Journal of Thrombosis and Haemostasis最新文献

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Bleeding symptoms in persons with rare bleeding disorders and a heterozygous genotype: data from the RBiN study.
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-06 DOI: 10.1016/j.jtha.2025.02.030
Sterre P E Willems, Marjon H Cnossen, Nick van Es, Paul L den Exter, Ilmar C Kruis, Dominique P M S M Maas, Karina Meijer, Laurens Nieuwenhuizen, Sanna Rijpma, Joline L Saes, Annet Simons, Roger E G Schutgens, Marjan Weiss, Nicole M A Blijlevens, Waander L van Heerde, Saskia E M Schols
{"title":"Bleeding symptoms in persons with rare bleeding disorders and a heterozygous genotype: data from the RBiN study.","authors":"Sterre P E Willems, Marjon H Cnossen, Nick van Es, Paul L den Exter, Ilmar C Kruis, Dominique P M S M Maas, Karina Meijer, Laurens Nieuwenhuizen, Sanna Rijpma, Joline L Saes, Annet Simons, Roger E G Schutgens, Marjan Weiss, Nicole M A Blijlevens, Waander L van Heerde, Saskia E M Schols","doi":"10.1016/j.jtha.2025.02.030","DOIUrl":"https://doi.org/10.1016/j.jtha.2025.02.030","url":null,"abstract":"<p><strong>Background: </strong>Limited data exists on persons with rare bleeding disorders (RBDs) possessing a heterozygous genotype, as most studies focus on bi-allelic genotypes and more severe coagulation factor deficiencies. A growing body of evidence suggests that persons with a heterozygous genotype experience clinically relevant bleeding symptoms.</p><p><strong>Objectives: </strong>Explore the incidence of bleeding symptoms and postoperative bleeding in persons with a heterozygous genotype.</p><p><strong>Patients/methods: </strong>This cross-sectional sub-study of the Rare Bleeding Disorders in the Netherlands study (2017-2019) included persons with rare coagulation factor deficiencies and disorders of fibrinolysis with a heterozygous or bi-allelic genotype. Clinical data and laboratory samples were collected during a single study visit along with questionnaires.</p><p><strong>Results: </strong>Eighty-six persons with a heterozygous genotype and fifty-five with a bi-allelic genotype were included. Median factor activity levels in persons with a heterozygous genotype approached 50% with considerable heterogeneity (range: 11-93%). In 75%, persons with a heterozygous genotype reported bleeding severity of grade II or III. Female specific bleeding was common. In total, 425 surgical procedures were performed. Persons with a heterozygous genotype were less likely to receive periprocedural treatment, and omission of periprocedural treatment was associated with postoperative bleeding in procedures with intermediate-high bleeding risk. Postoperative bleeding was comparable for persons with a heterozygous genotype (35%; 59/171) and a bi-allelic genotype (35%; 86/247, p=.926).</p><p><strong>Conclusions: </strong>In our RBD population, the majority of persons possessing a heterozygous genotype exhibited spontaneous bleeding symptoms. Especially in intermediate-high risk procedures, a proactive approach to periprocedural hemostatic treatment in persons with a heterozygous genotype seems beneficial.</p>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143586177","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical and radiologic characteristics of chronic thromboembolic pulmonary hypertension patients without a history of acute venous thromboembolism.
