原发性气管支气管淀粉样变1例及文献复习

目的:探讨原发性气管支气管淀粉样变的流行病学特征、临床表现及诊断和治疗方法。方法:回顾1例原发性气管支气管淀粉样变病例的临床特点,并结合文献进行分析。结果:患者主要表现为咳嗽、咳痰、咯血及呼吸困难,经纤支镜活检标本刚果红染色阳性。经治疗后患者症状明显缓解。结论:原发性气管支气管淀粉样变临床上发病率极低,容易误诊,组织病理学检查是气管支气管淀粉样诊断的金标准,目前缺乏特异性治疗方法。     

原发性气管支气管肺淀粉样变1例

原发性气管支气管肺淀粉样变临床非常罕见,至今病因未明,极易误诊.现将我们近期遇到的1例原发性气管支气管肺淀粉样变报告如下,以提高临床医师对该病的诊断水平.     

原发性支气管肺淀粉样变的概述

原发性支气管肺淀粉样变是一种不可溶性淀粉样蛋白物质在气管、支气管及肺组织中沉积而表现各异的临床综合征,属少见病,且临床表现缺乏特异性,诊断主要依靠组织活检病理学检查。病程后期可出现支气管狭窄,引发呼吸困难及咯血等症状,预后差,目前尚无特异性治疗,故早诊断、早治疗对本病预后有所帮助。     

原发性结节型肺淀粉样变1例并文献复习

淀粉样变是淀粉样物质沉积于细胞、组织内,从而导致靶器官功能障碍的一组疾病。原发性肺淀粉样变是指在无继发或(和)系统性淀粉样变情况下,淀粉样物质沉积于肺内。其临床症状及影像学表现不典型,容易误诊。本文现回顾性分析我院1例经皮肺穿刺活检病理确诊的原发性结节型肺淀粉样变,同时复习相关文献资料,并对该病的临床症状、影像学、病理、诊断及治疗等进行描述,以提高对本病的认识。     

原发性支气管肺淀粉样变7例临床病理分析

目的探讨原发性支气管肺淀粉样变的临床表现、病理特征和诊断方法。方法对7例经病理诊断证实为原发性支气管肺淀粉样变的病例进行回顾性分析。结果本组男性5例,女性2例,男女之比为2.5∶1。以咳嗽、咳痰、气短、咯血等为主要临床表现;影像学主要表现为肺部斑片状或结节阴影和气管壁环形增厚、管腔狭窄及结节样隆起;病理学检查均查见淀粉样物质沉积于支气管黏膜下和肺组织中,刚果红染色阳性。结论该病临床及影像学表现无特异性,组织病理学检查及特殊染色是确诊的唯一方法;治疗方法主要有激光、药物和手术治疗。该病易复发,预后不良。     

原发性支气管肺淀粉样变9例临床分析

目的：探讨原发性支气管肺淀粉样变的临床表现、影像学特征和治疗措施。方法回顾分析我院1994-2012年收治的9例原发性支气管肺淀粉样变患者的临床资料并结合文献进行分析。结果9例中男6例,女3例,平均年龄54岁。肺实质结节型和气管支气管淀粉样变主要表现为咳嗽、咳痰,可伴咯血或无症状。肺间质弥漫型主要表现为胸痛、痰中带血。肺实质结节型胸部CT均表现为肺部结节影,气管支气管淀粉样变表现为气管、支气管管壁增厚,可伴有钙化,肺间质弥漫性淀粉样变表现为双肺弥漫分布小结节影、斑片影。肺实质结节型通常预后较好。气管支气管型病变者常反复感染加重,需间断气管镜下介入治疗,抗炎、止咳化痰、平喘等综合治疗有一定疗效。呈肺间质弥漫型病变者预后较差。结论原发性支气管肺淀粉样变临床和影像学表现无特异性,确诊依据病理学检查,抗炎等对症治疗可以缓解症状。     

