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Venous Thromboembolism Occurrence and Association with Gastrointestinal Disorders in Children with Cystic Fibrosis: An Analysis from the TriNetX Research Network Global Multicenter Real-World Dataset. 囊性纤维化儿童静脉血栓栓塞的发生及其与胃肠道疾病的关联:来自TriNetX研究网络全球多中心真实世界数据集的分析
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-10-01 Epub Date: 2025-01-13 DOI: 10.1055/s-0044-1801825
Carolena Trocchia, Dina Ashour, Maua Mosha, Bailey Hamner, Marisol Betensky, Neil Goldenberg, Racha Khalaf

The purpose of this study is to (1) estimate and compare the prevalence of venous thromboembolism (VTE) in children (age 0 to ≤21) with versus without cystic fibrosis (CF); (2) investigate putative associations between specific gastrointestinal (GI) manifestations and the development of VTE among children with CF. This was a multicenter case-control analysis among patients aged 0 to ≤ 21 years between 2010 and 2020, using the TriNetX Research Network. Data queries included ICD-9/10 (International Classification of Diseases-9th/10th Revision) diagnosis codes. Bivariate associations with VTE among CF patients were compared using Chi-square testing for categorical variables and Student's t-test for continuous variables. We used multivariable logistic regression to test for independent associations of GI manifestations with VTE among children with CF, with adjustment for other salient covariates. There was a total of 7,689 children with and 22,327,660 without CF. The frequency of occurrence of VTE was increased nearly 20-fold among those with, as compared with without CF (130 vs. 7 per 10,000 patients). Acute pancreatitis (adjusted odd ratio [aOR] = 3.80, [95% confidence interval, CI: 2.00-7.22]), biliary disease (aOR = 2.17 [95% CI: 1.17-4.03]), gastrostomy status (aOR = 2.01 [95% CI: 1.27-3.18]), and malabsorption/malnutrition (aOR = 2.41 [95% CI: 1.52-3.82]) were each associated with a higher likelihood of VTE among children with CF. In conclusion, we found a significantly increased frequency of VTE occurrence and association of specific GI diseases as independent risk factors for VTE among children with CF compared with those without.

本研究的目的是:(1)估计和比较患有和不患有囊性纤维化(CF)的儿童(0至≤21岁)静脉血栓栓塞(VTE)的患病率;(2)调查CF患儿中特定胃肠道(GI)表现与静脉血栓栓塞(VTE)发展之间的可能关联。这是一项多中心病例对照分析,研究对象为2010年至2020年期间0至≤21岁的患者,使用TriNetX研究网络。数据查询包括ICD-9/10(国际疾病分类-第9/10版)诊断代码。比较CF患者与VTE的双变量相关性,对分类变量使用卡方检验,对连续变量使用学生t检验。我们使用多变量逻辑回归来检验CF患儿胃肠道表现与静脉血栓栓塞的独立关联,并对其他显著协变量进行校正。共有7,689名患有CF的儿童和22,327,660名没有CF的儿童。与没有CF的儿童相比,患有CF的儿童发生静脉血栓栓塞的频率增加了近20倍(130比7 / 10,000)。急性胰腺炎(调整奇比[aOR] = 3.80,[95%可信区间,CI: 2.00-7.22])、胆道疾病(aOR = 2.17 [95% CI: 1.17-4.03])、胃造口状态(aOR = 2.01 [95% CI: 1.27-3.18])和吸收不良/营养不良(aOR = 2.41 [95% CI: 2.00-7.22])。[1.52-3.82])均与CF患儿发生静脉血栓栓塞(VTE)的可能性较高相关。总之,我们发现与非CF患儿相比,VTE的发生频率显著增加,特异性胃肠道疾病作为VTE的独立危险因素的关联也显著增加。
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引用次数: 0
Anticoagulants in Children with Renal Impairment: A Narrative Review. 儿童肾脏损害的抗凝治疗:一个叙述性的回顾。
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-10-01 Epub Date: 2025-03-28 DOI: 10.1055/a-2546-0126
Amy L Kiskaddon, Daniel M Witt, Marisol Betensky, Anthony A Sochet, Amanda Memken, Christoph Male, Neil A Goldenberg