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-06 DOI: 10.1016/j.jtha.2025.02.033
Coen van Kan, Josien van Es, Hachim El Idrissi, Dieuwertje Ruigrok, Esther J Nossent, Jurjan Aman, Frederikus A Klok, Harm Jan Bogaard, Lilian J Meijboom, Anton Vonk-Noordegraaf
{"title":"Clinical and radiologic characteristics of chronic thromboembolic pulmonary hypertension patients without a history of acute venous thromboembolism.","authors":"Coen van Kan, Josien van Es, Hachim El Idrissi, Dieuwertje Ruigrok, Esther J Nossent, Jurjan Aman, Frederikus A Klok, Harm Jan Bogaard, Lilian J Meijboom, Anton Vonk-Noordegraaf","doi":"10.1016/j.jtha.2025.02.033","DOIUrl":"10.1016/j.jtha.2025.02.033","url":null,"abstract":"<p><strong>Background: </strong>Chronic thromboembolic pulmonary hypertension (CTEPH) is thought to result from incomplete resolution of vascular obstruction following acute pulmonary embolism. However, at least 25% of patients with CTEPH do not have a documented episode of acute venous thromboembolism (VTE).</p><p><strong>Objectives: </strong>We hypothesized that patients without a VTE in their past medical history have different clinical and radiologic characteristics compared with patients with CTEPH with previous acute VTE.</p><p><strong>Methods: </strong>Baseline data and the history of VTE were retrospectively retrieved from the charts of all patients with CTEPH included between 2014 and 2022 in the Amsterdam University Medical Center CTEPH registry. Computed tomography pulmonary angiography, right heart catheterization, and pulmonary function tests were performed in all patients. Subsegmental disease was defined by the presence of a contrast defect in the pulmonary arterial vessels after the first arterial branch division.</p><p><strong>Results: </strong>A total of 262 patients with CTEPH were included; 47 patients (18%) did not have previous acute VTE. Baseline radiologic assessment showed that subsegmental disease was more frequent in patients without previous VTE (n = 16/43; 35%, 95% CI: 22-49) than in patients with previous VTE (n = 27/214; 13% [95% CI: 9.0-18], OR: 2.6 [95% CI: 1.2-5.7]). The patients without previous VTE were less frequently assigned to pulmonary endarterectomy than patients with acute VTE (30% [95% CI: 18-44] vs 56% [95% CI: 49-62]; OR: 0.5 [95% CI: 0.2-0.9]). Comorbidities, pulmonary function, and hemodynamics were not different.</p><p><strong>Conclusion: </strong>Patients with CTEPH without previous VTE had more distally located disease on imaging than patients with CTEPH with previous VTE and were less often subjected to pulmonary endarterectomy.</p>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143586180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Treatment and outcomes after on-treatment recurrent venous thromboembolism in patients with cancer: a post hoc analysis of the Hokusai venous thromboembolism cancer study.
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-04 DOI: 10.1016/j.jtha.2025.02.022
Vincent R Lanting, Kika van Bergen En Henegouwen, Floris T M Bosch, Michael A Grosso, Annelise Segers, Gary E Raskob, T Pieter Willem Kamphuisen, Harry R Büller, Peter Verhamme, Jeffrey I Weitz, Marcello Di Nisio, Marc Carrier, Nick van Es, Tzu-Fei Wang
{"title":"Treatment and outcomes after on-treatment recurrent venous thromboembolism in patients with cancer: a post hoc analysis of the Hokusai venous thromboembolism cancer study.","authors":"Vincent R Lanting, Kika van Bergen En Henegouwen, Floris T M Bosch, Michael A Grosso, Annelise Segers, Gary E Raskob, T Pieter Willem Kamphuisen, Harry R Büller, Peter Verhamme, Jeffrey I Weitz, Marcello Di Nisio, Marc Carrier, Nick van Es, Tzu-Fei Wang","doi":"10.1016/j.jtha.2025.02.022","DOIUrl":"10.1016/j.jtha.2025.02.022","url":null,"abstract":"<p><strong>Background: </strong>The management of recurrent venous thromboembolism (VTE) despite anticoagulant treatment in patients with cancer is uncertain. To address this, we used data from the Hokusai VTE Cancer trial, which compared edoxaban with dalteparin to treat cancer-associated VTE.