纤维化(IPF)的临床表现及辅助检查诊断价值。方法回顾性分析17例IPF患儿的临床资料及胸部X线、高分辨率CT(HRCT)、纤维支气管镜、肺功能、血气分析、肺活检等辅助检查资料。结果 IPF患儿临床以咳嗽、气促、发热、进行性呼吸困难伴肺底部Velcho啰音为主要表现;胸片/HRCT主要显示双肺斑片状、网格状或实变影、毛玻璃影、马赛克征。肺功能多数呈限制性通气功能和弥散障碍,血气分析提示低氧血症,17例中达呼吸衰竭诊断标准者5例。纤维支气管镜检查可见气管支气管内膜炎,支气管软化、扩张、狭窄等,无诊断特异性表现。1例患儿死亡后行肺活检,显示肺弥散性炎症伴灶型坏死,成人型小儿肺透明膜病。结论儿童IPF的诊断主要根据典型临床表现,结合胸片、HRCT、肺功能、血气分析等辅助检查,胸部影像学尤其HRCT是能反映IPF肺组织结构改变的敏感方法,纤维支气管镜检查有重要鉴别诊断作用,不典型病例确诊还需肺活检。     

经支气管镜介入治疗儿童原发性气道肿瘤八例分析

目的了解儿童气道肿瘤经支气管镜介入治疗的可行性。方法对2016年11月至2018年3月在首都医科大学附属北京儿童医院住院经支气管镜介入治疗的8例原发性气道肿瘤患儿的临床表现、介入治疗结果和预后情况进行回顾性分析。结果8例原发性气道肿瘤患儿中,男5例、女3例,年龄4岁8月龄至9岁2月龄。临床表现主要为咳嗽伴喘息6例、咯血3例和反复肺部感染2例,均无淋巴结转移及肺外转移。8例患儿均经支气管镜介入肿瘤切除,对4例富含血流的肿瘤患儿支气管镜介入治疗术前行肿瘤供血处支气管动脉栓塞术。术中及术后未发生大出血、气胸等并发症。4例为低度恶性支气管黏液表皮样癌,2例为炎性肌纤维母细胞瘤,1例为多形性腺瘤,1例为支气管平滑肌梭形细胞瘤。术后随访2~4年,复发3例,其中2例炎性肌纤维母细胞瘤患儿中,1例因病灶位置深行外科切肺治疗,1例联合化疗治疗后病灶稳定;1例黏液表皮样癌介入冷冻治疗后随访中。结论儿童气道肿瘤临床表现不典型,主要有干咳、喘息、反复肺部感染和咯血等症状。经支气管镜下介入治疗儿童气道内良性和低度恶性肿瘤,是一种可行的选择。     

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??Bronchiolitis is a common respiratory disorder in childhood and bronchiolitis generally refers to a group of disorders in which a variety of pathogenic factors cause bronchiolar damage??resulting in reactive inflammatory cell infiltration and fibrous tissue hyperplasia. Pathologically bronchiolar diseases are divied into primary and secondary categories. Bronchioles are the end point of airway conduction area and its unique anatomical and physiological characteristics make it a good place for many diseases. Acute infectious bronchiolitis is one of the most common types??while bronchiolitis obliterans??BO?? is attracting more and more attention in pediatric clinical practice and diffuse pan-bronchiolitis??DPB?? has also been reported in children. Pediatricians need to improve their knowledge of bronchiolitis. Bronchiolar diseases and diffuse interstitial lung fibrosis are quite overlapped. It is necessary to take a holistic view of the intrinsic relationship between lung parenchyma and pulmonary interstitial. It should be regarded as the only correct choice to make standard diagnosis and treatment of bronchiolitis.     

支气管镜检查在儿科临床中的应用

支气管镜检查在儿科肺疾病的评估和治疗中发挥了很重要的作用.支气管肺泡灌洗、支气管刷检及活检为诊断提供了很多帮助,甚至在很小的患儿中也能安全施行.文章描述了纤维支气管镜检查的经典过程及其在临床中适应证.     