Venous thromboembolism is a common cause of morbidity and mortality in children with renal disease. To properly treat and prevent thromboembolism in this patient population, it is important to be familiar with the multitude of anticoagulant agents currently available. Many anticoagulant drugs undergo some extent of renal elimination. There are important considerations for the selection, dosing, and monitoring of anticoagulant drugs for patients with renal impairment due to various pharmacokinetic alterations that may occur. While there are data to help guide dosing and monitoring in adults, evidence regarding renal dose adjustment of many anticoagulant drugs in children are limited. For the clinician, anticoagulation management in children with renal impairment presents unique challenges. In addition to considering overall bleeding risk, the extent of renal impairment may vary by patient, making a one-size-fits-all approach to managing these patients difficult. These factors, combined with limited data, can make managing anticoagulation in children with renal impairment a challenge. Therefore, the focus of this review will be to describe the pharmacokinetics of the following anticoagulants in children with impaired renal function: unfractionated heparin, enoxaparin, dalteparin, rivaroxaban, apixaban, edoxaban, fondaparinux, bivalirudin, argatroban, dabigatran, and warfarin.

静脉血栓栓塞是儿童肾脏疾病发病率和死亡率的常见原因。为了正确治疗和预防这一患者群体的血栓栓塞,熟悉目前可用的多种抗凝剂是很重要的。许多抗凝药物都经历了一定程度的肾脏消除。对于可能发生各种药代动力学改变的肾功能损害患者,在选择、给药和监测抗凝药物时需要考虑一些重要因素。虽然有数据可以帮助指导成人的剂量和监测,但关于许多抗凝药物在儿童中肾脏剂量调整的证据有限。对于临床医生,抗凝治疗的儿童肾功能损害提出了独特的挑战。除了考虑整体出血风险外,肾脏损害的程度可能因患者而异,因此很难采用一刀切的方法来管理这些患者。这些因素,加上有限的数据,可以使管理抗凝治疗儿童肾功能损害是一个挑战。因此,本综述的重点将是描述以下抗凝血剂在肾功能受损儿童中的药代动力学:未分级肝素、依诺肝素、达特帕林、利伐沙班、阿哌沙班、依多沙班、氟达哌啶、比伐鲁定、阿加班、达比加群和华法林。
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引用次数: 0
Mortality from Pulmonary Embolism in Patients with Post-Coronavirus Disease Syndrome. 冠状病毒后疾病综合征患者肺栓塞死亡率
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-10-01 Epub Date: 2025-03-28 DOI: 10.1055/a-2552-1829
Giuseppe Lippi, Camilla Mattiuzzi, Emmanuel J Favaloro
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引用次数: 0
Prediction of Recurrent Venous Thromboembolism and Arterial Cardiovascular Events after Discontinuation of Anticoagulation: The R-VTE-predict and MACE-predict Risk Scores. 停止抗凝治疗后静脉血栓栓塞和动脉心血管事件复发的预测:r - vte预测和mace预测风险评分
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-10-01 Epub Date: 2025-04-09 DOI: 10.1055/s-0045-1807262
Steve R Noumegni, Benjamin Espinasse, Romain Didier, Raphael Le Mao, Claire De Moreuil, Cécile Tromeur, Emmanuelle Le Moigne, Pierre-Yves Le Roux, Francis Couturaud

Patients who had venous thromboembolism (VTE) are not only at increased risk of recurrent VTE but also of major adverse cardiovascular events (MACEs) than the general population. Therefore, the prediction of the risk of these events is important for a tailored prevention and mitigation strategy. We aimed to develop simple scores to estimate recurrent VTE and MACE risks after the discontinuation of anticoagulation in a large cohort of individuals who suffered VTE (EDITH cohort). The primary endpoints were recurrent symptomatic VTE and MACE (composite of non-fatal acute coronary syndrome, stroke and cardiovascular death). Arterial thrombotic event (ATE) exclusively was also considered. Independent predictors of main outcomes were derived from multivariable Cox regression models. Weighted integer points based on the effect estimate of identified predictors were used to derive the final risk scores. A total of 1,999 participants (mean age: 54.78 years, 46.4% male, 43.6% unprovoked VTE) were included in the derivation cohort and 10,000 in the validation cohort (built using bootstrapping). During a median post-anticoagulation follow-up of 6.9 years, recurrent VTE occurred in 29.5% of participants and MACE in 14.8%. Independent predictors of recurrent VTE were male sex, age >65 years, cancer-associated VTE, and unprovoked VTE (vs. transient risk factor-associated VTE). Independent predictors of MACE were age >65 years, cancer-associated VTE, hypertension, renal insufficiency, and atrial fibrillation. The risk of recurrent VTE (moderate vs. low: hazard ratio [HR]: 2.62, 95% confidence interval [CI]: 2.06-3.34; high vs. low: HR: 3.78, 95% CI: 2.91-4.89), MACE (moderate vs. low: HR: 6.37, 95% CI: 3.19-12.69; high vs. low: HR: 12.32, 95% CI: 6.09-24.89), and ATE (based on MACE-predict risk score) increased gradually from the lowest to highest of the respective prediction risk score groups. These results were confirmed in the validation cohort with overall reasonable models' discrimination performance (recurrent VTE C-statistic: 0.62-0.63, MACE and ATE C-statistic: 0.72-0.77). Contemporary simple risk scores based on readily available clinical characteristics can reasonably predict the risk of recurrent VTE and MACE after the discontinuation of anticoagulation. These findings may influence the choice of anticoagulation strategy after the acute phase of VTE and, therefore, need confirmation by further studies.

