11例血友病甲肌肉血肿的处理

我院自1975年1月～1989年6月共诊治血友病甲219例,对其中11例根据肌肉血肿形成时限、部位、血浆Ⅷ:C水平,有否继发感染及血制品供应情况制定处理意见的结果报告如下:临床资料一.一般情况:11例均男性,18～61岁(中数年龄25岁)。出血时间、血小板计数及凝血酶原时间均正常,KPTT 5例延长,凝血时间2例延长,1例有Ⅷ:C抗体存在,血浆Ⅷ:C不同程度减     

高龄胆囊癌肉瘤患者伴发胆囊结石1例报告并文献复习

<正>胆囊癌肉瘤(gallbladder carcinosarcoma)是恶性上皮成分与恶性间叶成分交织混合生长的恶性肿瘤,由Landsteiner[1]于1907年首先报道,其恶性程度高,预后差,临床上极其罕见。本文通过总结我院肝胆外科1例胆囊癌肉瘤的诊治经验,结合相关文献资料,对该病的发生机制、临床表现、影像特征、病理特点、鉴别诊断及预后进行探讨,现报告如下。1病例资料男性患者,82岁,因"反复右上腹疼痛20年,再发1个月"     

自发性椎管内硬膜外血肿保守治疗1例报告及文献复习

正1病例资料自发性椎管内硬膜外血肿(spontaneous spinal epidural hematoma,SSEH)是一种罕见报道的疾病,但随着抗凝药物的使用以及磁共振成像(MRI)应用的普及,该病的发病率及诊断率明显提高。SSEH是指无明显外伤及非医源性因素所导致的血液在椎管内硬脊膜外腔隙内非特异性的聚集~([1])。大多数学者普遍认为及时手术是该病治疗的最佳选择,但逐渐有学者报     

Hoffmann综合征一例并文献复习

患者男，57岁。主因“四肢肌僵硬、乏力2个月”入院。患者于2个月前无明显诱因出现四肢肌肉僵硬，尤以双L臂和大腿明显，并逐渐增粗，伴活动后肌肉酸胀、乏力。2d前自觉吞咽时胸骨后不适，咽下无力。入院体检：T37℃，P82次／min，R20次／min，BP150／90mmHg（1mmHg=0．133kP。），颜面非可凹性水肿，甲状腺Ⅱ度对称弥漫肿大，木触及结节，无压痛。双上臂和大腿外观粗壮，肌肉坚硬，皮肤皱纹减少，无明显雎痛，双手、双足及躯干肌肉未累及，     

韦氏肉芽肿3例报告并文献复习

韦氏肉芽肿３例报告并文献复习徐玉华，史利恒，马兰英，赵向东，晏华琪关键词韦格内肉芽肿病韦氏肉芽肿（Ｗｅｇｅｎｅｒ’ｓｇｒａｎｕｌｏｍａｔｏｓｉｓ，ＷＧ）是一种始于呼吸道的坏死性肉芽肿，伴全身性血管炎，常因肾小球肾炎引起尿毒症死亡，临床罕见。国内迄今报...     

自发性食管壁内血肿1例

病人，女性，48岁，因突然呕血伴吞咽困难1天就诊。1天前吃午饭时，病人感吞咽困难，进食中，突然呕鲜血约l00ml，混有刚吃进的食物，伴胸骨后钝痛。无返酸、烧心，无发热咳嗽，未诊治，在家休息未再呕血，但胸骨后痛无缓解，饮水时有梗阻感，为明原因来我院就诊。病人近期否认进食鱼鸡等过硬食物，亦未进食过热食物。既往无肝炎、肝硬化、食管炎及消化性溃疡病史。皮     

自发性眼眶内血肿1例

患者女,51岁,因右眼球突出伴眼部疼痛9 d于2006年3月30日收入我院眼科。患者9 d前突然出现右眼球突出、眼睑肿胀、眼部疼痛,在当地医院就诊后予抗生素眼水点眼、静滴头孢曲松钠治疗1周后无明显效果,遂转至我院。既往无高血压、血液病病史,否认外伤史,全身检查、血液常规检查均未见异常。     