长期误诊的原发性气管支气管淀粉样变

目的：探讨原发性气管支气管淀粉样变的临床症状和诊断方法。方法：分析1例被误诊多年的原发性气 管支气管淀粉样变病例的临床表现和诊断经过。结果：本例患者为68岁男性,30多年前开始出现反复咳嗽、咳痰以 及渐进性的呼吸困难,曾多次在外院就诊,诊断为慢性支气管炎、支气管扩张以及支气管结核,反复给与抗生素治 疗,并曾行抗结核治疗3个月,但治疗效果差,患者症状逐渐加重。患者来中南大学湘雅医院就诊后,经完善支气管 镜检查后病理确诊为原发性气管支气管淀粉样变。结论：气管支气管淀粉样变是一种气管和支气管黏膜下异常的淀 粉样物质沉积所导致的罕见病,临床症状多样,常被误诊为其他肺部疾病。支气管镜检查是诊断气管支气管淀粉样 变的重要方法,病理学检查是诊断的金标准,对于不明原因慢性咳嗽患者,建议及早行支气管镜检查。     

气管支气管淀粉样变3例病例报道及文献复习

目的 总结气管支气管淀粉样变的临床表现、影像表现、鉴别诊断及治疗方法。 方法 报道就诊于山东大学附属省立医院经支气管镜活检病理确诊为气管支气管淀粉样变的3例病例,复习相关文献。 结果 气管支气管淀粉样变临床症状及影像学无特异性,临床症状主要为:咳嗽、胸闷、憋气、呼吸困难、咯血等,影像学上可出现管壁钙化或增厚,管腔狭窄,增强扫描可见病灶有强化,其中管壁钙化为较特异性的特征,临床误诊及漏诊常见,局限的气管支气管淀粉样变容易漏诊,病变较弥漫的可伴有肺门淋巴结肿大及钙化、肺部结节状或斑片状高密度影,容易误诊为支气管哮喘、肺结核、慢性支气管炎等疾病。 结论 气管支气管淀粉样变临床少见,症状无特异性,由临床医师对影像学观察及认识不足导致误诊漏诊常见。提高对本病的认识,对其影像学表现及临床特征综合分析,并及时行支气管镜检查,病理学显示淀粉样物质沉积,刚果红染色阳性可明确诊断,有助于减少误诊。     

系统性淀粉样变的呼吸系统表现

目的提高临床对系统性淀粉样变患呼吸系统受累的认识。方法回顾性分析1985年-2003年收治的46例系统性淀粉样变患的呼吸系统表现。结果46例系统性淀粉样变患中，37例具有呼吸系统临床表现，分别累及上呼吸道、气管支气管、肺实质、胸膜和纵隔、肺门淋巴结，12例经活检证实。胸膜病变和肺实质病变包括局限性和弥漫性病变分别占呼吸系统受累的系统性淀粉样变患的62．12％、51．35％和40．54％。结论系统性淀粉样变患呼吸系统受累远高于既往献报道，尤其是胸膜病变，故应提高系统性淀粉样变对呼吸系统受累的认识，早期诊断,早期治疗。     

原发性气管支气管肺淀粉样变并嗜酸粒细胞增多症一例

淀粉样变(amyloidosis)是可发生于全身许多器官、表现各异的临床综合征,其共同点为患者均具有细胞外淀粉样物质沉积于血管壁及组织中的一组疾病。淀粉样变常累及呼吸系统,但原发性气管支气管肺淀粉样变较少见,根据病变部位分为3种类型:气管支气管型、肺结节实变型、肺泡间隔或肺间质型。气管支气管肺淀粉样变合并外周血嗜酸粒细胞增多更为少见,国内尚未见报道,国外也仅有数例报道。现报告1例并结合文献复习如下,旨在提高对气管支气管肺淀粉样变的认识。临床资料患者男性,40岁。因“咳嗽、咳痰3年,气促3个月”,于2006年11月7日入院。患者于3…     

原发性气管支气管淀粉样变临床分析

目的 探讨原发性气管支气管淀粉样变的临床特征、诊疗方法及预后.方法 对本院1例及1989至2012年中文文献报道的50例原发性气管支气管淀粉样变病例进行回顾性总结,分析其基本资料、临床表现、影像学表现、支气管镜下表现及治疗情况等资料.结果 本院患者因“气急”入院,行胸部CT、支气管镜以及肺功能检查,以支气管镜活检获取标本确诊,经环磷酰胺治疗后好转.文献报道的50例患者主要临床表现为咳嗽(96％,48/50)、气急(70％,35/50)、咳痰(66％,33/50)、发热(24％,12/50)及咯血(22％,11/50)等;胸片检查以肺部纹理增粗(45.5％,15/33)最为常见,胸部CT影像学表现以气管壁增厚、管腔狭窄(76.2％,32/42)最为常见;最常见的支气管镜下表现为气管壁增厚、管腔狭窄(40.8％,20/49),其次为黏膜充血、水肿(32.7％,16/49);肺功能检查以阻塞性通气功能障碍为最主要表现.3例伴有并发症,24例误诊为其他肺部疾病.最终47例(94％)患者经支气管镜活检确诊,2例经CT引导下肺穿刺活检确诊,1例经手术后病理确诊.50例患者中42例接受药物、支气管镜下介入、胸部外照射及手术等治疗,病情均好转.结论 原发性气管支气管淀粉样变是一种临床少见病,易出现误诊,确诊需依靠病理学检查,目前尚无特异性治疗方法.     