与一般人群相比,静脉血栓栓塞(VTE)患者不仅VTE复发的风险增加,而且发生主要不良心血管事件(mace)的风险也增加。因此,预测这些事件的风险对于制定有针对性的预防和缓解战略非常重要。我们的目的是建立简单的评分,以估计在大量静脉血栓栓塞患者(EDITH队列)停止抗凝治疗后静脉血栓栓塞和MACE复发的风险。主要终点是复发性症状性静脉血栓栓塞和MACE(非致死性急性冠脉综合征、中风和心血管死亡的组合)。动脉血栓事件(ATE)也被考虑在内。主要结局的独立预测因子来自多变量Cox回归模型。基于所识别的预测因子的效果估计,加权整数点被用来得出最终的风险评分。衍生队列共纳入1999名参与者(平均年龄54.78岁,46.4%为男性,43.6%为非诱发性VTE),验证队列纳入10,000名参与者(使用bootstrapping建立)。在抗凝后平均随访6.9年期间,静脉血栓栓塞复发率为29.5%,MACE发生率为14.8%。静脉血栓栓塞复发的独立预测因素为男性、年龄在65岁以下、癌症相关静脉血栓栓塞和非诱发性静脉血栓栓塞(相对于短暂性危险因素相关静脉血栓栓塞)。MACE的独立预测因子为年龄50 ~ 65岁、癌症相关性静脉血栓栓塞、高血压、肾功能不全和心房颤动。静脉血栓栓塞复发风险(中度vs低):风险比[HR]: 2.62, 95%可信区间[CI]: 2.06-3.34;高vs低:HR: 3.78, 95% CI: 2.91-4.89), MACE(中度vs低:HR: 6.37, 95% CI: 3.19-12.69;高对低:HR: 12.32, 95% CI: 6.09-24.89), ATE(基于mace预测风险评分)在各预测风险评分组中由低到高逐渐增加。这些结果在总体合理模型判别性能的验证队列中得到证实(复发性VTE c统计量:0.62-0.63,MACE和ATE c统计量:0.72-0.77)。基于易于获得的临床特征的当代简单风险评分可以合理预测停药后静脉血栓栓塞和MACE复发的风险。这些发现可能影响静脉血栓栓塞急性期后抗凝策略的选择,因此需要进一步的研究证实。
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引用次数: 0
Lipoprotein(a) and Cerebral Venous Sinus Thrombosis: A Case and the Evidence. 脂蛋白(a)与脑静脉窦血栓形成:1例及证据。
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-10-01 Epub Date: 2025-05-23 DOI: 10.1055/a-2601-9261
Anthony R Sandre, Ali Panju, Sonia S Anand
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引用次数: 0
Nebulized Heparin in 2025: at the Interface between Promising Benefits and the Need for Further Research. 2025年雾化肝素:在前景看好和需要进一步研究之间的界面。
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-10-01 Epub Date: 2025-03-06 DOI: 10.1055/a-2552-1886
Giuseppe Lippi, Emmanuel J Favaloro
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引用次数: 0
Recent Advances in Thrombosis and Hemostasis-Part XII. 血栓和止血的最新进展——第十二部分。
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-10-01 Epub Date: 2025-09-12 DOI: 10.1055/s-0045-1809697
Sam Schulman
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引用次数: 0
Magnetic Resonance Screening for Cerebral Venous Sinus Thrombosis during Treatment with Pegaspargase. Pegaspargase治疗期间脑静脉窦血栓的磁共振筛查。
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-10-01 Epub Date: 2025-04-25 DOI: 10.1055/a-2575-7359
Xiao Zhang, Yang Fu, Hongsheng Wang, Xiaohua Zhu, Yi Yu, Junye Jiang, Ping Cao, Xiaowen Qian, Chen Shen, Xiaowen Zhai

