Congenital dysfibrinogenemia (CD) is a qualitative congenital fibrinogen (Fbg) disorder characterized by normal antigen levels of dysfunctional Fbg. A 41-year-old Japanese woman visited the emergent room of our hospit...Congenital dysfibrinogenemia (CD) is a qualitative congenital fibrinogen (Fbg) disorder characterized by normal antigen levels of dysfunctional Fbg. A 41-year-old Japanese woman visited the emergent room of our hospital due to acute and severe abdominal pain. Catheterization of the full bladder released her abdominal pain. Magnetic resonance imaging showed a huge pelvic mass, suggesting an intra-mural giant myoma. Before the removal operation of myoma, screening tests showed no abnormalities, including prothrombin time and activated partial thromboplastin time. However, Fbg level was not determined. The patient wanted to receive early surgical treatment, and an abdominal hysterectomy was performed as usual and the intra-operative blood loss was 100 g (ml). However, we found subcutaneous and pelvic hematomas, although active bleeding was not recognized on an emergent computed tomography examination. At that time, we noticed a low level of plasma Fbg (47 mg/dl). We performed a re-laparotomy to remove hematomas. All ligated blood vessels were re-ligated, and oozing points were vaporized. Around the re-operation, six units of fresh frozen plasma and twelve units of red blood cell suspension were transfused. The clinical course after the 2<sup>nd</sup> operation was uneventful except for the low level of Fbg. An additional study showed that the value of the Fbg activity and antigen was dissociated, and the patient was diagnosed CD with <span style="white-space:nowrap;">γ</span>275 Arg to His (CGC to CAC) mutation.展开更多
Background/aim:This study was aimed at evaluating 5-year effectiveness and safety in participants after XIENCE PRIME Everolimus Elution Coronary Stent System(EECSS)implantation.Materials and methods:From December 2013...Background/aim:This study was aimed at evaluating 5-year effectiveness and safety in participants after XIENCE PRIME Everolimus Elution Coronary Stent System(EECSS)implantation.Materials and methods:From December 2013 to May 2014,108 patients(127 lesions)were treated with the XIENCE PRIME EECSS.The entire follow-up included annual assessments for 5 years after treatment or until one of the clinical endpoints was reached.We evaluated the 5-year clinical outcomes with Kaplan-Meier analysis and the Cox regression model.Results:Nearly three-quarters of the participants were men(76.8%),and the average age was 65.6±10.8 years.Bifurcation lesions accounted for 96.1%(122 lesions),and left main lesions accounted for 3.9%(five lesions),with a total count of 127 lesions.The cumulative rate of major adverse cardiac events was as follows:1 year,1.9%;2 year,4.0%;and 5 year,10.0%.No definite or probable stent thrombosis was observed,and the rate of target lesion failure was only 3.3%over 5 years.The cumulative rate of major bleeding eventually increased to only 4.4%.Conclusions:The 5-year clinical outcomes were favorable in patients treated with XIENCE PRIME EECSS,and the incidence of stent thrombosis and target lesion failure was relatively low.The incidence of major bleeding gradually increased but remained moderate.展开更多
文摘Congenital dysfibrinogenemia (CD) is a qualitative congenital fibrinogen (Fbg) disorder characterized by normal antigen levels of dysfunctional Fbg. A 41-year-old Japanese woman visited the emergent room of our hospital due to acute and severe abdominal pain. Catheterization of the full bladder released her abdominal pain. Magnetic resonance imaging showed a huge pelvic mass, suggesting an intra-mural giant myoma. Before the removal operation of myoma, screening tests showed no abnormalities, including prothrombin time and activated partial thromboplastin time. However, Fbg level was not determined. The patient wanted to receive early surgical treatment, and an abdominal hysterectomy was performed as usual and the intra-operative blood loss was 100 g (ml). However, we found subcutaneous and pelvic hematomas, although active bleeding was not recognized on an emergent computed tomography examination. At that time, we noticed a low level of plasma Fbg (47 mg/dl). We performed a re-laparotomy to remove hematomas. All ligated blood vessels were re-ligated, and oozing points were vaporized. Around the re-operation, six units of fresh frozen plasma and twelve units of red blood cell suspension were transfused. The clinical course after the 2<sup>nd</sup> operation was uneventful except for the low level of Fbg. An additional study showed that the value of the Fbg activity and antigen was dissociated, and the patient was diagnosed CD with <span style="white-space:nowrap;">γ</span>275 Arg to His (CGC to CAC) mutation.
基金This study was supported by grants from Clinical Research Plan of SHDC(SHDC2020CR1040B).
文摘Background/aim:This study was aimed at evaluating 5-year effectiveness and safety in participants after XIENCE PRIME Everolimus Elution Coronary Stent System(EECSS)implantation.Materials and methods:From December 2013 to May 2014,108 patients(127 lesions)were treated with the XIENCE PRIME EECSS.The entire follow-up included annual assessments for 5 years after treatment or until one of the clinical endpoints was reached.We evaluated the 5-year clinical outcomes with Kaplan-Meier analysis and the Cox regression model.Results:Nearly three-quarters of the participants were men(76.8%),and the average age was 65.6±10.8 years.Bifurcation lesions accounted for 96.1%(122 lesions),and left main lesions accounted for 3.9%(five lesions),with a total count of 127 lesions.The cumulative rate of major adverse cardiac events was as follows:1 year,1.9%;2 year,4.0%;and 5 year,10.0%.No definite or probable stent thrombosis was observed,and the rate of target lesion failure was only 3.3%over 5 years.The cumulative rate of major bleeding eventually increased to only 4.4%.Conclusions:The 5-year clinical outcomes were favorable in patients treated with XIENCE PRIME EECSS,and the incidence of stent thrombosis and target lesion failure was relatively low.The incidence of major bleeding gradually increased but remained moderate.
