Factor XIII deficiency is a rare cause of early abortion. The obstetrical outcome of four pregnancies in two women with factor XIII deficiency is reported. Both women were treated with substitution therapy using locally-prepared cryoprecipitate. The outcome in these two women demonstrated the need for substitution therapy in early pregnancy leading to an increased chance of obstetrical success.
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Blood Transfus
December 2024
Department of Anesthesiology and Intensive Care Medicine, University Hospital Center Rijeka, Rijeka, Croatia.
CEN Case Rep
January 2025
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
A 69-year-old Japanese man developed abdominal pain, purpura, proteinuria, and hematuria while receiving treatment for pulmonary tuberculosis. A skin biopsy revealed IgA-positive leukocytoclastic vasculitis, and a renal biopsy showed IgA-positive mesangial proliferative glomerulonephritis with crescent formation. Based on these findings, we diagnosed IgA vasculitis with nephritis (IgAVN) and initiated treatment.
View Article and Find Full Text PDFBackground: Germline haplodeficiency (RHD) is associated with thrombocytopenia, platelet dysfunction and predisposition to myeloid malignancies. Platelet expression profiling of a RHD patient showed decreased encoding for the A subunit of factor XIII, a transglutaminase that cross-links fibrin and induces clot stabilization. FXIII-A is synthesized by hematopoietic cells, megakaryocytes and monocytes.
View Article and Find Full Text PDFBMC Cardiovasc Disord
January 2025
Department of Laboratory Medicine, Zhujiang Hospital, Southern Medical University, 253 Industrial Avenue Central, Guangzhou, 510280, P. R. China.
Objective: To establish the reference intervals of plasma Plasminogen, Factor XII activity, and Factor XIII Antigen in healthy adults in Guangzhou.
Methods: A total of 168 young people (75 males and 93 females, aged 18-65 years) who underwent physical examination in Zhujiang Hospital of Southern Medical University from 2020 to 2022 were recruited. Sysmex CS5100 automatic coagulation analyzer and matching reagents were used to detect Plasminogen.
Ital J Pediatr
January 2025
Pediatric Hematology and Oncology, SBU Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey.
Background: Rare bleeding disorders (RBDs) include fibrinogen (Factor I), prothrombin (Factor II), Factor V(FV), combined Factor V and Factor VIII, Factor VII, Factor X, Factor XI, Factor XII, and Factor XIII deficiencies. This group accounts for 3-5% of all factor deficiencies. Different symptoms may occur, ranging from mild or moderate bleeding to serious and life-threatening bleeding, which may not be related to the factor level.
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