弥漫性泛细支气管炎

本文介绍DPB 的病因、流行病学、病理学、临床特点、诊断和治疗。我国有无此病尚待研究.     

弥漫性泛细支气管炎一例

弥漫性泛细支气管炎一例贺正一刘晓霞李铁一焦蕴敏刘绍珊患者男，４３岁。１９９５年１１月２日因咳嗽，咳痰，活动后气短１７年，发热５天住院。患者从２６岁开始经常咳嗽，咳黄痰，症状逐年加重。１９８７年８月因咯血痰，怀疑支气管扩张住院治疗，行支气管碘油造影未见...     

注意识别弥漫性泛细支气管炎

弥漫性泛细支气管炎(ＤＰＢ)是完全不同于慢性阻塞性肺疾病(ＣＯＰＤ)的独立的疾病。发现ＤＰＢ的历史背景要追朔到1967年加拿大Ｍａｃｋｌｅｍ等提出小气道病概念的同一时期,日本的本间、中山、泷泽3位学者从病理学、临床及Ｘ线学报道了属于小气道范围的以呼吸性细支气管为主要区域的弥漫性炎症性病变,并将其命名为ＤＰＢ。至今病因尚不清楚。ＤＰＢ的病理学特点是其炎症病变累及呼吸性细支气管组织的全层,故称之为泛细支气管炎。由于炎症病变致使管腔狭窄和形成散在息肉样肉芽肿以及不同程度的管壁纤维化,与肺Ｘ线影像上见到的散在性粟粒…     

弥漫性泛细支气管炎4例

弥漫性泛细支气管炎（diffusepanbronchiolitis,DPB）是一种以弥漫存在于两肺呼吸性细支气管区域的慢性炎症为特征,引起严重呼吸障碍而病因尚属不明的一种疾病。突出的临床表现是咳嗽、咳痰及活动时气促。临床上常易误诊为慢性阻塞性肺病、支气管扩张等疾病。我院2009年5月至2013年6月收治4例DPB患者资料,报道如下。     

弥漫性泛细支气管炎研究进展

弥漫性泛细支气管炎是近 2 0余年来发现的新病种。病因不明。早期以日本报道为主。近年来全球均有报道。笔者复习了有关弥漫性泛细支气管炎的国内外文献 ,对本病进行如下综述     

弥漫性泛细支气管炎一例

弥漫性泛细支气管炎一例王厚东孙铁英李燕明患者男性，１９岁。因反复咳嗽、咳痰４年，加重伴劳累性呼吸困难８个月于１９９５年９月２７日入院。患者从１５岁起反复咳嗽，间断伴有咳痰，１９９５年１月因咳嗽、咳痰，发热，活动后呼吸困难于当地医院就诊，胸部Ｘ线检查发...     

弥漫性泛细支气管炎研究进展

弥漫性泛细支气管炎是近20余年来发现的新病种。病因不明。早期以日本报道为主。近年来全球均有报道。笔者复习了有关弥漫性泛细支气管炎的国内外文献．对本病进行如下综述。     

弥漫性泛细支气管炎和大环内酯类药物疗法

弥漫性泛细支气管炎 (ＤＰＢ)是东亚地区所特有的人种特异性疾病 ,其疾病概念和治疗方法均确立于日本。随着红霉素疗法的发现和确立 ,ＤＰＢ的预后有了划时代的变化。最近 ,关于ＤＰＢ研究的最大进步有以下两个方面∶第一 ,关于ＤＰＢ的人种特异性和疾病易感基因的研究 ;第二 ,关于红霉素及其它 14员大环内酯类药物疗法的作用机制的研究。一、弥漫性泛细支气管炎1.ＤＰＢ的概念和诊断标准∶ＤＰＢ是弥漫存在于两肺呼吸细支气管区域的以慢性炎症为特征的特殊气道疾病 ,可导致严重的呼吸功能障碍。其疾病概念于 196 9年由日本的本间、山中等[1…     

弥漫性泛细支气管炎9例临床分析

目的 增强对弥漫性泛细支气管炎(DPB)临床特征的认识及诊断. 方法 收集南京医科大学第一附属医院呼吸科9例曾被误诊的DPB患者,通过实验室检查、肺功能及动脉血气检查、影像学检查等分析其临床特点. 结果 所有患者均有慢性咳嗽、咳痰、活动后气促的症状,合并副鼻窦炎.9例患者用阿奇霉素(500mg/d),联合抗生素、氧疗和对症、支持疗法等方法治疗后症状均有明显改善、痰量减少,肺部高分辨CT提示明显好转. 结论 DPB具有一定的临床特异性,只有正确地认识及诊断才能改善其预后.     

Diffuse panbronchiolitis in China

OBJECTIVE: Diffuse panbronchiolitis (DPB) is a progressive inflammatory disease, well recognized in Japan, that is characterized by chronic sinusitis and obstructive small airway disease. A total of 40 patients with DPB in mainland China were reviewed in order to describe the epidemiology of DPB in China and to compare their clinical characteristics with those of cases reported in Japan. METHODOLOGY: A systematic search was conducted of the Chinese language literature published in mainland China from 1996 to 2003. The 40 subjects who fulfilled the diagnostic criteria for DPB were retrospectively analysed for clinical, physiological, radiological, and pathological features and their geographic distribution. RESULTS: The 40 patients included in this study (31 male and nine female) were distributed in 12 provinces and three other cities in China. All presented with chronic cough, sputum production, and 32 had exertional dyspnoea. All but three had a history of sinusitis. Lung function assessment showed a mixed obstructive-restrictive pattern in 30 cases. CXR revealed diffuse fine nodular shadows in all patients. Human leukocyte antigen-B54 was positive in five patients (n = 12). A total of 30 (75%) patients had been misdiagnosed prior to their diagnosis of DPB being made. CONCLUSIONS: DPB is not rare in China but its incidence is relatively low. Poor recognition and/or genetic factors may be the reason. The clinical, radiographic and histological features of Chinese patients resemble those described in Japanese patients.     

