BACKGROUND Abernethy malformation,also known as congenital extrahepatic portosystemic shunt,is an uncommon malformation resulting from aberrant development of the portal venous system.Cystic fibrosis(CF)is an autosoma...BACKGROUND Abernethy malformation,also known as congenital extrahepatic portosystemic shunt,is an uncommon malformation resulting from aberrant development of the portal venous system.Cystic fibrosis(CF)is an autosomal recessive genetic disease caused by mutations in the CFTR gene.It mainly affects the exocrine glands of the respiratory,digestive and reproductive systems.It is considered extremely rare in the Asian population.We present a clinical case involving a pediatric patient of Asian descent who was diagnosed with Abernethy malformation and CF.CASE SUMMARY A 12-year-old girl presented with a medical history of recurring respiratory infections and hemoptysis,and chest computed tomography(CT)showed bronchiectasis.Whole exome sequencing was performed for the patient,yielding findings that revealed a compound heterozygous variant of the CFTR gene:c.233_c.234insT/p.Trp79fsTer3(maternal origin);c.2909G>A/p.Gly970Asp(paternal origin).CF was diagnosed.The physician’s attention was drawn to the presence of splenomegaly during disease progression.Abdominal enhanced CT revealed splenomegaly,compression of the left kidney,and multiple tortuous dilated vascular shadows were seen at the splenic hilum,which flowed back into the left renal vein and portal vein,suggesting Abernethy malformation type II.Intraoperatively,the abnormal blood flow was seen to merge into the inferior vena cava through the left renal vein without hepatic processing,and the pathology of liver biopsy showed hypoplastic,dilated or absent portal vein branches,both of which supported the diagnosis of Abernethy malformation type II.This represents the initial documented instance of Abernethy malformation accompanied by a CFTR gene mutation in the existing body of literature.CONCLUSION Coexisting Abernethy malformation and CF are rare.Detailed medical history information,abdominal enhanced CT,venography and genetic testing contribute to diagnosis as well as differential diagnosis.展开更多
BACKGROUND Bedaquiline is among the prioritized drugs recommended by the World Health Organization for the treatment of extensively drug-resistant tuberculosis(XDRTB).Many patients have not achieved better clinical im...BACKGROUND Bedaquiline is among the prioritized drugs recommended by the World Health Organization for the treatment of extensively drug-resistant tuberculosis(XDRTB).Many patients have not achieved better clinical improvement after bedaquiline is stopped at 24 wk.However,there is no recommendation or guideline on bedaquiline administration beyond 24 wk,which is an important consideration when balancing the benefit of prognosis for XDR-TB against the uncertain safety concerning the newer antibiotics.CASE SUMMARY This paper reported 2 patients with XDR-TB(a female of 58 years of age and a female of 18 years of age)who received bedaquiline for 36 wk,as local experience to be shared.The 2 cases had negative cultures after 24 wk of treatment,but lung imaging was still positive.After discussion among experts,the consensus was made to bedaquiline prolongation by another 12 wk.The 36-wk prolonged use of bedaquiline in both cases achieved a favorable response without increasing the risk of cardiac events or new safety signals.CONCLUSION Longer regimen,including 36-wk bedaquiline treatment,might be an option for patients with XDR-TB.More studies are needed to explore the effectiveness and safety of prolonged use of bedaquiline for 36 wk vs standard 24 wk in the treatment of multidrug-resistant/XDR-TB or to investigate further the biomarkers and criteria indicative for extension of bedaquline to facilitate clinical use of thisnovel drug.展开更多
Objective Apatinib is a novel inhibitor of vascular endothelial growth factor receptor-2.The goal of this study was to evaluate overall survival(OS)after a combination of transarterial chemoembolization(TACE)and apati...Objective Apatinib is a novel inhibitor of vascular endothelial growth factor receptor-2.The goal of this study was to evaluate overall survival(OS)after a combination of transarterial chemoembolization(TACE)and apatinib in patients with advanced hepatocellular carcinoma(HCC)and to identify the factors affecting patient survival.Methods Fifty-one patients with advanced HCC who received TACE in combination with apatinib in our hospital from June 2015 to May 2017 were enrolled.The OS and progression-free survival(PFS)were calculated using the Kaplan-Meier method.The log-rank test and Cox regression model were used to determine the factors affecting OS.Results The median OS and PFS of the patients were 15 months and 10 months,respectively.The 1-,2-,and 3-year survival rates were 64.7%,23.5%,and 1.8%,respectively.Univariate survival analysis showed that patients with Child-Pugh A(P=0.006),reduction rate of proper hepatic artery(P=0.016),hand-foot syndrome(P=0.005),secondary hypertension(P=0.050),and without ascites(P=0.010)had a better OS.Multivariate analysis showed that hand-foot syndrome(P=0.014),secondary hypertension(P=0.017),and reduction rate of proper hepatic artery(P=0.025)were independent predictors of better OS.Conclusion TACE combined with apatinib is a promising treatment for advanced HCC.Hand-foot syndrome,secondary hypertension,and the reduction rate of proper hepatic artery were associated with a better OS.展开更多
