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淋巴管平滑肌瘤病治疗的研究进展

Kartagener Syndrome: Three Cases Report and Literature Review

Objective To improve the knowledge of kartagener syndrome and the understanding of primary ciliary dyskinesia ( PCD) . Methods Three cases of Kartagener syndrome were reported and analyzed on clinical manifestations, symptoms, imaging appearances of chest, and family history. Meanwhile the related literatures were reviewed. Results The three patients deserved special recognition because of recurrent upper and lower respiratory tract infection. Of them, the third case showed mainly the symptoms of nasosinusitis at an early age, and the distinct pulmonary symptoms appeared in his adulthood. Further examination showed that the triad of chronic sinusitis, bronchiectasis, and situs inversus is present in all cases. The parents of the two cases were consanguineous marriaged. Examination of the bronchial mucosal biopsy specimen of the first case under transmission electron microscopy showed cilia with the absence of inner dynein arms. The three cases got improvement after treatment of anti-infection and expectorant. Conclusion For patients with recurring infection in the upper and lower respiratory tract from infancy, the possibility of PCD should be considered no matter whether a situs inversus exists.

Congenital Cystic Adenomatoid Malformation of Lung: A Case Report and Review of the Literature

目的　全面介绍先天性囊性腺瘤样畸形(CCAM)其可能的病因，临床、病理特点及诊断手段，循证探讨针对CCAM的治疗方法及预后。 方法　对我院2011年11月收治的1例罕见的CCAM患者的临床资料进行分析，并对相关文献进行复习。 结果　患者数次误诊后最终诊断为CCAM，予手术治疗后痊愈，随访1年无复发。 结论　 CCAM是一种少见的、非遗传性的、错构瘤样的肺发育异常，为一种良性的肺部畸形，其特点是局部肺终末呼吸性细支气管过度生长。CCAM多通过产前影像学检查、活组织检查或术后病检诊断。手术为治愈该病的最根本、最重要措施。

雷贝拉唑与奥美拉唑治疗幽门螺杆菌阳性消化性溃疡的临床比较

【摘要】 目的 比较雷贝拉唑与奥美拉唑三联疗法治疗幽门螺杆菌（helicobacter pylori，Hp）阳性消化性溃疡的临床疗效。 方法 2009年1-12月将96例Hp阳性的消化性溃疡及慢性胃炎患者，根据治疗方法不同随机分为雷贝拉唑组（52例）和奥美拉唑组（44例）。两组联合的抗菌药均为克拉霉素和阿莫西林，疗程1周。各组在治疗后4～8周分别检测Hp感染情况，并观察溃疡的愈合情况。 结果 雷贝拉唑组Hp根除率及3 d症状缓解率分别为96.2%（50/52）、82.7%（43/52），奥美拉唑组分别为88.6%（39/44）、72.7%（32/44），雷贝拉唑组均明显高于奥美拉唑组（Plt;0.05）。雷贝拉唑组溃疡愈合率为98.1%（51/52），与奥美拉唑组的95.5%（42/44）比较无统计学意义（Pgt;0.05）。 结论 雷贝拉唑联合克拉霉素和阿莫西林是根除Hp比较有效的一种治疗方案。

Combination Therapy in Bleeding Esophageal Varices with Under Gastroscope Injection of Sclerosing Agent and Drug Treatment

目的:观察比较胃镜下硬化剂注射联合药物治疗和单纯硬化剂注射治疗、药物治疗食管静脉曲张出血的疗效。方法:95例肝硬化并食管静脉曲张出血患者,分成三组。32例患者给予硬化剂注射联合药物治疗,31例患者给予硬化剂治疗,32例患者给予药物治疗。观察三组患者急诊止血率,近期出血率、远期再出血率、曲张静脉消失率、曲张静脉复发率。 结果: 联合组、硬化剂组、药物组急诊止血率分别为93.8%.90.3%、68.8%;联合组、硬化剂组、药物组近期出血率分别为6.3%、9.7%、46.9%;联合组、硬化剂组、药物组曲张静脉消失率分别为90.6%、83.9%、1.9%;联合组、硬化组与药物组急诊止血率、近期出血率、曲张静脉消失率有显著差异。联合组、硬化剂组、药物组远期出血率分别为9.4%、59.4%、31.3%; 联合组、硬化剂组、药物组曲张静脉复发率分别为18.8%、45.2%、87.5%;联合组与硬化组、药物组远期出血率、曲张静脉复发率有显著差异。结论:胃镜下硬化剂注射联合药物治疗食管静脉曲张出血可显著提高疗效。