局限性气管支气管淀粉样变性的CT影像学特征分析

【】 目的 探讨局限性气管支气管淀粉样变性的CT平扫表现特点及其诊断价值,以提高其认识水平。方法 回顾性分析7例经病理证实的局限性气管支气管淀粉样变性患者的临床及CT平扫表现及伴随征象,其中男5例,女2例,年龄平均48岁(32~69岁)。7例均行胸部CT平扫。7例均经病理组织学检查示淀粉样变性。结果 7例气管壁弥漫增厚伴管腔不同程度狭窄,7例气管均无软化,气管粘膜下斑块3例呈环形分布,4例呈不均匀分布,4例中2例以气管前壁分布为著,4例中1例以右侧壁分布为著,4例中1例以前壁及两侧壁分布为著;4例累及两侧主支气管,1例累及左上叶支气管伴管腔狭窄,1例未累及支气管,1例累及两侧主支气管及广泛分支伴管腔不同程度狭窄、扩张;2例可见气管腔内软组织结节;2例伴随肺部并发症,其中1例伴随左下肺不张,另1例伴随两肺弥漫支气管狭窄、扩张。7例均行病理组织学检查示淀粉样变性。结论 局限性气管支气管淀粉样变性CT平扫表现具有一定的特征性,并可发现肺部并发症,对疾病的诊断治疗有一定的指导作用。     

气管支气管淀粉样变MSCT表现及文献复习（附4例病例）

目的：总结气管支气管淀粉样变MSCT征象,提高对该病CT表现的认识,减少误诊。方法报道4例经支气管纤维镜病理确诊为气管支气管淀粉样变的临床及CT图片,并复习相关文献。结果4例病例男女比例为2：2,年龄49-65岁,中位年龄51岁,胸片主要显示气管支气管管腔狭窄,CT横断位及多平面重建工作站重建后可见特征性表现,气管支气管淀粉样变显示病变主要以管壁增厚钙化为主,病变范围长,呈连续性及围管性增厚,管壁钙化以支气管明显,钙化呈块状或长条状,管腔明显狭窄;继发肺部阻塞性炎症3例。结论气管支气管淀粉样变临床比较少见,但在CT图像上有一定的特征表现,抓住其CT特征,可提高诊断的准确性。     

上气道阻塞76例临床分析

目的探讨上气道阻塞的特点,提高对上气道阻塞的认识和早期诊断率。方法回顾性分析2004年1月～2007年4月间在北京协和医院确诊为上气道阻塞的76例患者的病因谱、临床特点、肺功能、纤维支气管镜下表现及病理学特点。结果76例患者中,呼吸道淀粉样变19例(25.0%),复发性多软管炎23例(30.3%),肿瘤25例(32.9%),结核8例(10.5%),甲状腺多发实性结节1例(1.3%)。上气道阻塞的临床表现多种多样,主要症状包括:咳嗽46例(60.5%)、声音嘶哑43例(56.6%)、不同程度的呼吸困难36例(47.4%)和咯痰29例(38.2%)。56例患者进行了肺通气功能检查,其中27例(48.2%)表现为阻塞性通气功能障碍,14例(25.0%)表现为混合性通气功能障碍。70例患者进行了支气管镜或喉镜检查,67例(95.7%)有明显异常发现,其中35例患者(50%)表现为气管、支气管壁增厚和/或狭窄,32例患者(45.7%)表现为气管内结节或新生物。60例进行病理学检查的患者中,符合淀粉样变者16例,复发性多软骨炎5例,结核4例,肿瘤25例,支气管黏膜肉芽肿伴慢性炎症10例。结论对于临床怀疑上气道阻塞的患者应及时行肺功能、纤维支气管镜检查,有助于上气道阻塞的早期诊断与治疗。     