原发性肝脏T细胞淋巴瘤1例报告并文献复习

原发性肝脏淋巴瘤(primary hepatic lymphoma,PHL)是一种源自肝内淋巴组织的少见恶性肿瘤,迄今报道均为非霍奇金淋巴瘤,国内外文献报道仅100余例,约占结外淋巴瘤的0.14％,占肝脏恶性肿瘤的0.11％[1].国外报道部分PHL患者可合并自身免疫性疾病、慢性肝炎、原发性胆汁性肝硬化、乙型肝炎病毒(hepatitis B virus,HBV)和丙型肝炎病毒(hepatitis C virus,HCV)感染以及获得性免疫缺乏综合征(acquired immune deficiencysyndrome,AIDS)等[2].     

Spontaneous intramural esophageal hematoma: case report and review.

Intramural esophageal hematoma is a rare form of esophageal injury. The presenting symptoms are nonspecific. Esophagogastroscopy and computed tomography scan are usually needed to establish the diagnosis of intramural esophageal hematoma. Presented here is a patient with spontaneous intramural esophageal hematoma who was successfully treated with conservative measures.     

巨气管支气管症三例并文献复习

目的 分析3例巨气管支气管症(tracheobronchomegaly,TBM),即莫-昆综合征(Mounier-Kuhn syndrome)患者的临床特征,以提高对该病的认识,从而有利于早期诊断和治疗并发症,改善预后.方法 回顾性分析北京朝阳医院确诊的3例TBM患者的临床特点、影像学和病理特征,并结合文献报道进行总结.结果 3例患者均为男性,年龄分别为58、65和71岁.从出现症状到确诊的时间,分别为2个月、1年和2年.主要症状为反复咳嗽和咳痰,偶有咯血;疾病后期肺功能受损时出现呼吸困难和呼吸衰竭的相应表现.所有患者均通过X线胸片和胸部CT发现显著的气管、支气管扩张确诊.经抗感染治疗病情均能控制.TBM是一种罕见的、先天性疾病,特征性的改变为气管、主支气管的弹力纤维和平滑肌组织萎缩、纤细,导致气管支气管显著扩张,可合并远端支气管扩张.男性好发,发病年龄多为30～40岁.临床表现无特异性,常见症状为反复咳嗽和咳痰,偶有咯血.影像学特征为气管和主支气管扩张.当疾病进一步进展,可出现呼吸困难或呼吸衰竭.治疗主要是预防和控制感染,原发病缺乏有效的治疗手段.结论 早期诊断并及时控制反复发生的支气管肺部感染,可以阻止TBM患者肺功能的下降,对改善预后至关重要.

Abstract：

Objective To analyze the clinical, radiological, and pathological characteristics of tracheobronchomegaly (TBM, Mounier-Kuhn syndrome ).Methods The clinical, radiological and pathological characteristics of 3 cases of TBM were analyzed, and the literatures were reviewed.Results All 3 patients were men, between the age of 58 -71 years old.From the onset to diagnosis, the shortest time was 2 months, and the longest 43 years.The most usual presentations included recurrent cough and sputum,and occasional haemoptysis.In the advanced stage of the disease, patients would present shortness of breath and the symptoms associated with respiratory failure because of the reduction in pulmonary function.All the diagnoses were confirmed by X-ray and CT of the chest finding that the trachea and the main bronchi dilated markedly.After anti-infection treatment, all patients recovered.Mounier-Kuhn syndrome was a rare congenital abnormality characterized by atrophy or absence of elastic fibers and thinning of smooth muscle layer in the trachea and main bronchi.These airways were thus flaccid and markedly dilated on inspiration and collapsed on expiration.The usual presentation was recurrent respiratory tract infections with a broad spectrum of functional impairment ranging from minimal disease with preservation of lung function to severe disease in the form of bronchiectasis, emphysema and pulmonary fibrosis, ultimately culminating in respiratory failure and death.Computed tomography scan of the chest was used for the diagnosis.Treatment was mainly supportive with chest physiotherapy and antibiotics.Conclusions Mounier-Kunh syndrome should be suspected in patient with recurrent respiratory infections and chronic sputum production.A careful analysis of the central airways at the chest radiograph of these patients is required.     