</p><p><strong>Objectives: </strong>To characterize and evaluate anticoagulant treatment strategies during and after on-treatment recurrent VTE, including the type and dose of anticoagulant.</p><p><strong>Methods: </strong>In this post hoc analysis, all patients with adjudicated on-treatment recurrent VTE within 12 months after randomization were included. Outcomes were second recurrent VTE and major bleeding within 3 months after the first recurrent VTE.</p><p><strong>Results: </strong>A total of 67 patients developed on-treatment recurrent VTE while receiving therapeutic-dose edoxaban (31%), therapeutic-dose low-molecular-weight heparin (LMWH) (34%), maintenance-dose LMWH (21%), or other therapies (14%). After the recurrent event, 28 patients (42%) received an increased dose, 35 (52%) a comparable dose, and 4 (6%) a reduced dose or stopped anticoagulants. Common treatment regimens included supratherapeutic-dose LMWH (21%), therapeutic-dose LMWH (51%), direct oral anticoagulants (16%), or another treatment strategy (12%). In the 3 months after recurrent VTE, 6 (9%) patients had a second recurrence and 7 (10%) had major bleeding.</p><p><strong>Conclusion: </strong>Treatment strategies for recurrent VTE in patients with cancer are heterogeneous. The risk of a second recurrence and major bleeding are considerable. More studies are needed to determine the optimal treatment strategy for recurrent cancer-associated thrombosis.</p>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143573382","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Out-of-hospital cardiac arrest associated with venous thromboembolism–a Swedish population-based cohort study 院外心脏骤停与静脉血栓栓塞相关——瑞典一项基于人群的队列研究
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-01 DOI: 10.1016/j.jtha.2024.11.021
Emma Bendz , Anna Oksanen , Susanna Larsson , Joel Ohm , Therese Djärv , Maria Bruzelius
{"title":"Out-of-hospital cardiac arrest associated with venous thromboembolism–a Swedish population-based cohort study","authors":"Emma Bendz ,&nbsp;Anna Oksanen ,&nbsp;Susanna Larsson ,&nbsp;Joel Ohm ,&nbsp;Therese Djärv ,&nbsp;Maria Bruzelius","doi":"10.1016/j.jtha.2024.11.021","DOIUrl":"10.1016/j.jtha.2024.11.021","url":null,"abstract":"<div><h3>Background</h3><div>Venous thromboembolism (VTE) is a common and preventable cardiovascular disease but is potentially fatal if it presents as pulmonary embolism. There are few population-based studies on out-of-hospital cardiac arrest (OHCA) associated with VTE.</div><div>Objectives: We aimed to investigate the prevalence and survival of VTE-associated OHCA in relation to sex, age, and presenting rhythm.</div></div><div><h3>Methods</h3><div>This population-based cohort included all individuals over 15 years registered with a medical OHCA in Sweden 2008-2018. Data from national registers were used. Association with presenting rhythm, 30-day survival, and sex was estimated using adjusted multinomial and binary logistic regression for odds ratios (ORs) with 95% CI.</div></div><div><h3>Results</h3><div>OHCA was associated with VTE in 852 (2.0%) of 41 813 individuals. The median age for VTE-associated OHCA was 69, compared to 73 for the entire cohort, with a higher female prevalence (45% vs 34%). Younger women had a proportionally higher incidence of VTE-associated OHCA than men of the same age. Pulseless electrical activity and asystole had adjusted ORs of 17.2 (95% CI, 11.3-26.0) and 9.59 (95% CI, 6.37-14.4) for VTE-associated OHCA compared with the entire cohort. The 30-day survival was substantially lower in the VTE group compared with the overall medical OHCA group, 2.0% vs 12%, adjusted OR 0.25 (95% CI, 0.13-0.47).