累及气道的复发性多软骨炎2例并文献复习

目的提高对侵犯气管、支气管的复发性多软骨炎的认识,减少误诊和漏诊率。方法分析我院2例累及气道的复发性多软骨炎的临床资料。结果1例患者因就诊时较晚,最后死于呼吸衰竭,另1例患者较早得到诊断,激素治疗预后较好。结论对伴有气管、支气管受累的复发性多软骨炎及时正确诊断,在疾病早期糖皮质激素治疗能显著改善症状,延缓疾病进展。     

累及气道的复发性多软骨炎的临床特征及其处理

目的提高对侵犯气管、支气管的复发性多软骨炎的临床特征及治疗方法的认识。方法回顾性分析2000年8月至2006年10月收治的13例侵犯气管、支气管的复发性多软骨炎患者的临床资料。结果13例累及气管、支气管的复发性多软骨炎患者中男9例，女4例，年龄30-61岁，平均年龄50岁。疾病早期临床表现以咳嗽、咽喉痛及声嘶多见，疾病后期常表现为胸闷、气促、呼吸困难，重者可表现为下呼吸道反复感染和（或）呼吸衰竭。支气管镜检查早期可见喉、声带水肿，气管、支气管黏膜充血、水肿，软骨环肿胀及管腔狭窄；疾病后期由于气管、支气管软骨破坏而出现气道软骨环消失，管壁软化，呼气时部分气道陷闭。胸部CT检查早期可见气管、支气管壁弥漫性增厚伴管腔狭窄；疾病后期出现管腔重度狭窄，部分患者可有气道壁的钙化。肺功能测定表现为不同程度的阻塞性通气功能障碍。13例患者中有12例患者接受了糖皮质激素和（或）免疫抑制剂治疗，其中6例疾病早期患者症状均有不同程度的改善，其余6例患者症状无改善，在给予气道金属支架置入后（共放置气管、支气管支架12枚）5例患者气道阻塞明显改善，1例无缓解，仍需呼吸机辅助通气。随访1—48个月后有12例存活，1例随访2年后死亡。结论侵犯气管、支气管的复发性多软骨炎患者疾病早期糖皮质激素治疗可显著改善症状，疾病后期出现明显气道阻塞时可行支架置入、气管切开及呼吸机辅助通气治疗，以延长生存期。     

复发性多软骨炎致气管狭窄1例及文献复习

复发性多软骨炎是一种罕见的以软骨组织炎症为特征的自身免疫性疾病,其特点是软骨组织复发性、退化性炎症,表现为全身多处软骨和结缔组织受累.复发性多软骨炎常累及呼吸道,导致气管、支气管软骨的缺失,管壁塌陷,气管狭窄等.最终多因呼吸道感染、呼吸衰竭而死亡,预后极差.由于早期症状隐匿,表现复杂,并且很多医师对该病缺乏认识,临床易导致漏诊、误诊、误治.现将我院收治的1例复发性多软骨炎报道如下.     

复发性多软骨炎19例临床分析

目的：为提高对复发性多软骨炎的认识。方法：报道１９例确诊的复发性多软骨炎的临床特征和诊治。结果：发病年龄２７岁～６８岁，平均５０３岁，其中男性１４例，女性５例。７３７％有呼吸系统受累，６８４％有耳软骨炎，４２％有鼻软骨炎，部分病人有关节、心脏、肾脏、皮肤等受累，３６％合并有其他风湿性疾病或自身免疫病。结论：呼吸系统受累为其主要死亡原因。早期诊断，给予激素和免疫抑制剂治疗是改善预后的关键。     

76例上气道阻塞临床分析

目的探讨上气道阻塞的认识和早期诊断。方法回顾分析2004年1月-2007年4月确诊为上气道阻塞的76例病例的临床及病理学特点。结果本组患者呼吸道淀粉样变19例；复发性多软骨炎23例；肿瘤25例，结核8例；临床表现为咳嗽46例；呼吸困难36例；声嘶43例、略痰29例；肺功能检查56例，其中27例（48．21％）表现为阻塞性通气功能障碍，14例（25％）表现为混合性通气功能障碍；有70例纤维支气管镜或喉镜检查，有明显阳性发现者67例，约占95．71％，其中气管、支气管壁增厚和／或狭窄者35例，占50％；异常气管内结节或新生物32例，占45．71％；76例中60例进行病理学检查：刚果红染色阳性者16例；符合复发性多软骨炎5例；肿瘤25例；支气管黏膜慢性炎10例。讨论肺功能测定、纤支镜检查可用于诊断和评估上气道阻塞。肺功能与纤支镜检查，有助于上气道阻塞的诊断与评估，并可用于病情的随访。     

