Combined life ‐threatening internal organ bleeding and postpartum hemorrhage associated with acquired hemophilia A
We report a 38-year-old woman who presented in an emergency department with severe postpartum hemorrhage 2  weeks after cesarean section. Her investigation showed an isolated prolongation of partial thromboplastin time (PTT), low factor VIII assay and a factor VIII inhibitor test, resulting in abnormal Bethesda units which consistent with AHA. This case report highlights the importance of early diagnos is and treatment of AHA. With timely and appropriate management, most patients can achieve a good outcome. (Source: Clinical Case Reports)
Source: Clinical Case Reports - January 3, 2024 Category: General Medicine Authors: Chanakarn Kanitthamniyom, Pharit Siladech, Natchaya Polpichai, Maireigh McCullough, Sakditad Saowapa Tags: CASE REPORT Source Type: research

Comparison of posture and muscle stiffness between adolescent boys with haemophilia and age-matched healthy peers: An observational comparative study
CONCLUSION: This study revealed that the ankle, knee, lumbar, and craniovertebral segments' posture angles were changed and the stiffness of the rectus femoris muscle decreased in HP. Postural alterations and reduced rectus femoris stiffness should be considered in management of the musculoskeletal complications of haemophilia.PMID:38160338 | DOI:10.3233/BMR-230185 (Source: Journal of Back and Musculoskeletal Rehabilitation)
Source: Journal of Back and Musculoskeletal Rehabilitation - December 31, 2023 Category: Orthopaedics Authors: Volkan Deniz Nevin Atalay G üzel Bayram Kelle Ali B ülent Antmen Source Type: research

Low-dose Emicizumab prophylaxis in severe haemophilia A patients -a retrospective study bringing new hope for our patients
CONCLUSION: Low-dose Emicizumab offers a cost effective treatment option and can improve access in developing countries. These findings need to be confirmed in a larger and better controlled study.PMID:38160726 | DOI:10.1016/j.jtha.2023.12.023 (Source: Thrombosis and Haemostasis)
Source: Thrombosis and Haemostasis - December 31, 2023 Category: Hematology Authors: Rucha Patil Chandrakala Shanmukhaiah Nithya J Gogtay Puloma Pandey Kirti Patil Farah Jijina Manisha Madkaikar Source Type: research

The under-evaluated impacts of the therapeutic revolution of hemophilia on women and girls
J Thromb Haemost. 2023 Dec 29:S1538-7836(23)00929-7. doi: 10.1016/j.jtha.2023.12.027. Online ahead of print.ABSTRACTThe advent of new treatment options over the last decades has markedly improved the lives of male persons with hemophilia (PwH). However, this therapeutic revolution has not benefited women and girls with Hemophilia (WGH) and symptomatic carriers of the disease to the same extent as their male counterparts. This inequity is primarily due to the exclusion of WGH from clinical trials and a failure to fully recognize their specific treatment needs. Additionally, the indirect impact of innovative therapies, when ...
Source: Thrombosis and Haemostasis - December 31, 2023 Category: Hematology Authors: Cedric Hermans Evelien Krumb Dawn Rotellini Glenn F Pierce Source Type: research

Comparison of posture and muscle stiffness between adolescent boys with haemophilia and age-matched healthy peers: An observational comparative study
CONCLUSION: This study revealed that the ankle, knee, lumbar, and craniovertebral segments' posture angles were changed and the stiffness of the rectus femoris muscle decreased in HP. Postural alterations and reduced rectus femoris stiffness should be considered in management of the musculoskeletal complications of haemophilia.PMID:38160338 | DOI:10.3233/BMR-230185 (Source: Journal of Back and Musculoskeletal Rehabilitation)
Source: Journal of Back and Musculoskeletal Rehabilitation - December 31, 2023 Category: Orthopaedics Authors: Volkan Deniz Nevin Atalay G üzel Bayram Kelle Ali B ülent Antmen Source Type: research

