Abstract： Marfan syndrome （MFS） is a congenital and heritable autosomal dominant disorder of the connective tissue which is often passed down through families. Its clinical presentation typically involves the skeletal， cardiovascular and ocular systems with a high natural mortality. Aortic root aneurysm and consecutive acute aortic dissection represent the main cardiovascular manifestations and main causes of morbidity and mortality in MFS. At present， the predominant therapeutic method is surgery， but surgical outcomes are quite unsatisfactory. Recent studies demonstrate that losartan， a common antihypertensive agent， is useful to treat MFS， the mechanism of which may results from inhibiting overactivation of transforming growth factor β （TGF-β） signaling. This discovery will definitely promote the transition of traditional surgical treatment of MFS into pharmacotherapy. In this review， we focus on the molecular biological pathogenesis， traditional and new therapeutic strategies for MFS patients.
Abstract： Objective To investigate diagnosis and treatment of concealed intrathoracic anastomotic leak of the esophagus. Methods We retrospectively analyzed the clinical data of 32 patients who presented with unexplained sepsis （temperature＞38 ℃ and elevated white blood cell count） after esophagectomy and intrathoracic anastomosis for esophageal carcinoma or gastric cardia carcinoma in Affiliated Hospital, Medical College of Qingdao University from January 2006 to December 2010. All the patients underwent oral water-soluble contrast esophagogram and oral water-soluble contrast computerized tomography of the chest. None of the patients had any sign of contrast leak in these diagnostic examinations, but their chest computerized tomography all showed peri-anastomotic bubble and encapsulated effusion. Fifteen patients were treated as concealed intrathoracic anastomotic leak of the esophagus, including fasting, broad spectrum antibiotic treatment, prolonged gastrointestinal decompression and enteral nutrition via naso-intestinal feeding tube. The other 17 patients were not treated as anastomotic leak of the esophagus and only received broad spectrum antibiotic treatment. Results None of the 15 patients who were treated as concealed intrathoracic anastomotic leak finally developed anastomotic leak proved by oral water-soluble contrast esophagogram and computerized tomography of the chest （0%, 0/15）. Among the 17 patients who were not treated as anastomotic leak, fourteen patients developed anastomotic leak later （82.4%, 14/17）, 2 patients died of aorto-esophageal fistula and 3 patients died of multiple organ dysfunction syndrome. Conclusion Peri-anastomotic bubble and irregular encapsulated effusion in oral water-soluble contrast esophagogram and computerized tomography of the chest should be considered as specific signs of concealed intrathoracic anastomotic leak of esophagus after esophagectomy and intrathoracic anastomosis. Patients with such signs should be treated as anastomotic leak.
目的 总结我科2002年3月至2005年10月收治的下肢深静脉血栓形成(deep vein thrombosis,DVT)患者的诊治经验.方法 本组下肢DVT患者275例,患肢294条.男134例,女141例,年龄14～92岁,平均(50.76±16.43)岁.单侧下肢DVT 256例(左侧159例,右侧97例),19例为双下肢DVT(其中有8例是仅一侧肢体有临床表现); 属混合型194条,中央型74条,周围型26条.行彩色多普勒或静脉造影检查了解血栓部位及范围; 对怀疑有肺动脉栓塞(pulmonary embolism, PE)者行肺部增强CT扫描.治疗包括抗凝、溶栓、祛聚、梯度压力静脉弹力袜以及对症处理.对反复发生PE、存在抗凝治疗禁忌证、需要手术取栓、下肢静脉存在悬浮游离血栓者安置下腔静脉滤器.对发生股青肿患者行手术取栓.疗效评价: 住院期间观察症状变化,监测膝关节上、下15 cm处周径; 对随访患者行CEAP(clinical-etiology-anatomic-pathophysiologic)临床表现分级、功能障碍评分(venous disability score, VDS).结果 本组DVT常见的危险因素包括活动受限史、2周以内有手术史、恶性肿瘤及外伤史.本组患肢主要临床表现包括: 肿胀、疼痛、皮温增高、皮肤暗红、浅静脉迂曲等.并发症: PE 9例,股青肿2例,下腔静脉阻塞综合征3例.275例中2例行手术取栓,25例安置下腔静脉滤器.本组患者症状经治疗后1～5 d开始缓解,出院时膝关节上、下周径较入院时明显缩小(P＜0.05).随访时间6个月～3年,195例(70.9%)的201条(68.4%)患肢获得随访,患肢CEAP分级: C0级78条,C1级53条,C2级16条,C3级20条,C4级15条,C5级11条,C6级8条; VDS评分: 0分77例,1分66例,2分33例,3分19例.结论 下肢DVT的诊断中应注意对危险因素以及无症状的DVT的搜寻; 早期治疗以非手术治疗为主,通过抗凝、溶栓等综合治疗可以达到满意效果; 在早期如发生股青肿应及时手术治疗; 有选择地安置下腔静脉滤器可防止PE的发生; 后期应根据病情特点确定治疗方案和疗程.