复发性多软骨炎诊治指南(草案)

复发性多软骨炎(relapsing polychondritis，RP)是一种较少见的炎性破坏性疾病，其特点是软骨组织复发性退化性炎症，表现为耳、鼻、喉、气管、眼、关节、心脏瓣膜等器官及血管等结缔组织受累。复发性多软骨炎的病因及发病机制目前仍不清楚。软骨基质受外伤、炎症、过敏等因素的影响暴露出抗原性，导致机体对软骨局部或有共同基质成分的组织(如巩膜、葡萄膜、玻璃体、视神经内膜及神经束膜、主动脉中层和内层的结缔组织、心瓣膜、心肌肌纤维膜、气管黏膜下基底膜、关节滑膜和肾小球及肾小管基底膜等)的免疫反应。复发性多软骨炎可与类风湿关节炎、系统性血管炎、系统性红斑狼疮以及其他结缔组织病并发。     

2例复发性多软骨炎累及气道的临床护理

临床资料2例复发性多软骨炎(relapsing polychondritis,RP)患者均为中年男性,平均年龄42岁,入院前有咳嗽,咳痰,气促症状(时间从6月至1年不等),在院外行肺功能及CT检查考虑:1慢性阻塞性肺疾病;2气管狭窄原因可能性大[1]。我院纤支镜下检查示:气管、左右主支气管黏膜明显肿胀,呈纵行皱襞样改变,致管腔变窄,软骨环消失(声门、气管膜部未累及)。内镜诊断符合多发性软骨炎表现。2例患者无其他     

复发性多软骨炎六例诊治体会

复发性多软骨炎是一种少见的风湿病 ,主要累及耳、鼻、气管支气管等富含软骨部位和内耳、眼等特殊感官器官 ,部分患者因气管塌陷窒息或感染致死亡。本科自 1992年以来诊治 6例复发性多软骨炎病人 ,现将该 6例病人的临床表现和诊治体会总结如下。1　临床资料诊治 6例病人中 ,5例为女性 ,1例为男性 ,年龄范围在38～ 6 7岁之间。从出现症状到确诊所需时间最短为 2个月 ,最长为 3年。除例 4、例 5、例 6首发症状为耳廓软骨炎症外 ,余 3例分别为持续咳嗽 ,经抗感染治疗无效 ,双眼相继出现角膜溃疡 ,声嘶 (见表 1)。2　病历摘要例 1:患者在持续咳…     

复发性多软骨炎诊断和治疗指南

1 概述 复发性多软骨炎(relapsing polychondritis,RP)是一种软骨组织复发性退化性炎症,表现为耳、鼻、喉、气管、眼、关节、心脏瓣膜等器官及血管等结缔组织受累.     

复发性多软骨炎1例报告并文献复习

正复发性多软骨炎(Relapsing polychondritis,RP)是一种罕见以软骨的复发性炎症为特征,最终造成软骨组织结构的进行性破坏的严重的自身免疫性疾病,该病不仅可累及耳、鼻、喉、气管支气管等软骨,还可累及富含蛋白质结构的组织,如眼睛、心脏、血管等。RP的发现已有一个世纪的时间,但目前病因仍不明确,因临床表现的多样性常常导致误诊。研究显示下呼吸道受累可达20%-50%[1],但病初因     

