目的分析长QT综合征(LQTS)和尖端扭转性室性心动过速(TdP)的发病原因、临床表现,研究其诊断方法、治疗进展,强调患者临床特点的重要性。方法回顾总结16例已确诊为长QT综合征和尖端扭转性室性心动过速的临床患者,男2例,女14例,年龄9-78岁...目的分析长QT综合征(LQTS)和尖端扭转性室性心动过速(TdP)的发病原因、临床表现,研究其诊断方法、治疗进展,强调患者临床特点的重要性。方法回顾总结16例已确诊为长QT综合征和尖端扭转性室性心动过速的临床患者,男2例,女14例,年龄9-78岁,平均年龄(39±22．7)岁,最早发病年龄为3岁。对每例患者均进行详细的临床资料及心电图资料收集。结果①一般临床情况:10例诊断为原发性LQTS,6例诊断为继发性LQTS。4例有明确家族史,4例情绪激动或活动后诱发、2例劳累后诱发、2例休息或睡眠时发作,12例有晕厥、4例有黑朦症状。②心电图表现:QT时间>0．48 s 11例, QT时间0．46-0．47 s 3例,QT时间≤0．45 s 2例。③治疗情况:10例原发性LQTS患者均接受β-阻滞剂治疗、部分接受苯妥英钠和美西律治疗,3例植入起搏器。6例继发性LQTS患者中,3例因房室传导阻滞而行起搏器植入术,其余注意治疗原发病、纠正病因(如补钾等)。所有患者经治疗后未再出现TdP复发。结论LQTS主要是由于编码心肌细胞离子通道蛋白的基因突变引起钠、钾离子通道功能障碍所致。折返和早期后除极(EAD)两种机制在TdP发病中均起作用。LQTS诊断主要依靠家族史,不明原因的晕厥和心电图QTc延长,其中更重要的是心电图和家系调查．已有很有效的治疗措施,如β-阻滞剂、LCSD、心脏起搏、ICD治疗等,如果漏诊或误诊而未接受正确治疗则可能发生致命后果。展开更多
Patients with chronic headache associated with ocular symptoms regularly seek ophthalmologists’ opinions. We report an unusual case of chronic paroxysmal hemicrania (CPH), a rare but well-described variant of cluster...Patients with chronic headache associated with ocular symptoms regularly seek ophthalmologists’ opinions. We report an unusual case of chronic paroxysmal hemicrania (CPH), a rare but well-described variant of cluster headache in a female presenting to an eye department with recurrent episodes of severe unilateral periorbital swelling with a chronic history of headaches. Clinical features, review of literature, and therapeutic response to indomethacin helped to establish the diagnosis as CPH. This is the first report of severe orbital inflammation with CPH to our knowledge.展开更多
AIM:To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei(PMP) in Chinese patients.METHODS:The clinicopathologic features and followup data of 92 patients with PMP were...AIM:To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei(PMP) in Chinese patients.METHODS:The clinicopathologic features and followup data of 92 patients with PMP were reviewed and retrospectively analyzed.The cases were categorized into three groups:disseminated peritoneal adenomucinosis(DPAM),peritoneal mucinous carcinomatosis(PMCA),and peritoneal mucinous carcinomatosis with intermediate or discordant features(PMCA-I/D).The log-rank test was used to analyze survival for each group and various clinicopathological parameters.Multivariate Cox proportional-hazard models were constructed to determine the important factors associated with survival.RESULTS:The median age at diagnosis was 51.9 years(range:22-76 years).The median follow up was 124 mo.The 3-,5-and 10-year survival rates were 74.0%,67.4% and 49.1%,respectively.There were 49(53.2%)patients with DPAM,26(28.3%) with PMCA-I and 17(18.5%) with PMCA.Patients with DPAM,PMCA-I/D and PMCA exhibited statistically significant difference in survival(P = 0.001).The 3 year survival for DPAM,PMCAI/D and PMCA was 97.0%,80.0% and 67.0%,respectively;the 5 year survival was 80.0%,67.0% and 50.0%,respectively;and the 10 year survival was 65.0%,28.0% and 14.0%,respectively.Survival rate was significantly lowest in patients < 40 age years of age(P = 0.011).Appendiceal tumor and extra-ovarian parenchymal organ involvement were significantly related to overall survival.Patients with appendiceal mucinous adenocarcinoma(MACA) showed the significantly poorer prognosis(P = 0.011).Multivariate analysis showed that pathological classification,age,appendiceal tumor were significant related to overall survival.CONCLUSION:The clinical process "PMP" should be pathologically classified into DPAM,PMCA and PMCA-I/D.Pathological classification,age,appendiceal MACA are survival independent predictors in Chinese patients with PMP.展开更多