Pediatric postprimary pulmonary tuberculosis

BACKGROUND: Postprimary pulmonary tuberculosis (TB) is not commonly seen in children. OBJECTIVE: The purpose of this study was to determine the radiographic findings and patient characteristics of pediatric postprimary pulmonary TB. MATERIALS AND METHODS: We reviewed the clinical charts and chest radiographs in six patients. RESULTS: The radiographic findings of pediatric postprimary pulmonary TB include upper-lobe consolidation and cavitation, multifocal ill-defined airspace opacities, evidence of prior pulmonary TB, and apical pleural thickening. Pleural effusions and lymphadenopathy are not commonly present. Although postprimary disease typically does not affect young children, five of the children in this series were less than ten years of age at the time of presentation. CONCLUSION: The possibility of postprimary TB should be considered in pediatric patients at risk for this disease who present with upper-lobe pulmonary consolidation and cavitation. These patients are highly infectious and early recognition and treatment can limit transmission of TB.     

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??Objective To approach the diagnosis and treatment of eosinphilic gastroenteritis in children. Methods In 12 cases of children with eosinophilic gastroenteritis in Nanjing Children's Hospital Affiliated to Nanjing Medical University from May 2009 to Nov.2011??the clinical manifestations??laboratory data??endoscopic and pathological findings and treatment were collected and analyzed. Results In the 12 patients??mucosal type was present in 8 cases??muscle-type in 1 case??and serous type in 3 cases. Most of the pediatric eosinophilic gastroenteritis had no specific clinical manifestations. From direct endoscopic vision??edema and erosion were the main performance of gastrointestinal mucosa. Histopathologic examination revealed that there was dense infiltration in gastrointestinal mucosa lamina propria by eosinophils. The effects of dietetic and antianaphylactic treatments were good. Conclusion The clinical manifestations of pediatric eosinophilic gastroenteritis are various and nonspecific. The dietetic and antianaphylactic treatments have obvious therapeutic effect . About 1 to 2 weeks of glucocorticoids therapy can rapidly alleviate the symptoms??and it continues for 3 months by reducing the dosage gradually.     

经支气管镜介入治疗在气道发育异常中的应用

支气管镜是诊治儿童呼吸系统疾病不可缺少的方法之一,是儿童肺介入治疗技术不断发展的基础。随着临床经验的不断积累及支气管镜在儿科的广泛应用,其价值得到了一致肯定。该文将对经支气管镜介入治疗在气道发育异常中的应用概况作一综述。     

28例儿童肺部弥漫性疾病的病因和诊断分析

目的 探讨儿童肺部弥漫性疾病的病因和诊断思路。方法 回顾性分析28例肺部弥漫性疾病患儿的诊断以及确诊过程。结果 确定病因25例,包括支原体肺炎1例、沙眼衣原体肺炎2例、巨细胞病毒肺炎2例、EB病毒肺炎1例、血型播散性肺结核3例、金黄色葡萄球菌败血症性肺炎1例、肺隐球菌病1例、侵袭性肺曲霉菌病2例、广泛支气管扩张合并肺部感染2例、特发性肺含铁血黄素沉着症1例、特发性肺纤维化1例、HIV合并淋巴细胞间质性肺炎1例、鸽粪引起的外源性变应性肺泡炎1例、韦格内肉芽肿1例、郎格罕细胞组织细胞增生症2例、恶性淋巴瘤3例。疑似诊断3例,包括奴卡菌感染1例、少年类风湿性关节炎合并肺纤维化1例、HIV合并卡氏肺囊虫1例。18患儿经X线检查、病史和体格检查以及其他非创伤性检查诊断,8例经皮肤活检或肺活检诊断,2例由尸解确诊。结论 儿童肺部弥漫性疾病的病因包括肺部感染性疾病、特发性疾病和全身疾病的肺部表现。影像学表现、病史和体格检查以及其他非创伤性检查能确定多数患儿的病因,少数病例需经创伤性检查诊断。     

Noninvasive positive-pressure ventilation in children with lower airway obstruction.