Tracheobronchial adenoid cystic carcinoma: a clinicopathologic study of 14 cases

STUDY OBJECTIVES: To investigate the clinicopathologic features of primary tracheobronchial adenoid cystic carcinoma (ACC), and to examine kit protein (CD117) expression, and a possible correlation between the histologic grade and Ki-67 positivity. DESIGN: Retrospective 10-year study (from 1992 to 2001). SETTING: Referral center for interventional pulmonology. PATIENTS: Fourteen patients referred to our institution for laser resection of tracheal or endobronchial tumors. INTERVENTIONS: Twelve patients were treated primarily by laser resection via bronchoscopy with subsequent radiation therapy. The remaining two patients had tracheal resection with postoperative radiation and radiation therapy alone. RESULTS: Fourteen patients (5 men and 9 women) had an average age of 44 years at diagnosis (age range, 29 to 57 years). Five of 14 patients were smokers (average smoking history, 28.5 pack-years), 8 were nonsmokers, and the smoking history was unknown in 1 patient. Major symptoms at presentation included cough (57.1%), dyspnea (71.4%), and hoarseness (14.3%). Two patients (14.3%) presented with respiratory failure. Eleven patients (78.6%) had one or more recurrences, and 4 patients (28.6%) had metastases, primarily to the lungs. The average time to either recurrence or metastasis was 4.6 years after the initial diagnosis. Five patients (35.7%), all of whom had recurrences and/or metastasis during the course of their disease, died of their disease (average time, 8.2 years). Thirteen of 13 cases stained for CD117 were positive in the tumor cell cytoplasm with membranous accentuation. Our cases included 8 grade I tumors, 4 grade II tumors, and 2 grade III tumors, which did not correlate with the degree of Ki-67 positivity. CONCLUSIONS: Tracheobronchial ACCs in our study were more common in women and nonsmokers who presented with nonspecific respiratory symptoms and followed a clinical course generally comparable to that of tumors in the salivary glands. CD117 expression was present in all cases tested, and Ki-67 stain results did not correlate with the tumor grade.     

Crohn's disease over 20 years after diagnosis in a referral population

OBJECTIVES: Patients frequently ask questions about the lifetime prognosis of Crohn's disease. The aim of this study was to describe the outcomes of Crohn's disease more than 20 years after diagnosis. METHODS: Data from all patients with Crohn's disease whose diagnosis had been performed before 1st January 1978 were analyzed. All referred patients filled in a medical and health-related quality-of-life questionnaire. RESULTS: Among 273 patients with Crohn's disease diagnosed more than 20 years ago, 141 (52%) patients answered our questionnaire, 45 (16%) patients were alive but did not wish to answer our questionnaire, 51 (19%) could not be traced and 36 (13%) died before July 1998. At the end of follow-up, 25.7 (20.0-59.3) years after diagnosis, 24% had a relapse in the previous 12 months, and 48% and 28% had quiescent disease with and without treatment, respectively. These ratios were not different from those observed three years after Crohn's disease diagnosis. Sixteen patients died within 20 years after CD diagnosis, including 11 from CD-related causes. The risk of death estimated by Kaplan-Meier life-tables analysis was non-significantly higher if death was related to CD. An ileal or colic adenocarcinoma was noted in 6 (3.4%) patients. CONCLUSIONS: Crohn's disease activity does not burn out with time, and roughly one-quarter of the patients had active disease 20 years after diagnosis.     

Localized amyloidosis: a survey of 35 French cases.

Since the prognosis of localized amyloidosis remains unclear, we conducted a survey to define the characteristics and the course of this disease. The charts of 35 patients with either laryngeal (14 patients), tracheobronchial (10 patients), colonic (1 patient), or lower urinary tract amyloidosis (10 patients) were analyzed. The average age at diagnosis was 52.7+/-12 years (range 33-73 years). The amyloid protein type was specified to be amyloid light chain (AL) in 15 cases. All patients had undergone additional biopsies (accessory salivary glands, rectal, fat pad and bone marrow aspirates) to rule out a systemic disease. Symptomatic treatments included endoscopic excision and laser therapy. Colchicine and chemotherapy with prednisone and melphalan were prescribed with limited success. During a mean follow-up period of 6.1+/-5.3 years no patient developed a systemic form of amyloidosis. Six deaths were reported, one related to the disease because of a fatal airway hemorrhage.We suggest that immunolabeling studies should be more routinely performed. There was no risk of developing a systemic disease from local amyloid deposits in our survey. However, local evolution can be life-threatening. Such patients should be referred to specialist centers for further evaluation. Management requires close follow-up to exclude recurrence and to determine the appropriate symptomatic treatment.     