</div></div><div><h3>Conclusion</h3><div>This nationwide study confirms that VTE-associated OHCA is uncommon and almost always fatal. Despite fewer women experiencing OHCA, a higher proportion had VTE-associated OHCA, especially younger women. Our findings highlight the need for better prevention and identification of VTE-associated OHCA, particularly in women.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"23 3","pages":"Pages 968-977"},"PeriodicalIF":5.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142813643","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Intercellular synergy between protease-activated receptors 1 and 4 during mouse development 小鼠发育过程中蛋白酶激活受体1和4的细胞间协同作用。
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-01 DOI: 10.1016/j.jtha.2024.11.022
Rahul Rajala , Courtney T. Griffin
{"title":"Intercellular synergy between protease-activated receptors 1 and 4 during mouse development","authors":"Rahul Rajala ,&nbsp;Courtney T. Griffin","doi":"10.1016/j.jtha.2024.11.022","DOIUrl":"10.1016/j.jtha.2024.11.022","url":null,"abstract":"","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"23 3","pages":"Pages 1117-1119"},"PeriodicalIF":5.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818496","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical and genetic characteristics of 40 patients with nonmuscle myosin heavy chain 9-related disease (MYH9-RD) misdiagnosed as immune thrombocytopenia: a retrospective analysis in China 40例被误诊为ITP的非肌球蛋白重链9相关疾病(MYH9-RD)患者的临床和遗传特征:中国的一项回顾性分析。
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-01 DOI: 10.1016/j.jtha.2024.12.001
Abdul Rehman Arif , Hu Zhou , Yongjun Fang , Yunfeng Cheng , Jieyu Ye , Wenlan Chen , Yajie Ding , Li Cai , Mei Xue , Heng Mei , Yadan Wang
{"title":"Clinical and genetic characteristics of 40 patients with nonmuscle myosin heavy chain 9-related disease (MYH9-RD) misdiagnosed as immune thrombocytopenia: a retrospective analysis in China","authors":"Abdul Rehman Arif ,&nbsp;Hu Zhou ,&nbsp;Yongjun Fang ,&nbsp;Yunfeng Cheng ,&nbsp;Jieyu Ye ,&nbsp;Wenlan Chen ,&nbsp;Yajie Ding ,&nbsp;Li Cai ,&nbsp;Mei Xue ,&nbsp;Heng Mei ,&nbsp;Yadan Wang","doi":"10.1016/j.jtha.2024.12.001","DOIUrl":"10.1016/j.jtha.2024.12.001","url":null,"abstract":"<div><h3>Background</h3><div>Myosin heavy chain 9-related diseases (MYH9-RDs) are rare autosomal dominant platelet disorders characterized by macrothrombocytopenia and leukocyte inclusion bodies. They can manifest with nonhematological complications, including deafness, nephropathy, or cataracts. Due to its rarity and its similar clinical presentation with immune thrombocytopenia (ITP), MYH9-RD is often misdiagnosed as ITP, leading to inappropriate treatment and delayed management of complications.</div></div><div><h3>Objectives</h3><div>This study aimed to evaluate clinical, therapeutic, and genetic aspects of patients with MYH9-RD misdiagnosed with ITP, comparing differences between Chinese pediatric and adult cases of this condition.</div></div><div><h3>Methods</h3><div>This multicenter retrospective study included data obtained from Chinese patients diagnosed with MYH9-RD between January 2014 and December 2023 at 5 centers.</div></div><div><h3>Results</h3><div>Adults exhibited significantly longer median misdiagnosis (9 years vs 0.2 years, <em>P</em> &lt; .001) and treatment durations (1.5 years vs 0.1 years, <em>P</em> &lt; .001) than children. Nonhematological manifestations were exclusive to adults (10/21). All patients received inappropriate ITP treatments, with adults receiving more different treatments. Genetic analysis revealed 21 spontaneous mutations (52.5%), 12 familial mutations, and 7 mutations with unknown inheritance patterns. Two novel mutations (p.G1517V and p.K1674Q) were identified. Patients with the p.R702C mutation demonstrated early-stage kidney injury and hearing loss.