韦格纳肉芽肿耳鼻咽喉-头颈部表现及其诊治进展

韦格纳肉芽肿病（Wegener’s granulomatosis,WG）是一种全身系统性疾病,临床上特征性地表现为上、下呼吸道坏死性肉芽肿改变、多发性血管炎,和肾小球肾炎。该病由德国病理学家Friedrich Wegener于1936年首次描述,学者Godman和Chury于1954年明确了WC的上述三大诊断标准。     

躯体形式障碍在耳鼻咽喉-头颈外科的表现(附4例报告)

目的：为强调“生物-心理-社会”医学模式在耳鼻咽喉临床工作中的重要性。方法：报告4例典型的躯体形式障碍（somato-form disorder）的耳鼻咽喉患者的临床表现和治疗,并对其发生原因、经验教训进行讨论。结果：4例患者虽有耳鼻咽喉头颈外科疾病,但均有躯体型精神障碍,其中2例自杀,另2例经适当治疗治愈。结论：在临床实践中应注意患者的心理社会因素在疾病发生过程中的作用,以改进治疗措施。     

前列腺素与耳鼻咽喉—头颈外科疾病

前列腺素具有广泛的生物活性作用,作为炎症介质参与机体的炎症、免疫过程,其不同的组分具有不同的作用。在耳鼻咽喉—头颈外科领域的某些疾病中,前列腺素起着重要的作用。了解前列腺素在这些疾病病理机制中的地位和作用,有利进一步揭示疾病的本质、提出新的治疗设想、获得突破性进展。     

耳鼻咽喉—头颈外科寻访百年

耳鼻咽喉科学作为一个专门学科形成于二十世纪初，是由耳科学和喉科学这两个原来相互独立的领域结合在一起形成的。那时的耳科医生是外科医生，而喉科医生是内科医生，同时负责处理鼻部和胸部疾病。 二十世纪初，人们开始了勇敢的尝试，在原始麻醉技术和无抗生素的条件下开展了精湛的外科手术。两次世界大战促进了科学技术的发展，同时为探索新的和复兴旧的外科技术提供了机会。抗生素的问世，使急性乳突炎和中耳炎的主要并发症显著减少，同时，扁桃体和腺样体切除手术的数量也明显减少，但是，分泌性中耳炎明显增加，在全麻下施行鼓膜切开…     

上海市第一人民医院耳鼻咽喉头颈外科

上海市第一人民医院建于1864年，1946年耳鼻咽喉科独立或科，2000年更名为耳鼻咽喉头颈外科，解披初期由胡懋廉院长兼任科主任，1952年李继孝任科主任后开展喉癌手术，完或了国人气管及支气管的活体测量。填补了该领域的空白并开展硬性内镜手术。进行针麻手术的临床研究获得成功，创办学龄前聋儿听力言语康复训练班，得到了国家残联领导的肯定。     

艾滋病在耳鼻咽喉科的表现（附45例报告）

报告４５例艾滋病（ＡＩＤＳ）病人的耳鼻咽喉科表现，常见的表现是耳带状疱疹、扁桃体炎、无明显原因的咽痛、口腔和咽部多发性溃疡，以及颈淋巴结肿大；血清ＨＩＶ抗体均阳性。并就ＡＩＤＳ的病因、发病机理及诊断进行讨论。     

上海市第一人民医院耳鼻咽喉头颈外科

学科带头人 董频，男1964年出生于山东新泰市．1 984年毕业于山东医学院．1990年获医学博士学位。现为上海交通大学跗属第一人民医院．上海市第一人民医院、上海市红十字会医院耳鼻咽喉头颈外科主任主任医师上海交通大学教授博士生导师头颈肿瘤外科研究室主任，鼾症王作室主任，复旦大学兼职教授博士生导师．南京医科大学兼职教授。担任《山东大学耳鼻喉眼学报》编委会副主任委员，《中华耳鼻咽喉头颈外科杂志》、《中国耳鼻咽喉头颈外科杂志》、《肿痛》《中国眼耳鼻咽喉杂志》，《听力学及言语疾病杂志》编委为上海耳鼻喉学会委员．头颈肿瘤组副组长．上海市卫生高级职称评审耳鼻喉科学组成员。     

上海市第一人民医院耳鼻咽喉头颈外科

学科带头人 董淑，男，1964年出生于山东新泰市，1984年毕业于山东医学院，1990年获医学博士学位。现为上海交通大学附属第一人民医院，上海市第一人是医院、上海市红十字会医院耳鼻咽喉头颈外科主任，主任医师，上海交通大学教授、博士生导师。     

喉Wegener型肉芽肿1例

1 临床资料 患者男,46岁,以“声嘶一年”为主诉入院。患者于一年前无明显诱因出现声嘶,半年后声嘶逐渐加重,遂来我院救治。间接喉镜检查见：右侧室带苍口水肿,表面覆有坏死、溃疡组织,诊断为“喉肿瘤”。建议取病理。但患者未配合治疗。今因声嘶加重,经门诊以“喉肿瘤”收入院。患者无明显喉痛,咽喉部异物感明显,无明显呼吸困难,无吞咽困难,     

