<div><h4>Management of pregnancy and delivery in congenital fibrinogen disorders: communication from the ISTH SSC Subcommittee on Factor XIII and Fibrinogen.</h4><i>Casini A, Abdul Kadir R, Abdelwahab M, Manco-Johnson MJ, ... Santoro C, Acharya S</i><br /><AbstractText>Congenital fibrinogen disorders (CFDs) are a heterogeneous group of rare congenital quantitative and/or qualitative fibrinogen deficiencies. The spectrum of molecular anomalies is broad, leading to several subtypes of fibrinogen disorders (ie, afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia). Pregnancy in women with CFDs is a high-risk clinical situation, with an increased tendency for miscarriages, bleeding, and thrombosis. Even though it is well established that management of such pregnancies requires a multidisciplinary approach involving specialists (hematologists and maternal/fetal medicine experts with expertise in the management of inherited bleeding disorders), specific guidelines are lacking. In this International Society on Thrombosis and Haemostasis (ISTH) Scientific and Standardization Committee communication, we aim to propose an expert consensus opinion with literature evidence where available on the strategy for management of pregnancy, delivery, and puerperium in CFDs.</AbstractText><br /><br />Copyright © 2024 International Society on Thrombosis and Haemostasis. Published by Elsevier Inc. All rights reserved.<br /><br /><small>J Thromb Haemost: 01 May 2024; 22:1516-1521</small></div>

<div><h4>Pediatric pulmonary thromboembolism: a 3-year Canadian Pediatric Surveillance Program study.</h4><i>Krmpotic K, Ramsay L, McMullen S, Chan AKC, Plint AC, Moorehead P</i><br /><b>Background</b><br />Pediatric pulmonary embolism (PE) is a rare event associated with significant morbidity and mortality. Awareness of clinical presentation and practices unique to children may aid clinicians in prompt identification and treatment.<br /><b>Objectives</b><br />To describe the incidence, risk factors, clinical presentation, diagnostic and therapeutic practices, and short-term outcomes of pediatric PE.<br /><b>Methods</b><br />We conducted a 3-year national surveillance study through the Canadian Pediatric Surveillance Program. Over 2800 pediatric specialists and subspecialists were contacted monthly from 2020 to 2022 and requested to report all new cases of PE in patients up to 18 years of age. Case-specific data were obtained through voluntary completion of a detailed questionnaire.<br /><b>Results</b><br />Fifty-eight cases (78% female, n = 45) were reported (2.4 cases per million children), with rates highest in adolescents 15 to 18 years (6.6 cases per million). Detailed information, available for 31 (53%) cases, documented at least 1 risk factor in 28 (90%) cases; 24 (77%) patients presented with 2 or more symptoms. Computed tomography pulmonary angiography was used for diagnostic confirmation in 25 (81%) cases. Anticoagulation was initiated in 24 (77%) of 31 cases; fewer than 5 patients underwent thrombolysis or surgical interventions. Of 28 patients who received therapeutic interventions, 8 (29%) experienced treatment-related complications. Fewer than 5 mortalities were reported.<br /><b>Conclusion</b><br />Pediatric PE is a rare event, with female adolescents at the highest risk. Although the presentation is often nonspecific, clinicians should maintain a high index of suspicion, particularly in patients with risk factors and when other diagnoses that may explain symptoms have been excluded.<br /><br />Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.<br /><br /><small>J Thromb Haemost: 01 May 2024; 22:1366-1371</small></div>

<div><h4>DQB1 antigen matching improves rejection-free survival in pediatric heart transplant recipients.</h4><i>Wright LK, Gajarski RJ, Hayes E, Parekh H, Yester JW, Nandi D</i><br /><b>Background</b><br />Presence of donor-specific antibodies (DSAs), particularly to class II antigens, remains a major challenge in pediatric heart transplantation. Donor-recipient human leukocyte antigen (HLA) matching is a potential strategy to mitigate poor outcomes associated with DSAs. We evaluated the hypothesis that antigen mismatching at the DQB1 locus is associated with worse rejection-free survival.<br /><b>Methods</b><br />Data were collected from Scientific Registry of Transplant Recipients for all pediatric heart transplant recipients 2010-2021. Only transplants with complete HLA typing at the DQB1 locus for recipient and donor were included. Primary outcome was rejection-free graft survival through 5 years.<br /><b>Results</b><br />Of 5,115 children, 4,135 had complete DQB1 typing and were included. Of those, 503 (12%) had 0 DQB1 donor-recipient mismatches, 2,203 (53%) had 1, and 1,429 (35%) had 2. Rejection-free survival through 5 years trended higher for children with 0 DQB1 mismatches (68%), compared to those with 1 (62%) or 2 (63%) mismatches (pairwise p = 0.08 for both). In multivariable analysis, 0 DQB1 mismatches remained significantly associated with improved rejection-free graft survival compared to 2 mismatches, while 1 DQB1 mismatch was not. Subgroup analysis showed the strongest effect in non-Hispanic Black children and those undergoing retransplant.<br /><b>Conclusions</b><br />Matching at the DQB1 locus is associated with improved rejection-free survival after pediatric heart transplant, particularly in Black children, and those undergoing retransplant. Assessing high-resolution donor typing at the time of allocation may further corroborate and refine this association. DQB1 matching may improve long-term outcomes in children stabilized either with optimal pharmacotherapy or supported with durable devices able to await ideal donors.<br /><br />Copyright © 2024 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.<br /><br /><small>J Heart Lung Transplant: 01 May 2024; 43:816-825</small></div>