</div></div><div><h3>Conclusion</h3><div>Adult patients with MYH9-RD have greater risk of misdiagnosis, prolonged inappropriate treatment, and nonhematological complications than pediatric patients. Enhanced awareness, consideration of mean platelet volume, family history, and genetic screening are crucial for accurate MYH9-RD diagnosis and management. The incidence of spontaneous mutations and identified genotype–phenotype correlations warrant further investigation in the Chinese population.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"23 3","pages":"Pages 1043-1051"},"PeriodicalIF":5.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142822211","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Transfusion biomaterials for hemostasis 用于止血的输血生物材料。
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-01 DOI: 10.1016/j.jtha.2024.11.030
Ethan Pozy , Ashley C. Brown
{"title":"Transfusion biomaterials for hemostasis","authors":"Ethan Pozy ,&nbsp;Ashley C. Brown","doi":"10.1016/j.jtha.2024.11.030","DOIUrl":"10.1016/j.jtha.2024.11.030","url":null,"abstract":"<div><div>Bleeding is a leading cause of trauma deaths and surgical complications. Excessive bleeding has traditionally been treated with the transfusion of donated blood. However, the complicated logistics of sourcing and storing donated blood increases the cost and reduces the accessibility of treatment, particularly as rates of blood donation decline. Advances in biomaterials for targeted drug delivery have presented the opportunity for alternative synthetic injectable hemostats. Among these leading technologies are lipid and polymeric particles and polymer platforms that bind to ligands present at wound sites and amplify hemostatic pathways. As leading hemostatic biomaterials advance toward clinical application, we review current preclinical research models and findings as well as future research directions for next-generation biomaterial injectable hemostatic technologies.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"23 3","pages":"Pages 790-803"},"PeriodicalIF":5.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142829158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Hemophilia B Leyden: characteristics and natural history in the International Pediatric Network of Hemophilia Management Registry 血友病B莱顿:特征和自然历史在PedgNet注册。
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-01 DOI: 10.1016/j.jtha.2024.12.020
Mutlu Kartal-Kaess , Fernando Pinto , Veerle Labarque , Marloes de Kovel , Beatrice Nolan , Manuel Carcao , Roseline d’Oiron , Torben Stamm Mikkelsen , Rolf Ljung , Nadine G. Andersson
{"title":"Hemophilia B Leyden: characteristics and natural history in the International Pediatric Network of Hemophilia Management Registry","authors":"Mutlu Kartal-Kaess ,&nbsp;Fernando Pinto ,&nbsp;Veerle Labarque ,&nbsp;Marloes de Kovel ,&nbsp;Beatrice Nolan ,&nbsp;Manuel Carcao ,&nbsp;Roseline d’Oiron ,&nbsp;Torben Stamm Mikkelsen ,&nbsp;Rolf Ljung ,&nbsp;Nadine G. Andersson","doi":"10.1016/j.jtha.2024.12.020","DOIUrl":"10.1016/j.jtha.2024.12.020","url":null,"abstract":"<div><h3>Background</h3><div>A unique form of hemophilia B (HB) is HB Leyden. We evaluated the international Pediatric Network on Hemophilia Management Registry (PedNet) database to explore the natural history of HB Leyden, investigate genotype-phenotype associations, and guide clinical decision-making.</div></div><div><h3>Objectives</h3><div>To assess the association between genetic variants, endogenous factor (F)IX levels over time, treatment, and bleeding phenotype in children with HB Leyden.</div></div><div><h3>Methods</h3><div>Data on genetic variants, FIX levels at diagnosis and over time, bleeding, and treatment details were extracted from the international PedNet in children with hemophilia born since 2000.</div></div><div><h3>Results</h3><div>Of 457 individuals with HB, 24 showed an HB Leyden genotype. The most frequent <em>F9</em> variant was c.-35G&gt;A, affecting 14 individuals, followed by c.