Objective To evaluate the use of titanium mesh reconstruction after sternal tumor resection. Methods From January 2007 to January 2011, 14 patients with sternal tumors were admitted into Peking Union Medical Hospital....Objective To evaluate the use of titanium mesh reconstruction after sternal tumor resection. Methods From January 2007 to January 2011, 14 patients with sternal tumors were admitted into Peking Union Medical Hospital. The clinical characteristics, surgical resection, and technique of reconstruction were reviewed. Results Of the 14 patients, 3 had a metastatic sternal tumor, the primary sites of which were as follows: hepatic carcinoma in one case (metastasis 19 years after operation), breast carcinoma in another case (metastasis 5 years after operation), and renal carcinoma in the other case (found simultaneously). Two patients showed local involvement of the sternum: 1 had thymic carcinoma, and the other had myofibrosarcoma. The remaining 9 patients had primary tumors: 4 were osteochondroma, 3 chondrosarcoma, 1 eosinophilic granuloma, 1 non-Hodgekin's lymphoma. En bloc resection of the sternal tumor was performed in all the 14 patients. The defect was repaired with the titanium mesh adjusted to the shape of the defect and fixed with the stainless steel wire. Eleven patients were followed up for a period from 2 months to 4 years, during which no translocation or broken of the titanium mesh was observed. Conclusions Radical en bloc excision remains the treatment of choice for sternal tumors. Sternum defect reconstruction using titanium mesh as a rigid replacement proves appropriate and effective.展开更多
Objective:The aim of our study was to investigate the prognosis of obese breast cancer patients.Methods:This study was conducted on a total of 317 breast cancer patients who were histopathologically and clinically dia...Objective:The aim of our study was to investigate the prognosis of obese breast cancer patients.Methods:This study was conducted on a total of 317 breast cancer patients who were histopathologically and clinically diagnosed at the General Hospital of Shenyang Military Region(China)from 2004 to 2006.Clinical data including height,weight,age at diagnosis,tumor size,lymph node status,menopausal status,family history of cancer and hormone receptor status were collected.Log-rank test was performed to compare the disease free survival(DFS)and overall survival(OS).Cox proportional hazards regression analysis was conducted to make multivariate analysis.The Chi square test was used to compare the clinical features among normal weight group,overweight group,and obese group.Results:Obesity was an independent prognostic factor for DFS(P=0.022)and OS(P=0.032)in breast cancer patients.In the stratified analysis based on the hormone receptor status,obesity was independently associated with OS in patients with negative ER/PR(P=0.002),but such association was not observed in patients with positive hormone receptors.Obesity was also associated with lymph node status(P=0.001)and smoking(P=0.009).Conclusion:Obesity is associated with poor DFS and OS in patients with breast cancer.Therefore,maintaining normal weight may benefit breast cancer patients.展开更多
文摘目的分析长QT综合征(LQTS)和尖端扭转性室性心动过速(TdP)的发病原因、临床表现,研究其诊断方法、治疗进展,强调患者临床特点的重要性。方法回顾总结16例已确诊为长QT综合征和尖端扭转性室性心动过速的临床患者,男2例,女14例,年龄9-78岁,平均年龄(39±22．7)岁,最早发病年龄为3岁。对每例患者均进行详细的临床资料及心电图资料收集。结果①一般临床情况:10例诊断为原发性LQTS,6例诊断为继发性LQTS。4例有明确家族史,4例情绪激动或活动后诱发、2例劳累后诱发、2例休息或睡眠时发作,12例有晕厥、4例有黑朦症状。②心电图表现:QT时间>0．48 s 11例, QT时间0．46-0．47 s 3例,QT时间≤0．45 s 2例。③治疗情况:10例原发性LQTS患者均接受β-阻滞剂治疗、部分接受苯妥英钠和美西律治疗,3例植入起搏器。6例继发性LQTS患者中,3例因房室传导阻滞而行起搏器植入术,其余注意治疗原发病、纠正病因(如补钾等)。所有患者经治疗后未再出现TdP复发。结论LQTS主要是由于编码心肌细胞离子通道蛋白的基因突变引起钠、钾离子通道功能障碍所致。折返和早期后除极(EAD)两种机制在TdP发病中均起作用。LQTS诊断主要依靠家族史,不明原因的晕厥和心电图QTc延长,其中更重要的是心电图和家系调查．已有很有效的治疗措施,如β-阻滞剂、LCSD、心脏起搏、ICD治疗等,如果漏诊或误诊而未接受正确治疗则可能发生致命后果。
文摘Patients with chronic headache associated with ocular symptoms regularly seek ophthalmologists’ opinions. We report an unusual case of chronic paroxysmal hemicrania (CPH), a rare but well-described variant of cluster headache in a female presenting to an eye department with recurrent episodes of severe unilateral periorbital swelling with a chronic history of headaches. Clinical features, review of literature, and therapeutic response to indomethacin helped to establish the diagnosis as CPH. This is the first report of severe orbital inflammation with CPH to our knowledge.