Localized amyloidosis: A survey of 35 French cases

Since the prognosis of localized amyloidosis remains unclear, we conducted a survey to define the characteristics and the course of this disease. The charts of 35 patients with either laryngeal (14 patients), tracheobronchial (10 patients), colonic (1 patient), or lower urinary tract amyloidosis (10 patients) were analyzed. The average age at diagnosis was 52.7 ± 12 years (range 33–73 years). The amyloid protein type was specified to be amyloid light chain (AL) in 15 cases. All patients had undergone additional biopsies (accessory salivary glands, rectal, fat pad and bone marrow aspirates) to rule out a systemic disease. Symptomatic treatments included endoscopic excision and laser therapy. Colchicine and chemotherapy with prednisone and melphalan were prescribed with limited success. During a mean follow-up period of 6.1 ± 5.3 years no patient developed a systemic form of amyloidosis. Six deaths were reported, one related to the disease because of a fatal airway hemorrhage.

We suggest that immunolabeling studies should be more routinely performed. There was no risk of developing a systemic disease from local amyloid deposits in our survey. However, local evolution can be life-threatening. Such patients should be referred to specialist centers for further evaluation. Management requires close follow-up to exclude recurrence and to determine the appropriate symptomatic treatment.     

Prognosis in juvenile rheumatoid arthritis with systemic onset. A follow-up study

Thirty-three patients with systemic onset JRA were followed up for 4 to 24 years (median 10 years). None had positive RF or ANA. Most patients developed polyarthritis. Cardiac involvement occurred in 14 patients (42%). Cardiac prognosis was good for pericarditis but seemed to be worse for myocarditis or perimyocarditis. Three patients contracted renal amyloidosis (9%). Severe growth retardation was observed in 39 per cent. Half of the patients had low activity or were in remission after in average 5.9 years' duration of the disease. Seventy per cent of the patients were in a good functional state. Patients with less than average radiological progression during the first 5 years of disease and those with onset of disease after the age of 5, seemed to have a better functional prognosis.     

Respiratory manifestations of chronic granulomatous disease; a clinical survey of patients from Iranian primary immunodeficiency registry

Chronic granulomatous disease represents a group of inherited disorders of phagocytic system wherein recurrent infections are seen at different sites especially in the respiratory system. To determine the clinical spectrum of respiratory manifestations in chronic granulomatous disease patients, in this retrospective study, we used data from Iranian Primary Immunodeticiency Registry. The diagnosis was based upon WHO criteria for chronic granulomatous disease. We reviewed the records of 38 patients (26 males, 12 females), related to 33 families, 73% of whom were consanguineous. The median age at the time of the study was 12yrs (3 months-22 years). The median onset age of symptoms was 4 months (l month-12 years), and that of diagnostic age was 5 years (l month-20 years), with a diagnostic delay of 4.15 years, on an average. Sixty three percent of our patients had respiratory involvement in the course of their illness, including pneumonia (18 pts, 75%), tuberculosis (llpts, 46%), aspergillosis (3 pts, 12.5%), pulmonary abscess (3 pts, 12.5%), and bronchiectasis (1 pt, 4%). Only 4 of our patients presented with respiratory problems as their first manitestation. Lymph nodes were the first common site and the lungs were the second sites of involvement in chronic granulomatous disease patients; however, it is noteworthy that only in a few of our patients, it was the first manifestation of the disease. Thus special attention should be paid to the pulmonary complications while managing this disease.     

以消化道出血为主要表现的抗中性粒细胞胞质抗体相关性血管炎临床分析

目的 分析以消化道出血为主要表现的抗中性粒细胞胞质抗体(ANCA)相关性血管炎患者的临床特征及预后.方法 查阅我院1987年5月至2007年5月收治的ANCA相关性血管炎合并消化道出血的病例.对患者生存率进行Kaplan-Meier统计分析.结果 ANCA相关性血管炎共170例,其中8例以消化道出血为主要表现,男性和女性各4例;平均年龄(39±20)岁;从疾病出现首发症状到消化道出血平均(8±8)个月;其中韦格纳肉芽肿病合并消化道出血最常见;临床表现最常见为便血(7例,占7/8),远端小肠为最常见受累部位(5例,占5/8);2例患者采用激素冲击治疗,6例采用相当于泼尼松1～2mg·kg-1·d-1激素治疗.1例患者行肠穿孔修补术,2例患者行病变肠段切除术,均是在出现外科情况后手术.3例存活,5例死亡,平均总存活时间为(373±108)d,出现消化道出血后患者平均存活时间为(46±10)d.结论 ANCA相关性血管炎合并消化道出血罕见,预后差.     

Autoantibodies to cartilage and type II collagen in relapsing polychondritis and other rheumatic diseases.