Background:World Health Organization recommends countries introducing new drug and short treatment regimen for drug resistant tuberculosis(DR-TB)should develop and implement a system for active pharmacovigilance that ...Background:World Health Organization recommends countries introducing new drug and short treatment regimen for drug resistant tuberculosis(DR-TB)should develop and implement a system for active pharmacovigilance that allows for detection,reporting and management of adverse events.The aim of the study is to evaluate the frequency and severity of adverse events(AEs)of bedaquiline-containing regimen in a cohort of Chinese patients with multidrug-resistant(MDR)/extensively drug-resistant(XDR)-TB based on active drug safety monitoring(aDSM)system of New Drug Introduction and Protection Program(NDIP).展开更多
Background:E-learning is a growing phenomenon which provides a unique opportunity to address the challenges in continuing medical education(CME).The China-Gates Foundation Tuberculosis(TB)Control Program implemented o...Background:E-learning is a growing phenomenon which provides a unique opportunity to address the challenges in continuing medical education(CME).The China-Gates Foundation Tuberculosis(TB)Control Program implemented online training forTB health workers in three provinces of China.We aim to evaluate the implementation of E-learning CME programs,analyse the barriers and facilitators during the implementation process,and to provide policy recommendations.展开更多
Background:Given the context of rapid technological cha nge and COIVD-19 pan demies,E-lear ning may provide a unique opportunity for addressing the challenges in traditional face-to-face continuing medical education(C...Background:Given the context of rapid technological cha nge and COIVD-19 pan demies,E-lear ning may provide a unique opportunity for addressing the challenges in traditional face-to-face continuing medical education(CME).However,the effectiveness of E-learning in CME interventions remains unclear.This study aims to evaluate whether E-learning training program can improve TB health personnel's knowledge and behaviour in China.展开更多
文摘BACKGROUND Abernethy malformation,also known as congenital extrahepatic portosystemic shunt,is an uncommon malformation resulting from aberrant development of the portal venous system.Cystic fibrosis(CF)is an autosomal recessive genetic disease caused by mutations in the CFTR gene.It mainly affects the exocrine glands of the respiratory,digestive and reproductive systems.It is considered extremely rare in the Asian population.We present a clinical case involving a pediatric patient of Asian descent who was diagnosed with Abernethy malformation and CF.CASE SUMMARY A 12-year-old girl presented with a medical history of recurring respiratory infections and hemoptysis,and chest computed tomography(CT)showed bronchiectasis.Whole exome sequencing was performed for the patient,yielding findings that revealed a compound heterozygous variant of the CFTR gene:c.233_c.234insT/p.Trp79fsTer3(maternal origin);c.2909G>A/p.Gly970Asp(paternal origin).CF was diagnosed.The physician’s attention was drawn to the presence of splenomegaly during disease progression.Abdominal enhanced CT revealed splenomegaly,compression of the left kidney,and multiple tortuous dilated vascular shadows were seen at the splenic hilum,which flowed back into the left renal vein and portal vein,suggesting Abernethy malformation type II.Intraoperatively,the abnormal blood flow was seen to merge into the inferior vena cava through the left renal vein without hepatic processing,and the pathology of liver biopsy showed hypoplastic,dilated or absent portal vein branches,both of which supported the diagnosis of Abernethy malformation type II.This represents the initial documented instance of Abernethy malformation accompanied by a CFTR gene mutation in the existing body of literature.CONCLUSION Coexisting Abernethy malformation and CF are rare.Detailed medical history information,abdominal enhanced CT,venography and genetic testing contribute to diagnosis as well as differential diagnosis.
