conclusions an hb-driven algorithm, in combination with the “coagulation box” and the early use of clotting factors, could be a simple and effective tool for improving coagulopathy in multiple trauma patients. results over a 12-month investigation period, 123 severe multiple trauma patients needing intensive care therapy were admitted to our trauma center (mean age 48 years, mean iss (injury se...

rare bleeding disorders (rbds) are a heterogeneous group of disorders including different types of coagulation factor deficiencies. the disorders are inherited in an autosomal recessive manner with different frequencies varying from 1:500000 to 1:2000000. patients affected with rbds are presented with a wide spectrum of clinical manifestations ranging from mild to life threatening bleeding diat...

Hereditary combined vitamin K-dependent clotting factors deficiency (VKCFD) is a rare congenital bleeding disorder resulting from variably decreased levels of coagulation factors II, VII, IX and X as well as natural anticoagulants protein C, protein S and protein Z. The spectrum of bleeding symptoms ranges from mild to severe with onset in the neonatal period in severe cases. The bleeding sympt...

Hereditary factor XIII (FXIII) deficiency is a rare autosomal bleeding disorder which can cause life-threatening bleeding. Acquired be immune-mediated or due to increased consumption reduced synthesis. The most commonly used screening test insensitive, and widely quantitative assays have analytical limitations. present study sought validate Technofluor FXIII Activity, the first isopeptidase-bas...

Hereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu disease, is an autosomal dominant disorder of the fibrovascular tissue. It is characterized by the classic triad of mucocutaneous telangiectasias, recurrent hemorrhages, and familial occurrence. The cutaneous manifestation appear clinically as punctuate, linear, or splinter-like telangiectasias of the upper body, oral, and n...

The problem of impaired hemostasis remains relevant even today. Rare bleeding disorders that cause life-threatening in patient are often overlooked by clinicians. blood coagulation a genetically determined group coagulopathies caused deficiency plasma proteins involved hemostasis, as well fibrinogen, prothrombin, factor V (FV), factors and VIII (FV+FVIII), VII (FVII), X (FX), XI (FXI), XII (FXI...

Classic hemophilia, (hemophilia A), is an X-linked hereditary bleeding disorder affecting half of the male offspring of female carriers. Prenatal diagnosis offers an option, namely to restrict abortions to hemophilic fetuses only, and thus retain the chance of bearing normal sons. Recently, the authors have made a prenatal diagnosis of hemophilia A in an obligate carrier with a male fetus at 24...

Abstract It is an autosomal dominant vascular disorder, which has a variety of clinical manifestations, with epistaxis being one of the most common. Many treatment options exist for epistaxis, but with no consensus on which is the method of choice. We describe the case of a patient with hereditary hemorrhagic telangiectasia (HHT) secondary epistaxis with septoplasty managed with synthetic hard ...

It is an autosomal dominant vascular disorder, which has a variety of clinical manifestations, with epistaxis being one of the most common. Many treatment options exist for epistaxis, but with no consensus on which is the method of choice. We describe the case of a patient with hereditary hemorrhagic telangiectasia (HHT) secondary epistaxis with septoplasty managed with synthetic hard graft, wh...

Von Willebrand disease, the most common hereditary bleeding disorder, arises from quantitative or qualitative defect of von Willebrand factor (VWF). The aim of the treatment is to correct the dual defect of hemostasis caused by the abnormal/reduced VWF and the concomitant deficiency of factor VIII (FVIII). The synthetic vasopressin analogue desmopressin is the mainstay of therapy in about 80% o...