Gastric intramural hematoma: A case report and literature review

Intramural hematoma of the gastrointestinal tract is an uncommon occurrence, with the majority being localized to the esophagus or duodenum. Hematoma of the gastric wall is very rare, and has been described most commonly in association with coagulopathy, peptic ulcer disease, trauma, and amyloid-associated microaneurysms. A case of massive gastric intramural hematoma, secondary to anticoagulation therapy, and a gastric ulcer that was successfully managed with conservative therapy, is presented. A literature review of previously reported cases of gastric hematoma is also provided.     

Osteopoikilosis: report of 3 cases and review of the literature

Osteopoikilosis, osteopathia condensans disseminata, is a rare hereditary autosomal dominant sclerosing bone dysplasia, more common in males. The diagnosis is usually made incidentally from radiographs which show multiple, small, well-defined, variably shaped and widely distributed (over the skeleton) sclerotic areas. The involvement is symmetrical, and the predilected locations are the phalanges of the hand, carpal bones, metacarpals, foot phalanges, metatarsals, tarsal bones, ilium, femur, radio and sacrum. It must be distinguished from melorheostosis, osteopathia striata and fundamentally from osteoblastic bone metastases, on the basis of the clinical, radiological (roentgenographs, computed tomography and magnetic resonance) and radionuclide scanning characteristics. Histologically, there are focal condensations of compact lamellar bone within the spongiosa. We report three cases of osteopoikilosis and review the literature. Two cases didn't have affectation in phalanges of the hand, which had not been previously reported, to our knowledge.     

Spontaneous pneumopericardium: case report and review of the literature

An 84-year-old man presented with acute dyspnea and chest pain attributed to his long medical history of cardiorespiratory problems. Admission chest roentgenogram revealed a moderate pneumopericardium and a cavitary lesion in the base of the right lung. The lesion was found to be a bronchogenic carcinoma perforating through the upper pericardium to form a bronchopericardial fistula, confirmed by autopsy. A review of the literature concerning the various causes of pneumopericardium, the clinical features and the principles of treatment is included.     

胆囊神经内分泌癌3例诊治分析并文献复习

原发性胆囊神经内分泌癌临床少见,约占全部神经内分泌癌的0.2%[1]。临床表现无特异性,表现出类癌综合征的极少,多以急慢性胆囊炎或胆囊肿物就诊,术前常规检查诊断困难,主要依靠术后病理结合免疫组化明确诊断。本文对本院就诊的3例胆囊神经内分泌癌患者进行分析总结以便该病的临床诊断及治疗经验的积累。     

A pancreatic-induced intramural duodenal hematoma: a case report and literature review

Spontaneous intramural duodenal hematomas are not frequent and generally are related with an anticoagulant treatment. Pancreatic origin has seldom been described and the treatment is always controversial. We report a new case of a pancreatitis-induced intramural duodenal hematoma that was conservatively managed and we review the literature.     

Achalasia combined with esophageal intramural hematoma: case report and literature review

A 62-year-old male patient was admitted to our hospital due to severe chest pain,odynophagia,and hematemesis.Chest computed tomography showed an esophageal submucosal tumor.Esophagogastroduodenoscopy(EGD) revealed a longitudinal purplish bulging tumor of the esophagus.Endoscopic ultrasound(EUS) showed a mixed echoic tumor with partial liquefaction from the submucosal layer.The patient was diagnosed with esophageal intramural hematoma as well as achalasia by upper gastrointestinal endoscopy,esophagography an...