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A Comparative Analysis of the Bleeding Profile and Quality of Life Among Women With Hemophilia Genotype Compared to Other Bleeding Disorders.
Cureus
September 2024
Department of Pathology, Post Graduate Institute of Child Health, Noida, IND.
Article Synopsis
- * This study compares the bleeding profiles and quality of life (QOL) of women and girls with hemophilia to those with VWD and rare bleeding disorders, using the ISTH Bleeding Assessment Tool and the EuroQOL questionnaire.
- * Initial findings showed that 80 participants were enrolled, with a median age of 35 for hemophilia carriers and 15.5 for those with diagnosed bleeding disorders, revealing a significant family history of bleeding disorders in both groups.
Management and outcomes of women with low fibrinogen concentration during pregnancy or immediately postpartum: A UK national population-based cohort study.
Acta Obstet Gynecol Scand
July 2024
National Perinatal Epidemiology Unit, Nuffield Department of Population Health, University of Oxford, Oxford, UK.
Article Synopsis
- Pregnant women with fibrinogen levels below 2 g/L are at high risk for severe postpartum hemorrhage and related complications, yet the criteria for identifying those needing fibrinogen therapy are unclear.
- A UK study identified 124 cases of low fibrinogen in pregnant women, mainly linked to postpartum hemorrhage from events like placental abruption or trauma, with a low incidence of inherited conditions.
- The study found high maternal and perinatal mortality rates, including 27 stillbirths and two maternal deaths due to massive hemorrhage, indicating the seriousness of low fibrinogen levels in this population.*
Management of pregnancy and delivery in congenital fibrinogen disorders: communication from the ISTH SSC Subcommittee on Factor XIII and Fibrinogen.
J Thromb Haemost
May 2024
Hemostasis and Thrombosis Center, Northwell Health, New Hyde Park, New York, USA.
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.
View Article and Find Full Text PDFPlacenta accreta spectrum conservative management and coagulopathy: case series and systematic review.
Ultrasound Obstet Gynecol
June 2024
Division of Maternal-Fetal Medicine, Department of Obstetrics, Gynecology and Reproductive Sciences, University of Maryland School of Medicine, Baltimore, MD, USA.
Objective: Management of placenta accreta spectrum (PAS) with the placenta kept in situ aims to preserve fertility and minimize blood loss. However, this method is associated with a risk of coagulopathy and subsequent bleeding. The aim of this study was to evaluate the occurrence and pathophysiology of coagulopathy in cases of PAS managed conservatively.
View Article and Find Full Text PDF[Genetic analysis of a Chinese pedigree affected with Congenital dysfibrinogenemia due to variant of FGG gene].
Zhonghua Yi Xue Yi Chuan Xue Za Zhi
November 2023
Harbin Institute of Hematological Oncology, Harbin First Hospital Affiliated to Harbin Institute of Technology, Harbin, Heilongjiang 150010, China.
Article Synopsis
- - The study aimed to investigate congenital dysfibrinogenemia (CD) in a Chinese family by assessing coagulation deficits and genetic causes.
- - Blood tests revealed reduced fibrinogen activity in the proband and her father, while other family members showed normal results; two genetic variants were found in the FGA and FGG genes, with one variant likely linked to the condition.
- - The inheritance pattern of CD in this family was determined to be autosomal dominant, indicating that the newly identified variant in the FGG gene may play a significant role in the development of the disorder.
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