Birt–Hogg–Dubé(BHD) syndrome, a hereditary renal cancer syndrome caused by mutations in the folliculin(FLCN) gene, is characterized by the presence of ibrofolliculomas, pulmonary cysts, spontaneous pneumothorax...Birt–Hogg–Dubé(BHD) syndrome, a hereditary renal cancer syndrome caused by mutations in the folliculin(FLCN) gene, is characterized by the presence of ibrofolliculomas, pulmonary cysts, spontaneous pneumothorax, and renal cell carcinoma(RCC). Few BHD syndrome cases have been reported in Asian countries, and cutaneous presentations are relatively rare in Asian patients. Asian BHD patients may be misdiagnosed due to their atypical manifestations. Here, we report two Chinese BHD patients with novel FLCN mutations(c.946-947 del AG in exon 9 and c.770-772 del CCT in exon 7). Both of them had RCC and spontaneous pneumothorax without ibrofolliculomas. In patients with RCC and pulmonary cysts but without cutaneous lesions, screening for mutations in the FLCN gene should be performed, especially for those with a family history of RCC or pulmonary cysts(pneumothorax).展开更多
在机械产品性能仿真分析过程中会产生大量数据,这些分析结果与数据需要通过仿真报告进行有效管理。提出一种仿真报告自动生成的解决方案,该方案采用对商业智能报表工具BIRT(Business Intelligence and Reporting Tools)进行二次开发,同...在机械产品性能仿真分析过程中会产生大量数据,这些分析结果与数据需要通过仿真报告进行有效管理。提出一种仿真报告自动生成的解决方案,该方案采用对商业智能报表工具BIRT(Business Intelligence and Reporting Tools)进行二次开发,同时与Web应用集成的方法开发出一个仿真报告自动生成系统。首先对商务智能报表工具作了简介,然后重点叙述了仿真报告自动生成系统的设计,包括仿真报告的设计、系统的架构设计以及智能报表工具的二次开发的方法。最后给出了一个系统在静力学分析报告的应用实例,验证了系统的可行性。展开更多
Rationale and Objectives: Cystic lung disease may be accurately diagnosed by imaging interpretation of specialist radiologists, without other information. We hypothesized that with minimal training non-specialists cou...Rationale and Objectives: Cystic lung disease may be accurately diagnosed by imaging interpretation of specialist radiologists, without other information. We hypothesized that with minimal training non-specialists could perform similarly to specialist physicians in the diagnosis of cystic lung disease. Methods: 72 cystic lung disease cases and 25 cystic lung disease mimics were obtained from three sources: 1) a prospective acquired diffuse lung disease registry, 2) a retrospective search of medical records and 3) teaching files. Cases were anonymized, randomized and interpreted by 7 diffuse lung disease specialists and 15 non-specialist radiologists and pulmonologists. Clinical information other than age and sex was not provided. Prior to interpretation, non-specialists viewed a short PDF training document explaining cystic lung disease interpretation. Results: Correct first choice diagnosis of 85%-88% may be achieved by high-performing specialist readers and 71%-80% by non-specialists and lower-performing specialists, with mean accuracies in the diagnosis of LAM (91%, p Conclusion: With specific but limited training, non-specialist physicians can diagnose cystic lung diseases from CT appearance alone with similar accuracy to specialists, correctly identifying approximately 75% of cases.展开更多
基金supported by the National Natural Science Foundation of China(Grant Number:81172418)the Beijing Municipal Natural Science Foundation(Grant Number:7142160)
文摘Birt–Hogg–Dubé(BHD) syndrome, a hereditary renal cancer syndrome caused by mutations in the folliculin(FLCN) gene, is characterized by the presence of ibrofolliculomas, pulmonary cysts, spontaneous pneumothorax, and renal cell carcinoma(RCC). Few BHD syndrome cases have been reported in Asian countries, and cutaneous presentations are relatively rare in Asian patients. Asian BHD patients may be misdiagnosed due to their atypical manifestations. Here, we report two Chinese BHD patients with novel FLCN mutations(c.946-947 del AG in exon 9 and c.770-772 del CCT in exon 7). Both of them had RCC and spontaneous pneumothorax without ibrofolliculomas. In patients with RCC and pulmonary cysts but without cutaneous lesions, screening for mutations in the FLCN gene should be performed, especially for those with a family history of RCC or pulmonary cysts(pneumothorax).
文摘在机械产品性能仿真分析过程中会产生大量数据,这些分析结果与数据需要通过仿真报告进行有效管理。提出一种仿真报告自动生成的解决方案,该方案采用对商业智能报表工具BIRT(Business Intelligence and Reporting Tools)进行二次开发,同时与Web应用集成的方法开发出一个仿真报告自动生成系统。首先对商务智能报表工具作了简介,然后重点叙述了仿真报告自动生成系统的设计,包括仿真报告的设计、系统的架构设计以及智能报表工具的二次开发的方法。最后给出了一个系统在静力学分析报告的应用实例,验证了系统的可行性。
文摘Rationale and Objectives: Cystic lung disease may be accurately diagnosed by imaging interpretation of specialist radiologists, without other information. We hypothesized that with minimal training non-specialists could perform similarly to specialist physicians in the diagnosis of cystic lung disease. Methods: 72 cystic lung disease cases and 25 cystic lung disease mimics were obtained from three sources: 1) a prospective acquired diffuse lung disease registry, 2) a retrospective search of medical records and 3) teaching files. Cases were anonymized, randomized and interpreted by 7 diffuse lung disease specialists and 15 non-specialist radiologists and pulmonologists. Clinical information other than age and sex was not provided. Prior to interpretation, non-specialists viewed a short PDF training document explaining cystic lung disease interpretation. Results: Correct first choice diagnosis of 85%-88% may be achieved by high-performing specialist readers and 71%-80% by non-specialists and lower-performing specialists, with mean accuracies in the diagnosis of LAM (91%, p Conclusion: With specific but limited training, non-specialist physicians can diagnose cystic lung diseases from CT appearance alone with similar accuracy to specialists, correctly identifying approximately 75% of cases.