Wegener肉芽肿在耳鼻咽喉科的误诊分析

目的：探讨wegener肉芽肿(WG)在耳鼻咽喉科的早期临床表现及疾病的发展,以期提高对该病的认识,做到早期诊断,减少误诊,提高患者生活质量。方法：对首诊于耳鼻咽喉科的32例WG患者的临床资料进行回顾性分析,了解诊治过程中的误诊情况。结果：32例中曾发生耳鼻咽喉误诊的患者有27例,误诊率达84％。最常见误诊疾病为耳鼻咽喉炎症。结论：多数WG患者首诊于耳鼻咽喉科,对以耳鼻咽喉炎症就诊的患者,应详细询问病史,重视对肺部及肾脏情况的检查,对异常结果进行全面分析是减少误诊的关键环节。     

Foudroyanter Verlauf einer therapierefrakt?ren Wegener Granulomatose bei negativen c-ANCA

BACKGROUND: Wegener's Granulomatosis is a rare systemic disease characterized by the triad of necrotizing granulomas of blood vessels, upper respiratory tract, and kidneys. Diagnosis depends on clinical symptoms (ACR-criteria), detection of serum circulating antineutrophil cytoplasmic antibodies (c-ANCA) and bioptical histopathology. CASE-REPORT: A 34-year-old patient presented with intraoral ulcerating, necrotizing inflammations of the palate and the gingiva. Chest radiograph (nodules, infiltrates), urin analysis (red blood cell casts) being inconspicuous Wegener's granulomatosis was diagnosed by histopathology of an intraoral incisional biopsy. Conventional therapy with corticosteroids and cyclophosphamide relapsed while the disease was taking a progressive course (pulmonary infiltrates, necrotizing vasculitides all over the common integument). The patient died of cardiac decompensation three months after diagnosis. c-ANCAs were not present at any time of disease. CONCLUSIONS: 1. Symptoms of the head and neck are manifest in up to 95% of Wegener's granulomatosis. So knowledge of the disease is important for dentists and oral and maxillofacial surgeons in spite of its low prevalence. 2. c-ANCA-titers do not reflect disease activity. Negative c-ANCAs do not exclude Wegener's granulomatosis. 3. Wegener's granulomatosis can take a progressive, therapy resistent course.     

Dilatation tracheoscopy for laryngeal and tracheal stenosis in patients with Wegener’s granulomatosis

Wegener’s granulomatosis (WG) frequently involves the subglottis and trachea and may compromise the upper airway. The objective of this study is to evaluate retrospectively the effect of treatment of subglottic stenosis (SGS) and tracheal stenosis (TS) by dilatation tracheoscopy (DT) in patients with WG. We performed a cohort study on all patients who underwent DT between February 2001 and September 2005 in our institution. From this cohort we identified a total of nine WG patients. In all patients, clinical, serological and histopathological data had been prospectively collected by a standardized protocol from the time point of diagnosis. In the nine patients that were identified with SGS or TS due to WG (eight women and one man), a total of 22 DT’s were performed. Two patients needed a tracheostoma (one temporarily). The mean follow-up after the first DT was 25.4 ± 14.1 months. Two patients did not experience a recurrence of SGS or TS. Six patients required a second DT without recurrence of local disease. The remaining patient underwent 8 DT's in a 4-year period. DT can offer a simple and repeatable solution to SGS and TS due to WG. Seven of the nine patients required more than one dilatation and some patients experience a functional restriction. One patient has a definitive tracheostoma.     

人类免疫缺陷病毒感染及艾滋病在耳鼻咽喉头颈外科的临床表现

目的 提高对人类免疫缺陷病毒感染者及艾滋病患者在耳鼻咽喉头颈外科临床表现的认识,以便能及时诊治并做好必要的防护.方法 分析55例坦桑尼亚首诊于耳鼻咽喉头颈外科的HIV感染者及艾滋病患者的临床表现及其预后.55例患者中男23例,女32例;年龄3～67岁,中位年龄31岁.结果 55例患者中腮腺肿胀17例次(30.9%);鼻腔、口腔、咽部卡波西肉瘤19例次(34.5%);慢性化脓性中耳炎12例次(21.8%);颈部肿块11例次(20.0%);咽部肿物7例次(12.7%);口腔及咽部黏膜糜烂溃疡6例次(10.9%);口腔白色念珠菌感染2例次(3.6%).HIV感染者或艾滋病患者经治疗后耳鼻咽喉科感染状况可缓解,但易反复发作,且逐渐加重.结论 HIV感染者及艾滋病患者可首诊于耳鼻咽喉头颈外科.对耳鼻咽喉科感染等治疗缓解后反复发作者,应除外是否合并HIV感染或是否为艾滋病患者.