Expression and Significance of PCNA and VEGF in Gastric Cancer

摘要:目的: 探讨PCNA,VEGF在胃癌组织中的表达关系及其意义。 方法 :免疫组织化学法检测正常胃和胃癌组织中PCNA和VEGF的表达,并分析其与胃癌临床病理特征的相关性。 结果 :胃癌组织中PCNA、VEGF的阳性表达率分别为850%、683%,二者在正常胃组织中均为隐性表达。PCNA的表达与肿瘤分期、淋巴结转移有关(〖WTBX〗P lt;005),而与年龄、性别、肿瘤大小、组织分化程度无关(〖WTBX〗P gt;005);PCNA的表达与肿瘤分期、淋巴结转移有关(〖WTBX〗P lt;005),而与年龄、性别、肿瘤大小、组织分化程度无关(〖WTBX〗P gt;005)。PCNA和VEGF在胃癌中的表达呈正相关。 结论 :PCNA和VEGF在胃癌组织中的高表达促进了胃癌的发生发展。Abstract: Objective: To study the significance and relationship of expression of proliferating cell nuclear antigen(PCNA) and vascular endothelial growth factor (VEGF) in gastric cancer. Methods : The expression of VEGF and PCNA in normal gastric tissues and gastric cancer tissues were detected by immunohistochemistry (SP),and clinic pathological correlation with gastric cancer was analyzed. Results : The positive exression rates of PCNA and VEGF in gastric cancer were 850%、683%, respectively, while negative expression was shown in normal gastric tissues. It was found that the expressions of PCNA and VEGF were both in significant relation to TNM stages and lymph node metastasis (P lt;005), but in no relation to age, gender, diameter of tumor and tumor cell differentiation (P gt;005). The expressions of PCNA and VEGF were positively correlated with each other. Conclusion : It was indicated that the highlevel expressions of PCNA and VEGF might corporately accelerate the progression of gastric cancer.

Mounier-Kuhn综合征一例

A Comparative Study of Semirigid Thoracoscopy and Rigid Thoracoscopy in Diagnosis of Unexplained Pleural Effusion

ObjectiveTo compare the accuracy and safety of semirigid thoracoscopy and rigid thoracoscopy in the diagnosis of unexplained pleural effusion. MethodsA prospective randomized study was conducted, in which the patients with unexplained pleural effusion were enrolled consecutively from March 2012 to March 2014 in West China Hospital. The age of the patients ranged from 18 to 75 years. After informed consent, the subjects were randomized to a semirigid thoracoscopy group or a rigid thoracoscopy group to be examined. ResultsForty-nine patients were assigned to the semirigid thoracoscopy group, and 48 patients were assigned to the rigid thoracoscopy group. After thoracoscopy procedure, 44 patients were confirmed as malignant diseases, and 48 patients were confirmed as benign diseases. The overall diagnostic accuracy was 93.9% for the semirigid procedure and 95.8% for the rigid procedure. The diagnostic sensitivity and specificity for malignancy were 96.0% and 100.0% for the semirigid thoracoscopy, and 95.2% and 100.0% for the rigid thoracoscopy, respectively, without statistical significant differences between two groups. All the patients tolerated well with minor complications after two kinds of thoracoscopy. ConclusionsThe diagnostic accuracy of semirigid thoracoscopy and rigid thoracoscopy is comparable in the diagnosis of unexplained pleural diseases. The samples obtained by semirigid thoracoscopy are smaller, but adequate for pathological examination.

左下肺片团影

讨论病案为胸部 CT 平扫显示的肺部片团影像，是呼吸系统常见的影像学改变，临床多考虑感染性疾病、肿瘤或非感染非肿瘤性病变。初诊医师根据临床特征、影像学细微的差异，对患者进行胸部增强 CT 易于发现此病灶为血管结构，并非常见的感染或肿瘤病变。有条件的单位可进行肺血管重建、心室声学造影以及肺动脉造影，就能确诊肺动静脉瘘。临床医师以此为最终诊断是非常普遍的，但专科医师应结合患者受凉感冒后易发生鼻衄且有家族史，全面考虑并深入探究其可能的病因如先天性或遗传性因素的可能，应当熟悉各种罕见病的临床特征，如遗传性出血性毛细血管扩张症。通过家系资料收集、连锁分析，目标基因全外显子测序，最终证实该例患者是遗传性出血性毛细血管扩张症，临床表现出动静脉瘘并有其他部位出血。

淋巴管肌瘤病二例