气管支气管损伤的急诊手术治疗

目的:探讨气管支气管损伤的急诊外科治疗,评价急诊手术治疗效果.方法:对我院手术的¨例气管支气管损伤患者的临床资料进行分析.结果:8例病人急诊剖胸行气管支气管修补术,术后并发左下肺不张2例,经气管插管吸痰及正压通气治愈,1例发生右膈下脓肿,经剖腹探查引流治愈.全组无死亡,术后无支气管胸膜瘘及狭窄发生.结论:急诊剖胸气管支气管修补术是治疗气管支气管损伤的重要方法,安全、并发症少.     

Primary tracheobronchial amyloidosis in China: Analysis of 64 cases and a review of literature

Primary tracheobronchial amyloidosis (TBA) is a rare pulmonary disease.A systematic review was performed on 64 cases of primary TBA in China and progress in the diagnosis and treatment of this disease is discussed.The Chinese biological and medical databases from 1970 to 2010 were searched and 75 cases of complete clinical and pathological data were identified.The clinical characteristics of the disease were summarized and longitudinal comparisons were made of diagnostic and treatment methods over time.The results showed that the morbidity associated with primary TBA has increased over recent years.The clinical manifestations were non-specific.Progressive dyspnea, cough and sputum were the most common symptoms.The percentage of patients undergoing computed tomography (CT) scan has increased over the years.The bronchoscopy and transbrochial lung biopsy (TBLB) were usually sufficient to establish the diagnosis.Treatment was reported for a total of 44 cases.Bronchoscopic Nd:YAG laser irradiation, argon plasma coagulation (APC) and drugs administration such as steroids and colchicines were reported to be effective in some patients.It is concluded that the demographic characteristics and clinical manifestations of primary TBA patients in China are largely consistent with findings reported in other countries.Dramatically more cases were reported in recent years, mainly due to the extensive application of bronchoscopy since 1990s.Chest CT scan provides important clues for the diagnosis of the disease.The definite diagnosis was confirmed by bronchoscopic findings and Congo red staining of biopsy specimen.Bronchoscopic Nd:YAG laser irradiation, argon plasma coagulation (APC) and drugs administration, such as steroids and colchicines were reported to be effective in some patients.     

Respiratory manifestations in amyloidosis

Background Amyloidosis is a collection of diseases in which different proteins are deposited. Amyloid deposits occur in systemic and organ-limited forms. In both systemic and localized forms of the disease, lung can he involved. The aim of this study was to explore the different respiratory manifestations of amyloidosis. Methods Chest radiology, clinical presentations, bronchoscopic/laryngoscopic findings and lung function data of 59 patients with amyloidosis involving respiratory tract collected during January 1986 to March 2005, were analysed. Results Of the 16 cases with localized respiratory tract amyloidosis, 8 had the lesions in the trachea and the bronchi, 2 in the larynx and the trachea, 5 in the larynx and/or the pharynx, and 1 in the lung parenchyma. Of 43 systemic amyloidosis with respiratory tract involvement, 3 had the lesions in bronchi, 13 in lung parenehyma, 33 in pleura, 8 in mediastina, 1 in nose and 1 in pharynx. Chest X-rays were normal in most cases of tracheobronchial amyloidosis. CT, unlike chest X-rays, showed irregular luminal narrowing, airway wall thickening with calcifications and soft tissue shadows in airway lumen. Localized lung parenchymal amyloidosis presented as multiple nodules. Multiple nodular opacities, patch shadows and reticular opacities were the main radiological findings in systemic amyloidosis with lung parenchymal involvement. In pleural amyloidosis, pleural effusions and pleural thickening were detected. Mediastinal and/or hilar adenopathy were also a form of lung involvement in systemic amyloidosis. The major bronehoseopie findings of tracheobronchial amyloidosis were narrowing of airway lumen, while nodular, ＇ tumour like＇ or ＇ bubble like＇ masses, with missing or vague cartilaginous rings, were detected in about half of the patients. Conclusions Localized respiratory tract amyloidosis mostly affects the trachea and the bronchi. Chest X-rays are not sensitive to detect these lesions. Systemic amyloidosis often involves lung parenchyma and the pleura. Open lung biopsy or pleural biopsy should be performed for the diagnosis.