BACKGROUND Factor XIII(FXIII)deficiency is a rare yet profound coagulopathy.FXIII plays a pivotal role in hemostasis,and deficiencies in this factor can precipitate unchecked or spontaneous hemorrhaging.Immunological ...BACKGROUND Factor XIII(FXIII)deficiency is a rare yet profound coagulopathy.FXIII plays a pivotal role in hemostasis,and deficiencies in this factor can precipitate unchecked or spontaneous hemorrhaging.Immunological assays for detecting FXIII inhibitors are indispensable for diagnosing acquired FXIII deficiency;however,the availability of suitable testing facilities is limited,resulting in prolonged turnaround times for these assays.CASE SUMMARY In this case study,a 53-year-old male devoid of significant medical history presented with recurrent intracranial hemorrhages and a hematoma in the right hip.Subsequent genetic analysis revealed a homozygous mutation in the ACE gene,confirming the diagnosis of acquired FXIII deficiency.CONCLUSION This case underscores the significance of considering acquired deficiencies in clotting factors when evaluating patients with unexplained bleeding episodes.展开更多
Objective： To provide a comprehensive literature review on roles of coagulation factor XIII （FXIII） in coagulation, wound healing, neoplasm, bone metabolism, and pregnancy. Data Sources： All articles in PubMed wit...Objective： To provide a comprehensive literature review on roles of coagulation factor XIII （FXIII） in coagulation, wound healing, neoplasm, bone metabolism, and pregnancy. Data Sources： All articles in PubMed with key words ＂Coagulation factor XIII＂, ＂wound＂, ＂＇leukeraia＂, ＂tumor＂, ＂bone,＂ and ＂pregnancy＇＂ with published date from 2001 to 2016 were included in the study. Frequently cited publications before 2000 were also included. Study Selection： We reviewed the role of FXIll in biologic processes as documented in clinical, animal, and in vitro studies. Results： FXIII, a nlember of the transglutaminase （TG） family, plays key roles in various biological processes. Besides its well-known function in coagulation, the cross-linking of small molecules catalyzed by FXIII has been found in studies to help promote wound healing, improve bone metabolism, and prevent miscarriages. The study has also shown that FXIII concentration level differs in the blood of patients with leukemia and solid tumors and offers promises as a diagnostic indicator Conclusions： FXIII has many more biologic functions besides being known as coagulation factor. The TG activity of FXIII contributes to several processes, including wound healing, bone extracellular matrix stabilization, and the interaction between embryo and decidua of uterus. Further research is needed to elucidate the link between FXIII and leukemia and solid tumors.展开更多
BACKGROUND： Recurrent pregnancy loss （RPL） is a heterogeneous condition and thrombophilias have been considered as a probable cause. OBJECTIVE： The aim of this study was to investigate the prevalence of the coagul...BACKGROUND： Recurrent pregnancy loss （RPL） is a heterogeneous condition and thrombophilias have been considered as a probable cause. OBJECTIVE： The aim of this study was to investigate the prevalence of the coagulation factor XIII Va134Leu polymorphism among women with unexplained RPL. METHODS： A total of 140 women with a history of unexplained RPL and 100 age-matched healthy fertile women were recruited. The presence of FXIII Va134Leu polymorphism among the cases and controls was investigated using PCR-RFLP method. RESULTS： Genotype analyses of the subjects revealed that the patients had a significantly higher prevalence of V/L and L/L than the controls （P〈 0.05）： 33.5% vs. 15%, and 9.2% vs. 2%, respectively. CONCLUSION： These results indicate a significant association between FXIII Va134Leu polymorphism and unexplained RPL in the Iranian patient.展开更多
基金Supported by the Medical and Health Science Foundation of Zhejiang,No.2023KY186Hangzhou Science and Technology Development Plan Guide Project,No.20220919Y023the Hangzhou Medical Key Discipline Construction Program,No.2021.
文摘BACKGROUND Factor XIII(FXIII)deficiency is a rare yet profound coagulopathy.FXIII plays a pivotal role in hemostasis,and deficiencies in this factor can precipitate unchecked or spontaneous hemorrhaging.Immunological assays for detecting FXIII inhibitors are indispensable for diagnosing acquired FXIII deficiency;however,the availability of suitable testing facilities is limited,resulting in prolonged turnaround times for these assays.CASE SUMMARY In this case study,a 53-year-old male devoid of significant medical history presented with recurrent intracranial hemorrhages and a hematoma in the right hip.Subsequent genetic analysis revealed a homozygous mutation in the ACE gene,confirming the diagnosis of acquired FXIII deficiency.CONCLUSION This case underscores the significance of considering acquired deficiencies in clotting factors when evaluating patients with unexplained bleeding episodes.
文摘Objective： To provide a comprehensive literature review on roles of coagulation factor XIII （FXIII） in coagulation, wound healing, neoplasm, bone metabolism, and pregnancy. Data Sources： All articles in PubMed with key words ＂Coagulation factor XIII＂, ＂wound＂, ＂＇leukeraia＂, ＂tumor＂, ＂bone,＂ and ＂pregnancy＇＂ with published date from 2001 to 2016 were included in the study. Frequently cited publications before 2000 were also included. Study Selection： We reviewed the role of FXIll in biologic processes as documented in clinical, animal, and in vitro studies. Results： FXIII, a nlember of the transglutaminase （TG） family, plays key roles in various biological processes. Besides its well-known function in coagulation, the cross-linking of small molecules catalyzed by FXIII has been found in studies to help promote wound healing, improve bone metabolism, and prevent miscarriages. The study has also shown that FXIII concentration level differs in the blood of patients with leukemia and solid tumors and offers promises as a diagnostic indicator Conclusions： FXIII has many more biologic functions besides being known as coagulation factor. The TG activity of FXIII contributes to several processes, including wound healing, bone extracellular matrix stabilization, and the interaction between embryo and decidua of uterus. Further research is needed to elucidate the link between FXIII and leukemia and solid tumors.
文摘BACKGROUND： Recurrent pregnancy loss （RPL） is a heterogeneous condition and thrombophilias have been considered as a probable cause. OBJECTIVE： The aim of this study was to investigate the prevalence of the coagulation factor XIII Va134Leu polymorphism among women with unexplained RPL. METHODS： A total of 140 women with a history of unexplained RPL and 100 age-matched healthy fertile women were recruited. The presence of FXIII Va134Leu polymorphism among the cases and controls was investigated using PCR-RFLP method. RESULTS： Genotype analyses of the subjects revealed that the patients had a significantly higher prevalence of V/L and L/L than the controls （P〈 0.05）： 33.5% vs. 15%, and 9.2% vs. 2%, respectively. CONCLUSION： These results indicate a significant association between FXIII Va134Leu polymorphism and unexplained RPL in the Iranian patient.
基金the Key Program of Chinese People’s Armed Police Forces,No.WKH2009Z04the Program of Logistics College of Chinese People’s Armed Police Forces,No.WHM201209~~