Childhood diffuse panbronchiolitis: a case report

Diffuse panbronchiolitis (DPB) is a chronic, potentially life-threatening lower respiratory tract disease that is particularly common in Japanese people. If left untreated, it progresses to bronchiectasis, respiratory failure, and death. Lack of familiarity with DPB in the non-Far East may result in a failure to correctly diagnose and treat this disorder. We describe a child with DPB. We suggest that DPB is a sinopulmonary disease that is not exclusive to the Asian population and to adults. Its clinical and radiological features should be better known by pediatric pulmonary physicians.     

弥漫性泛细支气管炎的免疫学发病机制

弥漫性泛细支气管炎(diffuse panbronchiolitis,DPB)患者的呼吸性细支气管区域有淋巴细胞、浆细胞、巨噬细胞浸润和聚集,支气管组织中的树突细胞异常以及支气管肺泡灌洗液CD4+/CD8+细胞比值增高.血冷凝集试验效价持续升高.IgA增高等提示DPB的发病可能与免疫功能紊乱有关.     

Diffuse panbronchiolitis as a pulmonary complication in patients with adult T-cell leukemia

Forty-three cases of adult T-cell leukemia (ATL) admitted to our hospital between 1982 and 1987 were studied. Three of those were found to be complicated with diffuse panbronchiolitis (DPB). The incidence of DPB is considered to be significantly higher in patients with ATL. The three DPB-complicated cases composed one case each of the smoldering, chronic, and acute type of ATL. In each type, DPB preceded overt ATL and Candida albicans was found in sputa following detection for bacteria. The DPB complication apparently worsened the prognosis of the ATL patients. We have discussed a possible relationship between ATL and DPB.     

弥漫性泛细支气管炎2例临床分析并文献复习

目的 探讨弥漫性泛细支气管炎(DPB)的临床特点及治疗.方法 回顾性分析我院2例确诊为DPB患者的临床特点及治疗,并复习相关文献.结果 2例均以慢性咳嗽、咳痰伴活动后气促为主诉,肺部听诊均闻及湿啰音,HLA-B54均阳性,但冷凝集试验均阴性,肺功能均以阻塞性通气功能障碍为主;1例经病理确诊,胸部影像见典型弥漫小叶中心性结节影,但无慢性鼻窦炎病史,口服阿奇霉素0.25g,隔日1次,治疗12个月后肺部病灶明显吸收;1例经临床确诊,胸部影像见典型弥漫结节影伴支气管扩张和间质性肺纤维化,既往有慢性鼻窦炎病史,口服阿奇霉素0.25 g,隔日1次,治疗7个月后肺部病灶明显吸收;2例患者初期均长期误诊.结论 2例患者具有DPB典型临床表现,但某些特征有别于一般日本DPB患者,2例病例均显示长期口服小剂量阿奇霉素治疗DPB效果显著.     

Clinical efficacy of the FLUTTER device for airway mucus clearance in patients with diffuse panbronchiolitis

Abstract Expectoration of mucus is important in preventing the development of airway inflammation in patients with diffuse panbronchiolitis (DPB). To evaluate the clinical efficacy of the FLUTTER device in clearing mucus from the airways of patients with DPB who have difficulty expectorating, we assessed pulmonary function and symptoms in patients treated with FLUTTER. Eight patients in a stable clinical condition with DPB were included in the study. The study was divided into two consecutive, 1-week periods. The initial week was an observation week. During the following week, patients used FLUTTER four times daily. Expectorated sputum was collected in a container and weighed every day during 2 weeks. Pulmonary function, partial oxygen pressure and partial carbon dioxide pressure in arterial blood were measured in all patients on the last day of the observation week and the FLUTTER treatment week. A symptom score for difficulty of expectoration was determined by questionnaire. A pneumothorax developed in one patient during using FLUTTER. The mean daily sputum weight and peak expiratory flow rate increased significantly after treatment with FLUTTER ( P < 0.04 and P< 0.02, respectively). Symptom score improved significantly after using FLUTTER ( P < 0.02). We conclude that the use of FLUTTER is effective in clearing mucus from the airways. However, the development of a pneumothorax may complicate use of the procedure in some cases.     

胸腺瘤伴弥漫性泛细支气管炎一例报告并文献复习

目的　提高对弥漫性泛细支气管炎 (DPB)的认识。方法　对 1例经胸腔镜肺活检病理确诊为DPB患者的临床资料进行分析 ,并结合文献复习。结果　DPB是一种慢性小气道疾病 ,以两肺弥漫性呼吸性细支气管的炎症为特征。DPB的病因及发病机制尚不清楚 ,可能与遗传和免疫因素有关。临床表现为慢性咳嗽、咳痰、活动后气短 ,常合并铜绿假单胞菌感染。如不治疗 ,患者可发展为呼吸衰竭 ,预后不良。结论　遇到不明原因的双肺结节状阴影应想到DPB ,并争取做肺组织活检以明确诊断。长期低剂量红霉素治疗可能通过控制炎症作用改善患者预后