Cartilage antibodies were demonstrated by indirect immunofluorescence (IFL) on human fetal cartilage in 6 out of 9 patients with relapsing polychondritis (RPC), in 4 out of 260 patients with rheumatoid arthritis (RA), and in only 1 out of 1016 patients with other disorders. The antibodies were specific for cartilage and evenly stained the whole cartilage matrix. They were predominantly of IgG class and varied in titres from 1:1 to 1:320. Follow-up studies in the RPC patients indicated that higher titres were present during the early acute phase of the disease. Five of the 6 positive cases had developed the disease within the past 12 months, and the 3 negative cases had had the disease for 3 to 7 years when tested. The RA cases showing positive cartilage IFL had no clinical evidence of RPC. Sequential measurements in 2 of the 4 cases showed that these antibodies became detectable some years after the onset of arthritis. Absorption studies with human type II collagen and purified porcine proteoglycan failed to remove the cartilage IFL. Antibodies to human native type II collagen were measured by an enzyme-linked immunosorbent assay. The highest levels were found in the RA sera which also displayed cartilage IFL, but the 2 tests gave discordant results. RPC sera showed the same antibody levels by this method, as did cartilage-IFL-negative RA sera, though both groups had higher mean levels than health controls. The findings that cartilage antibodies are detected in the majority of cases of RPC and only rarely in other diseases suggests these antibodies may play an important role in the pathogenesis of cartilage destruction in RPC.     

Long term survival with the Bentall button operation in 150 patients

Between May 1980 and May 2000, 150 patients (123 males and 27 females) underwent surgery with the same surgeon for ascending aortic replacement with a valvular conduit and coronary reimplantation with the aid of a collar of aortic wall (button technique). The average age was 50 +/- 16 years. Within this population, 114 patients had isolated annulo-ectasial disease, 36 had Marfan syndrome and 20 had dissection (5 acute and 15 chronic). A carbon fibre valve with 2 leaflets was implanted in 124 patients, a mono-leaflet valve in 20 and 6 others required a heterograft due to their age or a contra-indication to anticoagulation. The associated procedures were: 12 arch replacements, 5 myocardial revascularisations, 4 mitral replacements, 1 tricuspid plasty, 1 inter-atrial communication closure. In 30 patients (20%) there was a cardiovascular surgical re-intervention. The operative and first month mortality amounted to one sudden death on the 19th day, ie 0.6%. Three patients were lost to follow up. The average survival was 7.87 +/- 5.37 years (minimum 1, maximum 20 years). The actuarial survival was 85% at 10 years and 60% at 20 years. These figures are much higher than those reported in our previous statistics from 1994 when the percentage of survivors at 12 years was only 61%. In the group of patients undergoing surgery before 60 years of age, the survival at 14 years was 94% and 81% at 20 years. Only four late re-interventions were attributable to the Bentall procedure, of which 2 were left coronary ostium stenoses. The rate of thrombosis and embolism was 0.42 per 100 patient-years and the rate of haemorrhagic accidents was identical, including minor accidents. This considerable improvement in long-term prognosis is explicable by the adoption of a single operative technique, considered to be the best, with the best myocardial protection thanks to coronary retro-perfusion and cold or hot cardioplegia, and also without doubt with the best medical survival.     

系统性红斑狼疮合并肠假性梗阻12例临床分析

目的 分析系统性红斑狼疮(SLE)合并肠假性梗阻病人的临床表现及实验室检查，提高对SLE合并肠梗阻的认识。方法对12例SLE合并肠梗阻的病人进行回顾性分析，收集整理其临床资料及有关实验室检查，分析其发病情况、临床累及脏器、实验室检查、病程、治疗及预后等特点。结果发病年龄2l～56岁，平均36岁，平均病程31个月。死亡5例。以肠梗阻为首发症状者2例。有肾脏累及者9例，血液系统累及者6例，中枢神经系统累及者2例，循环系统累及者8例。其他：胃肠出血4例，腹水4例。2例以肠梗阻为首发症状者入院前分别误诊为溃疡性结肠炎和肠炎。2例曾行剖腹探查并部分小肠切除术，病理报告均提示肠道血管炎。结论SLE合并肠梗阻患者病情较重，常伴有其他脏器累及，病死率较高。以肠梗阻为首发症状的SLE患者易被误诊。有肠梗阻症状并有其他脏器累及者应排除有无SLE可能。肠道血管炎是引起肠梗阻的主要原因。