In children with leukemia, cerebral venous sinus thrombosis (CVST) has a significant incidence and mortality rate, which may interfere with the chemotherapy process and lead to long-term neurological complications. However, large studies and population-based data on CVST in children are scarce. This study aims to characterize pediatric CVST associated with pegaspargase (PEG-ASP) and evaluate the significance of magnetic resonance venography (MRV) screening following induction remission in acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL). We present a retrospective cohort of a total of 27 children with CSVT and ALL/LBL. The study covers a 4-year period for MRV screening following induction remission and an 8-year comparison period, involving 716 children treated at the Department of Hematology, Children's Hospital of Fudan University. The detection rate of CVST significantly increased after MRV screening (8.4% vs. 1.6%, p < 0.01). Over half (58%) of the CVST cases were asymptomatic. Male (84% vs. 52%, p = 0.008), immune subtype of T (37% vs. 10%, p = 0.001) and higher initial platelet counts (196.25 ± 140.67 vs. 112.49 ± 115.62, p = 0.02) patients were more likely to develop CVST. The common symptoms were headache (56%), seizures (31%), vomiting (13%), lethargy (13%), coma (6%), hallucinations (6%), and schizophrenia (6%). Symptomatic patients had a higher likelihood of transverse sinus involvement (75% vs. 9%, p = 0.006). Asymptomatic patients had shorter treatment durations (25.5 ± 16.7 weeks vs. 51.6 ± 25.8 weeks, p = 0.02) and fewer long-term complications (50% vs. 0%, p = 0.02). Thromboelastographic amplitude values at 30 minutes after maximum amplitude were significantly higher in symptomatic patients (49.4 ± 13.2 vs. 35.1 ± 8.3, p = 0.01). This study highlights a significant incidence of PEG-ASP-related CVST in children, with MRV screening revealing a notably higher detection rate than previously reported. Most cases were asymptomatic, which demonstrated better prognoses, emphasizing the importance of MRV for early CVST diagnosis after induction remission in ALL/LBL.

在白血病儿童中,脑静脉窦血栓形成(CVST)具有显著的发病率和死亡率,它可能干扰化疗过程并导致长期的神经系统并发症。然而,关于儿童CVST的大型研究和基于人群的数据很少。本研究旨在描述与pegaspargase (PEG-ASP)相关的儿科CVST,并评估急性淋巴母细胞白血病(ALL)和淋巴母细胞淋巴瘤(LBL)诱导缓解后磁共振静脉造影(MRV)筛查的意义。我们提出了一个回顾性队列共27名儿童CSVT和ALL/LBL。该研究包括诱导缓解后的4年MRV筛查和8年的比较期,涉及716名在复旦大学儿童医院血液科治疗的儿童。MRV筛查后CVST检出率(8.4% vs. 1.6%, p = 0.008)、免疫T亚型(37% vs. 10%, p = 0.001)和初始血小板计数(196.25±140.67 vs. 112.49±115.62,p = 0.02)较高的患者更容易发生CVST。常见症状为头痛(56%)、癫痫发作(31%)、呕吐(13%)、嗜睡(13%)、昏迷(6%)、幻觉(6%)和精神分裂症(6%)。有症状的患者更有可能累及横窦(75% vs. 9%, p = 0.006)。无症状患者治疗时间较短(25.5±16.7周vs. 51.6±25.8周,p = 0.02),长期并发症较少(50% vs. 0%, p = 0.02)。有症状患者在最大振幅后30分钟的血栓弹性图振幅值显著高于对照组(49.4±13.2比35.1±8.3,p = 0.01)。本研究强调了儿童peg - asp相关CVST的显著发生率,MRV筛查显示其检出率明显高于先前报道。大多数病例无症状,这表明预后较好,强调了MRV对ALL/LBL诱导缓解后早期CVST诊断的重要性。
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引用次数: 0
Improving Lupus Anticoagulant Detection in Heparinized Patients: An Automated Heparin-Resistant Recalcifying Solution. 改善肝素化患者狼疮抗凝血检测:一种自动肝素耐药重钙化溶液。
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-09-26 DOI: 10.1055/a-2701-0113
Agathe Herb, Nathan Drouin, Amélie Rist, Jordan Wimmer, Laurent Mauvieux, Laurent Sattler
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引用次数: 0
Emicizumab in Acquired Hemophilia A: Benefits in Bleed Control, Safety, and Cost-Effectiveness. Emicizumab治疗获得性血友病A:出血控制、安全性和成本效益。
IF 4.1 2区 医学 Q2 HEMATOLOGY Pub Date : 2025-09-09 DOI: 10.1055/a-2691-6232
Gerard Gurumurthy, Lianna Reynolds, Martin Scott, Elizabeth Davies, Charles Hay, Jecko Thachil
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引用次数: 0
期刊
Seminars in thrombosis and hemostasis
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