Abstract：

Objective To improve the recognition of clinical manifestation of human immunodeficiency virus (HIV) infection and acquired immunodeficiency syndrome (AIDS) in otorhinolaryngology head and neck surgery, and to emphasize the early diagnosis, correct treatment and effective protection. Methods Fifty-five patients with HIV infection and AIDS encountered in the department of otorhinolaryngology head and neck surgery in Tanzania were included in this study. There were 23 males and 32 femals with age ranged from 3 -67 years, medium 31 year. The clinical manifestation and prognosis of the patients were analyzed and summed up. Results Parotid glands swelling was the most commonly seen clinical manifestation in the 55 cases. Among the cases reported here, 17 (30.9%) showed parotid glands swelling, 19(34. 5% ) had Kaposi's sarcoma in nasal cavity or oral cavity or pharynx,12(21.8% ) were accompanied with chronic suppurative otitis media, 11 (20.0%) had masses in the neck,7 ( 12.7% ) had masses in the pharynx, 6 ( 10.9% ) were seen to have ulcer in oral and pharyngeal mucosa,2(3.6% ) showed infection due to candidaalbicans. The infection in most cases could be controlled and symptoms relieved, but recurrence could occur over time and got worse gradually. Conclusions Many patients with HIV infection and AIDS first see a doctor in otorhinolaryngology head and neck surgery.Patients who had the above clinical manifestations in repeated poor results should be observed carefully,early diagnosis and treatment are important to prevent transmission, prolong life and maximize the quality of life of the patients.     

Unspecific parotitis can be the first indication of a developing Wegener’s granulomatosis

In an unusual course of Wegener’s granulomatosis (WG), a 71-year-old woman presented a 3-week history of unilateral painful parotid swelling unresponsive to antiphlogistic and antibiotic treatment. Following lateral parotidectomy with unspecific inflammatory histopathological findings, the patient developed disturbance of wound healing and high recurrent fever. Control chest X-ray showed several pulmonary round lesions. Immunologic testing for antinuclear cytoplasmatic antibodies (ANCA) was positive and CT navigated puncture revealed pronounced necrotic vasculitis of small arteries and veins. Consequently, systemic WG was diagnosed and specific immunosuppressive therapy was started. Long-term follow-up of 6 months showed a decline in the ANCA course, full remission of the presented symptoms and prevention of renal manifestations. Isolated inflammatory parotid enlargement can be the initial symptom of systemic WG and should be considered as differential diagnosis, especially when nondiagnostic histopathological results are obtained. Early testing for c-ANCA supplies valuable information and therefore should be prompted when additional symptoms occur. Once diagnosed as WG, appropriate therapy is able to prevent progression to severe clinical courses.     

Supraglottic stenosis in localized Wegener granulomatosis

We present what we believe is the first reported case of a patient with supraglottic stenosis secondary to Wegener granulomatosis. The diagnosis was unclear initially because the biopsy results were nonspecific, but a finding of an elevated cytoplasmic-pattern antineutrophil cytoplasmic antibody (c-ANCA) level established the diagnosis of localized supraglottic Wegener granulomatosis. Wegener granulomatosis is characterized by necrotizing vasculitis that is localized predominantly to the kidneys and the upper and lower airways. In the airways, subglottic involvement is well documented, but to the best of our knowledge, supraglottic stenosis has not previously been described. Localized forms of Wegener granulomatosis are characterized by limited disease that involves only the upper airway. The diagnosis in localized forms is complex because histology is diagnostic in only 50% of cases, and only 60% of patients have a positive c-ANCA level. We discuss the diagnostic criteria and management strategies for these localized forms.     

Wegener's granulomatosis

Summary The clinical entity now known as Wegener's granulomatosis was first reported in 1936. Since then the disease's histopathology has been clarified, although its etiology remains unknown. Treatment requires immunosuppressant therapy and careful following of affected patients. The present report reviews the historical background for the discovering of Wegener's granulomatosis and current clinical considerations.     

鼻肉芽肿性多血管炎IgG4免疫组织化学观察

目的　观察鼻肉芽肿性多血管炎（granulomatosis with polyangiitis,GPA）的组织病理形态及其IgG4免疫组织化学表达,探讨GPA与IgG4相关性疾病（IgG4 related disease,IgG4-RD）之间的关系。方法　收集首都医科大学附属北京同仁医院病理科2012年1月～2016年12月间病理诊断的12例鼻GPA患者的临床资料,EnVision免疫组化二步法行CD38、CD138、IgG、IgG4染色。结果  9例伴肺部异常改变;8例血清抗中性粒细胞胞浆抗体值升高;组织学评分,3分者8例,2分者1例,1分者3例;免疫组化结果显示8例IgG4+浆细胞计数超过10个/HPF,其中2例IgG4/IgG比值大于40%。结论　IgG4+浆细胞数量增多常见于鼻GPA,因GPA与IgG4-RD在组织形态学及临床特征上有重叠,对二者的鉴别非常重要,GPA的诊断需结合临床表现、影像学、血清学及病理学表现综合判定。