-35G&gt;C (<em>n</em> = 4), c.-49T&gt;A (<em>n</em> = 2), and c.-52C&gt;T, c.-34A&gt;G, and c.-22delT (<em>n</em> = 1 each). Major clinical differences in bleeding and treatment modality were observed when comparing c.-35G&gt;A with non-c.-35G&gt;A genotypes. For all children with a c.-35G&gt;A genotype, FIX levels increased before the age of 4 years but did not normalize over time, irrespective of initial severity. In children with non-c.-35G&gt;A genotypes, an increase in FIX was less common (4/9) and occurred later.</div></div><div><h3>Conclusion</h3><div>HB Leyden is caused by the variant c.-35G&gt;A in &gt;50% of cases in whom a FIX increase occurs at very young ages, which is associated with low bleeding rates. This contrasts with the phenotype of individuals with HB Leyden due to a non-c.-35G&gt;A variant. Our study may thus help guide clinical decision-making in this rare HB entity.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"23 3","pages":"Pages 921-927"},"PeriodicalIF":5.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915239","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
High risk of long-term recurrence after a first episode of venous thromboembolism during pregnancy or postpartum: the REcurrence after a PrEgnAncy related Thrombosis (REPEAT) Study 孕期或产后首次发生静脉血栓栓塞后长期复发的高风险:与血栓形成有关的孕期或产后复发(REPEAT)研究。
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-01 DOI: 10.1016/j.jtha.2024.09.039
Manal Ibrahim-Kosta , Sarah El Harake , Barbara Leclercq , Céline De Mari , Jean-François Secondi , Emilie Paoletti , Pierre Suchon , Yasmine Benredouane , Dominique Brunet , Marie-Christine Barthet , Maria Bruzelius , Gaëlle Munsch , David-Alexandre Trégouët , Pierre-Emmanuel Morange , Louisa Goumidi , Gabrielle Sarlon-Bartoli
{"title":"High risk of long-term recurrence after a first episode of venous thromboembolism during pregnancy or postpartum: the REcurrence after a PrEgnAncy related Thrombosis (REPEAT) Study","authors":"Manal Ibrahim-Kosta ,&nbsp;Sarah El Harake ,&nbsp;Barbara Leclercq ,&nbsp;Céline De Mari ,&nbsp;Jean-François Secondi ,&nbsp;Emilie Paoletti ,&nbsp;Pierre Suchon ,&nbsp;Yasmine Benredouane ,&nbsp;Dominique Brunet ,&nbsp;Marie-Christine Barthet ,&nbsp;Maria Bruzelius ,&nbsp;Gaëlle Munsch ,&nbsp;David-Alexandre Trégouët ,&nbsp;Pierre-Emmanuel Morange ,&nbsp;Louisa Goumidi ,&nbsp;Gabrielle Sarlon-Bartoli","doi":"10.1016/j.jtha.2024.09.039","DOIUrl":"10.1016/j.jtha.2024.09.039","url":null,"abstract":"<div><h3>Background</h3><div>The long-term recurrence risk after a pregnancy-associated venous thromboembolism (VTE) is sparsely assessed.</div></div><div><h3>Objectives</h3><div>To determine the rate of recurrence after a pregnancy-associated VTE and identify associated risk factors.</div></div><div><h3>Methods</h3><div>Five hundred eighty-seven women with a history of first VTE occurring during pregnancy or up to 3 months after delivery were referred to La Timone Hospital, Marseille, France. Women were consecutively included between 2000 and 2015. VTE characteristics and biological parameters were collected upon inclusion. During the 2016-2019 period, patients were recontacted to gather information on postinclusion VTE. A weighted Cox model, adapted to the study’s ambispective design, was used to analyze pre- and postinclusion VTE recurrences.</div></div><div><h3>Results</h3><div>After quality controls, 583 women were analyzed. The incidence of recurrent VTE was 2.4% person-years. The cumulative risk of VTE recurrence was 38% (<em>n</em> = 221), with a median follow-up of 31 years (95% CI: 27-35); 6%, 13%, 17%, and 30% at 2, 5, 10, and 30 years respectively. Pulmonary embolism at first event was associated with a 2-fold increased risk of pulmonary embolism at recurrence compared with isolated deep venous thrombosis (DVT, hazard ratio [HR]: 2.63; 95% CI: 1.44-4.82). Risk factors significantly associated with recurrence were interrupted pregnancies (HR: 1.85; 95% CI: 1.18-2.90), lower limb DVT (HR: 2.95; 95% CI: 1.16-7.49), and AB blood group (HR: 1.71; 95% CI: 1.06-2.77).