文摘AIM:To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei(PMP) in Chinese patients.METHODS:The clinicopathologic features and followup data of 92 patients with PMP were reviewed and retrospectively analyzed.The cases were categorized into three groups:disseminated peritoneal adenomucinosis(DPAM),peritoneal mucinous carcinomatosis(PMCA),and peritoneal mucinous carcinomatosis with intermediate or discordant features(PMCA-I/D).The log-rank test was used to analyze survival for each group and various clinicopathological parameters.Multivariate Cox proportional-hazard models were constructed to determine the important factors associated with survival.RESULTS:The median age at diagnosis was 51.9 years(range:22-76 years).The median follow up was 124 mo.The 3-,5-and 10-year survival rates were 74.0%,67.4% and 49.1%,respectively.There were 49(53.2%)patients with DPAM,26(28.3%) with PMCA-I and 17(18.5%) with PMCA.Patients with DPAM,PMCA-I/D and PMCA exhibited statistically significant difference in survival(P = 0.001).The 3 year survival for DPAM,PMCAI/D and PMCA was 97.0%,80.0% and 67.0%,respectively;the 5 year survival was 80.0%,67.0% and 50.0%,respectively;and the 10 year survival was 65.0%,28.0% and 14.0%,respectively.Survival rate was significantly lowest in patients < 40 age years of age(P = 0.011).Appendiceal tumor and extra-ovarian parenchymal organ involvement were significantly related to overall survival.Patients with appendiceal mucinous adenocarcinoma(MACA) showed the significantly poorer prognosis(P = 0.011).Multivariate analysis showed that pathological classification,age,appendiceal tumor were significant related to overall survival.CONCLUSION:The clinical process "PMP" should be pathologically classified into DPAM,PMCA and PMCA-I/D.Pathological classification,age,appendiceal MACA are survival independent predictors in Chinese patients with PMP.
文摘Objective To evaluate the use of titanium mesh reconstruction after sternal tumor resection. Methods From January 2007 to January 2011, 14 patients with sternal tumors were admitted into Peking Union Medical Hospital. The clinical characteristics, surgical resection, and technique of reconstruction were reviewed. Results Of the 14 patients, 3 had a metastatic sternal tumor, the primary sites of which were as follows: hepatic carcinoma in one case (metastasis 19 years after operation), breast carcinoma in another case (metastasis 5 years after operation), and renal carcinoma in the other case (found simultaneously). Two patients showed local involvement of the sternum: 1 had thymic carcinoma, and the other had myofibrosarcoma. The remaining 9 patients had primary tumors: 4 were osteochondroma, 3 chondrosarcoma, 1 eosinophilic granuloma, 1 non-Hodgekin's lymphoma. En bloc resection of the sternal tumor was performed in all the 14 patients. The defect was repaired with the titanium mesh adjusted to the shape of the defect and fixed with the stainless steel wire. Eleven patients were followed up for a period from 2 months to 4 years, during which no translocation or broken of the titanium mesh was observed. Conclusions Radical en bloc excision remains the treatment of choice for sternal tumors. Sternum defect reconstruction using titanium mesh as a rigid replacement proves appropriate and effective.
基金Supported by a grant from the Ministry of Science and Technology of China(No.2012zx09303016_002)
文摘Objective:The aim of our study was to investigate the prognosis of obese breast cancer patients.Methods:This study was conducted on a total of 317 breast cancer patients who were histopathologically and clinically diagnosed at the General Hospital of Shenyang Military Region(China)from 2004 to 2006.Clinical data including height,weight,age at diagnosis,tumor size,lymph node status,menopausal status,family history of cancer and hormone receptor status were collected.Log-rank test was performed to compare the disease free survival(DFS)and overall survival(OS).Cox proportional hazards regression analysis was conducted to make multivariate analysis.The Chi square test was used to compare the clinical features among normal weight group,overweight group,and obese group.Results:Obesity was an independent prognostic factor for DFS(P=0.022)and OS(P=0.032)in breast cancer patients.In the stratified analysis based on the hormone receptor status,obesity was independently associated with OS in patients with negative ER/PR(P=0.002),but such association was not observed in patients with positive hormone receptors.Obesity was also associated with lymph node status(P=0.001)and smoking(P=0.009).Conclusion:Obesity is associated with poor DFS and OS in patients with breast cancer.Therefore,maintaining normal weight may benefit breast cancer patients.