文摘BACKGROUND Bedaquiline is among the prioritized drugs recommended by the World Health Organization for the treatment of extensively drug-resistant tuberculosis(XDRTB).Many patients have not achieved better clinical improvement after bedaquiline is stopped at 24 wk.However,there is no recommendation or guideline on bedaquiline administration beyond 24 wk,which is an important consideration when balancing the benefit of prognosis for XDR-TB against the uncertain safety concerning the newer antibiotics.CASE SUMMARY This paper reported 2 patients with XDR-TB(a female of 58 years of age and a female of 18 years of age)who received bedaquiline for 36 wk,as local experience to be shared.The 2 cases had negative cultures after 24 wk of treatment,but lung imaging was still positive.After discussion among experts,the consensus was made to bedaquiline prolongation by another 12 wk.The 36-wk prolonged use of bedaquiline in both cases achieved a favorable response without increasing the risk of cardiac events or new safety signals.CONCLUSION Longer regimen,including 36-wk bedaquiline treatment,might be an option for patients with XDR-TB.More studies are needed to explore the effectiveness and safety of prolonged use of bedaquiline for 36 wk vs standard 24 wk in the treatment of multidrug-resistant/XDR-TB or to investigate further the biomarkers and criteria indicative for extension of bedaquline to facilitate clinical use of thisnovel drug.
基金supported by the National Natural Science Foundation of China(No.81771950,No.81471765 and No.81601578).
文摘Objective Apatinib is a novel inhibitor of vascular endothelial growth factor receptor-2.The goal of this study was to evaluate overall survival(OS)after a combination of transarterial chemoembolization(TACE)and apatinib in patients with advanced hepatocellular carcinoma(HCC)and to identify the factors affecting patient survival.Methods Fifty-one patients with advanced HCC who received TACE in combination with apatinib in our hospital from June 2015 to May 2017 were enrolled.The OS and progression-free survival(PFS)were calculated using the Kaplan-Meier method.The log-rank test and Cox regression model were used to determine the factors affecting OS.Results The median OS and PFS of the patients were 15 months and 10 months,respectively.The 1-,2-,and 3-year survival rates were 64.7%,23.5%,and 1.8%,respectively.Univariate survival analysis showed that patients with Child-Pugh A(P=0.006),reduction rate of proper hepatic artery(P=0.016),hand-foot syndrome(P=0.005),secondary hypertension(P=0.050),and without ascites(P=0.010)had a better OS.Multivariate analysis showed that hand-foot syndrome(P=0.014),secondary hypertension(P=0.017),and reduction rate of proper hepatic artery(P=0.025)were independent predictors of better OS.Conclusion TACE combined with apatinib is a promising treatment for advanced HCC.Hand-foot syndrome,secondary hypertension,and the reduction rate of proper hepatic artery were associated with a better OS.
文摘Background:World Health Organization recommends countries introducing new drug and short treatment regimen for drug resistant tuberculosis(DR-TB)should develop and implement a system for active pharmacovigilance that allows for detection,reporting and management of adverse events.The aim of the study is to evaluate the frequency and severity of adverse events(AEs)of bedaquiline-containing regimen in a cohort of Chinese patients with multidrug-resistant(MDR)/extensively drug-resistant(XDR)-TB based on active drug safety monitoring(aDSM)system of New Drug Introduction and Protection Program(NDIP).
文摘Background:E-learning is a growing phenomenon which provides a unique opportunity to address the challenges in continuing medical education(CME).The China-Gates Foundation Tuberculosis(TB)Control Program implemented online training forTB health workers in three provinces of China.We aim to evaluate the implementation of E-learning CME programs,analyse the barriers and facilitators during the implementation process,and to provide policy recommendations.
文摘Background:Given the context of rapid technological cha nge and COIVD-19 pan demies,E-lear ning may provide a unique opportunity for addressing the challenges in traditional face-to-face continuing medical education(CME).However,the effectiveness of E-learning in CME interventions remains unclear.This study aims to evaluate whether E-learning training program can improve TB health personnel's knowledge and behaviour in China.