</div></div><div><h3>Conclusion</h3><div>Although the recurrence risk is low within the first 10 years after a pregnancy-associated VTE, one-third of patients experienced a new event over a 30-year period. Interrupted pregnancies, lower limb DVT, and AB blood group were associated with higher risk of recurrence.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"23 3","pages":"Pages 937-946"},"PeriodicalIF":5.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142564310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of diagnostic coding schemas on major bleeding risk assessment for oral anticoagulants in patients with atrial fibrillation using administrative claims data 使用行政索赔数据的诊断编码方案对房颤患者口服抗凝剂大出血风险评估的影响
IF 5.5 2区 医学
Journal of Thrombosis and Haemostasis Pub Date : 2025-03-01 DOI: 10.1016/j.jtha.2024.12.010
Hsiu-Ting Chien , Tze-Fan Chao , Rosa Wang , Chia-Jui Chang , Shin-Yi Lin , Fang-Ju Lin
{"title":"Impact of diagnostic coding schemas on major bleeding risk assessment for oral anticoagulants in patients with atrial fibrillation using administrative claims data","authors":"Hsiu-Ting Chien ,&nbsp;Tze-Fan Chao ,&nbsp;Rosa Wang ,&nbsp;Chia-Jui Chang ,&nbsp;Shin-Yi Lin ,&nbsp;Fang-Ju Lin","doi":"10.1016/j.jtha.2024.12.010","DOIUrl":"10.1016/j.jtha.2024.12.010","url":null,"abstract":"<div><h3>Background</h3><div>Reliable diagnostic coding schemas are essential for accurately assessing bleeding risks in patients on oral anticoagulants, particularly in observational studies.</div></div><div><h3>Objectives</h3><div>This study evaluated how different published diagnostic coding schemas impact the assessment of major bleeding risks associated with direct oral anticoagulants (DOACs) and warfarin.</div></div><div><h3>Methods</h3><div>This retrospective cohort study included patients with atrial fibrillation who initiated DOACs or warfarin between 2012 and 2019 using Taiwan’s national claims database. Major bleeding events, including gastrointestinal bleeding, intracranial hemorrhage (ICH), and other major bleeding events, were identified using coding schemas from Cunningham et al., the Mini-Sentinel protocol, and Yao et al. Propensity score matching was performed to ensure covariate balance. Incidence rates and hazard ratios (HRs) were estimated to evaluate the bleeding risks.</div></div><div><h3>Results</h3><div>After matching, each cohort comprised 20 704 patients. The number of reported major bleeding events was influenced by the strictness of the coding schema, with Cunningham yielding the most events, followed by the Mini-Sentinel and Yao schemas. DOACs were associated with a consistently lower risk of composite major bleeding (HR range across different coding schemas, 0.73-0.76; all <em>P</em> &lt; .05) and ICH (HR range, 0.43-0.63; all <em>P</em> &lt; .05) but not gastrointestinal bleeding (HR range, 0.87-0.90; all <em>P</em> &gt; .05), regardless of the coding schema applied. Restricting ICH definitions to primary diagnosis or spontaneous cases revealed a more pronounced reduction in ICH risk associated with DOACs.</div></div><div><h3>Conclusion</h3><div>While the choice of coding schemas has a negligible impact on overall bleeding risk comparisons between DOACs and warfarin, it significantly affects ICH risk assessment. This underscores the importance of careful coding schema selection in observational studies evaluating major bleeding risks.</div></div>","PeriodicalId":17326,"journal":{"name":"Journal of Thrombosis and Haemostasis","volume":"23 3","pages":"Pages 877-887"},"PeriodicalIF":5.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142872469","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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