STUDY OBJECTIVES: Mechanical ventilation of patients with severe lower airway obstruction presents significant risks; therefore, avoiding the intubation in these patients has been a principal goal of clinical management. Noninvasive positive-pressure ventilation has been shown to be effective in treating adults with chronic obstructive pulmonary disease, but its use has not been studied prospectively in children with acute obstructive lower airways disease. The objective of this study was to determine whether noninvasive mask ventilation improved respiratory function in children with asthma and other obstructive lower airways diseases. STUDY DESIGN: A prospective, randomized, crossover study. PATIENTS: A total of 20 children admitted to the pediatric intensive care unit with acute lower airway obstruction. METHODS: Children were randomized to receive either 2 hrs of noninvasive ventilation followed by crossover to 2 hrs of standard therapy or 2 hrs of standard therapy followed by 2 hrs of noninvasive ventilation. RESULTS: Using a Clinical Asthma Score, we found that noninvasive ventilation decreased signs of work of breathing such as respiratory rate, accessory muscle use, and dyspnea as compared with standard therapy. There was no serious morbidity associated with noninvasive ventilation. CONCLUSIONS: We conclude that noninvasive ventilation can be an effective treatment for children with acute lower airway obstruction.     

儿童原发恶性气道肿瘤四例临床分析

目的 总结4例临床较为罕见的恶性肿瘤,旨在引起临床重视,并探讨气管镜介入治疗的安全性和疗效.方法 回顾性分析经气管镜病理确诊的4例儿童恶性气管肿瘤,探讨其临床特征、胸部CT及气管镜表现.结果 2例为高度恶性(分别为淋巴瘤和肉瘤),2例为低度恶性肿瘤(黏液表皮样癌).发病年龄7个月～7岁.所有患儿均无特征性临床表现,均出现咳嗽、喘息等症状,或伴咯血,术前均误诊为支气管炎、支气管哮喘或肺不张等.胸部CT提示气道内类圆形肿物,2例发生于主气管,2例发生于两侧支气管,均有阻塞性肺不张.4例患儿均行气管镜下介入治疗(1例局部麻醉,3例全身麻醉),手术成功3例.结论 儿童气道恶性肿瘤无特征性表现,常出现咳嗽、喘息等呼吸道症状.对不能手术切除的肿瘤,气管镜下介入治疗可取得良好效果,以全身麻醉下治疗较为安全.

Abstract：

Objective Primary airway neoplasms are extremely rare in the pediatric age group. This paper reports 4 children with primary airway neoplasms to explore the clinical manifestations, safety and efficacy of bronchoscopic interventions. Method The data of pathology, photographic documentation and imaging studies were analyzed. Result Of the 4 reported lesions, 2 were characterized by low-grade (2 with mucoepidermoid carcinoma) and 2 by high grade malignant (spindle cell carcinoma and malignant lymphoma). Onset of clinical manifestations occurred at the ages of 7 months to 7 years. All of them were initially misdiagnosed as bronchitis, asthma or atelectasis. The lesions located in trachea in 2 patients, in left bronchus of 1 patient and in right middle bronchus of 1 case. Atelectases occurred in bilateral bronchus where the lesions obstructed almost the entire lumen at the time of diagnosis. The diagnosis of airway masses depends upon maintaining a high index of suspicion, complemented by imaging and timely diagnostic endoscopy. The lesions were completely removed in 3/4 patients except 1 died during bronchoscopic procedures. Conclusion The children with malignant airway neoplasms were presented with cough and wheezing without specific manifestations. Bronchoscopic interventions were effective in the treatment of non-operative cases. General anesthesia is strongly recommended for interventional bronchoscopy.