Objective To investigate pathogenesis and therapeutic prospect of abdominal aortic aneurysm (AAA). Methods Relevant literatures about pathogenesis and ways of treatment for AAA in recent years were reviewed. Results The formation of AAA are associated with heredity, anatomy, environment and biochemistry and other factors. All factors influence and interact with each other. The metabolic disequilibrium of aortic intermediate extracellular matrix plays an important role in the pathogenesis of AAA. The main reasons for the formation of AAA may be the increase of activity of matrix metalloproteinases and the disequilibrium of genetic expressions of elastin and collagen. The therapy of AAA includes surgical and medical treatment. The methods of medical treatment are still in the process of exploration and research. Conclusion The formation of AAA is a synergistical result of multiple factors, and medical treatment is an important supplement of surgical treatment.
Objective To summarize our treatment experience for patients with chest injuries in “4•20” Lushan earthquake. Methods Medical records of 17 patients with chest injuries after 2013 Lushan earthquake who were admittedto the Department of Thoracic Surgery，West China Hospital were analyzed retrospectively. The diagnosis of chest injuries was mainly confirmed by medical history，physical examination，X-ray and CT scan of the chest. Clinical characteristics and treatment outcomes were analyzed. Among the 17 patients，there were 14 men and 3 women with their age of 57.3±16.1 years. Results There were 12 patients （70.6%）with crash injury of heavy objects，4 patients （23.5%） with fall injury and 1 patient with road traffic injury. Chest injuries were skin and soft tissue contusion in 17 patients （100%），rib fracturein 15 patients （88.2%） including 1 patient with abnormal respiratory movements，pulmonary contusion in 15 patients （88.2%），hemopneumothorax in 11 patients （64.7%），sternal fracture in 1 patient （5.9%） and bilateral pneumothorax with widespread subcutaneous emphysema in 1 patient （5.9%）. Thirteen patients （76.5%） had concomitant brain，abdominal，orthopedic or nerve injuries. One patient underwent left thoracotomy，clot removal and internal fixation of rib fractures for left coagulated hemothorax and left lower lobe atelectasis. All the 17 patients received timely and effective treatment and there was noin-hospital mortality. Conclusions Mechanisms of earthquake injuries are often complex，and patients often have multipleinjuries. The main types of chest injury are rib fractures and pulmonary contusion. Tube thoracostomy is a simple andeffective treatment strategy for them. Satisfactory pain management and bronchoscopy procedure can effectively help patientswith removal of respiratory secretions and maintenance of airway patency.
Objective?To investigate clinical characteristics， diagnosis， and treatment for patients with spontaneous esophageal rupture， and improve clinical diagnostic and treatment level.?Methods?We retrospectively analyzed the clinical data of 34 patients with spontaneous esophageal rupture who were treated in Subei People’s Hospital from January 1996 to June 2010. There were 28 male patients and 6 female patients with their age ranging from 32 to 80 years old (mean 57.6 years old). Main clinical manifestations included severe chest and abdominal pain after vomiting， fever， dyspnea and shock. The duration between disease onset and establishing diagnosis ranged from 4 hours to 7 days. Thirteen patients received conservative treatment including chest drainage， retrograde gastrointestinal decompression and enteral nutrition through jejunostomy. Twenty one patients received surgical treatment including layered anastomosis of the ruptured esophagus， retrograde gastrointestinal decompression and enteral nutrition through jejunostomy.?Results?All the patients were cured without in-hospital death. The mean hospital stay of the 13 patients who received conservative treatment was 46 days， while that of the 21 patients who received surgical treatment was 17 days. All the ruptured esophagus were one-stage healed. All the 34 patients were followed up from l to 8 years， including 11 patients in the conservative treatment group and 19 patients in the surgical treatment group， but 4 patients was lost during follow-up. All the patients had a normal diet without symptoms of esophageal stricture， reflux esophagitis or chronic thoracic empyema.?Conclusion Spontaneous esophageal rupture is a thoracic emergency with a high misdiagnosis rate and mortality．Early diagnosis， early surgical repair of ruptured esophagus and satisfactory chest drainage play a vital role in the treatment for patients with spontaneous rupture of esophagus.