Low-dose Emicizumab prophylaxis in severe haemophilia A patients -a retrospective study bringing new hope for our patients
CONCLUSION: Low-dose Emicizumab offers a cost effective treatment option and can improve access in developing countries. These findings need to be confirmed in a larger and better controlled study.PMID:38160726 | DOI:10.1016/j.jtha.2023.12.023 (Source: Thrombosis and Haemostasis)
Source: Thrombosis and Haemostasis - December 31, 2023 Category: Hematology Authors: Rucha Patil Chandrakala Shanmukhaiah Nithya J Gogtay Puloma Pandey Kirti Patil Farah Jijina Manisha Madkaikar Source Type: research

The under-evaluated impacts of the therapeutic revolution of hemophilia on women and girls
J Thromb Haemost. 2023 Dec 29:S1538-7836(23)00929-7. doi: 10.1016/j.jtha.2023.12.027. Online ahead of print.ABSTRACTThe advent of new treatment options over the last decades has markedly improved the lives of male persons with hemophilia (PwH). However, this therapeutic revolution has not benefited women and girls with Hemophilia (WGH) and symptomatic carriers of the disease to the same extent as their male counterparts. This inequity is primarily due to the exclusion of WGH from clinical trials and a failure to fully recognize their specific treatment needs. Additionally, the indirect impact of innovative therapies, when ...
Source: Thrombosis and Haemostasis - December 31, 2023 Category: Hematology Authors: Cedric Hermans Evelien Krumb Dawn Rotellini Glenn F Pierce Source Type: research

Comparison of posture and muscle stiffness between adolescent boys with haemophilia and age-matched healthy peers: An observational comparative study
CONCLUSION: This study revealed that the ankle, knee, lumbar, and craniovertebral segments' posture angles were changed and the stiffness of the rectus femoris muscle decreased in HP. Postural alterations and reduced rectus femoris stiffness should be considered in management of the musculoskeletal complications of haemophilia.PMID:38160338 | DOI:10.3233/BMR-230185 (Source: Journal of Back and Musculoskeletal Rehabilitation)
Source: Journal of Back and Musculoskeletal Rehabilitation - December 31, 2023 Category: Orthopaedics Authors: Volkan Deniz Nevin Atalay G üzel Bayram Kelle Ali B ülent Antmen Source Type: research

What is the rational for mesenchymal stromal cells based therapies in the management of hemophilic arthropathies?
Hemophilia A and B are rare X-linked genetic bleeding disorders due to a complete or partial deficiency in the coagulation factors VIII or IX, respectively. The main treatment for hemophilia is prophylactic and based on coagulation factor replacement therapies. These treatments have significantly reduced bleedings and improved the patients ’ quality of life. Nevertheless, repeated joint bleedings (hemarthroses), even subclinical hemarthroses, can lead to hemophilic arthropathy (HA). This disabling condition is characterized by chronic pain due to synovial inflammation, cartilage and bone destruction requiring ultimately ...
Source: Osteoarthritis and Cartilage - December 29, 2023 Category: Rheumatology Authors: Alexandre Th éron, Marie Maumus, Christine Biron-Andreani, Nicolas Sirvent, Christian Jorgensen, Danièle Noël Tags: Review Source Type: research

Etranacogene dezaparvovec-drlb gene therapy for patients with hemophilia B (congenital factor IX deficiency)
Volume 23, Issue 12, July - December 2023, Page 1173-1184 . (Source: Expert Opinion on Biological Therapy)
Source: Expert Opinion on Biological Therapy - December 28, 2023 Category: Drugs & Pharmacology Authors: Tro SekayanDana H. SimmonsAnnette von Drygalskia Division of Hematology/Oncology, Department of Medicine, University of California, San Diego, San Diego, CA, USAb Capitol Scientific, Washington DC, USA Source Type: research

FRONTIER1: a partially randomized phase 2 study assessing the safety, PK, and PD of Mim8, a factor VIIIa mimetic
CONCLUSIONS: These data support the continued clinical development of Mim8, and FRONTIER1 has proceeded onto an extension phase.PMID:38142846 | DOI:10.1016/j.jtha.2023.12.016 (Source: Thrombosis and Haemostasis)
Source: Thrombosis and Haemostasis - December 24, 2023 Category: Hematology Authors: Steven R Lentz Pratima Chowdary Lidia Gil Francisco J Lopez-Jaime Johnny Mahlangu Irina Matytsina Anne Louise Nielsen Jerzy Windyga Source Type: research