Hemophilia and von Willebrand disease together account for the large majority of congenital bleeding disorders. Contemporary management, including development of safer clotting factor concentrates and increased emphasis on long-term follow-up in comprehensive hemophilia centers, has improved both quality of life and longevity for patients with congenital bleeding disorders. In addition to facil...

Bleeding symptoms and clotting activity vary among mutations that alter mRNA splicing of either the factor VIII or factor IX genes. We analyzed splicing mutations in both genes for changes in individual information (R(i), in bits) involving both donor or acceptor sites. Mutations with low or negative R(i) values (<2.4 bits) or significant changes in R(i) (DeltaR(i) > or = 7 bits) exhibited eith...

seconds.2 Therefore, although the PTT measures the over-all time of the intrinsic clotting reactions, in most circumstances it essentially measures the rate of generation of intrinsic prothrombinase. It is now generally accepted that contact with a negatively charged surface such as glass initiates clotting in vitro by activating Hageman factor ( HF, factor XII ) . Activated HF then reacts with...

BACKGROUND Although hemophilia has a potentially high economic impact, there are no published estimates of healthcare costs for this disease in Portugal. The aim of this study was to evaluate costs of treatment and hospital utilization among patients with hemophilia A and B, with and without inhibitors, over a 3-year period in a Portuguese Comprehensive Care Hemophilia Centre. This is the first...

Hemostasis comes from the coordinated activation of platelets and plasma clotting factors to form a platelet fibrin clot. Two processes, primary and secondary hemostasis activation of the clotting cascade id done by collagen for the intrinsic pathway, and the extrinsic pathway is activated by the release of tissue factors from the damaged zone. The two converge ento the common pathway which beg...

Administration of vitamin K-antagonists induces the appearance of abnormal clotting factors in the blood (man, cow) or in the l iver (rat). These abnormal clotting factors (descarboxyfactors) contain glutanfc acid (G1u) residues instead of the 7-carboxyglutamic acid (Gia) residues, which occur in normal clotting factors [1]. In human and bovine plasma -90% of the abnormal proteins consists of d...

Histidine-rich glycoprotein (HRG) circulates in plasma at a concentration of 2 M and binds plasminogen, fibrinogen, and thrombospondin. Despite these interactions, the physiologic role of HRG is unknown. Previous studies have shown that mice and humans deficient in HRG have shortened plasma clotting times. To better understand this phenomenon, we examined the effect of HRG on clotting tests. HR...

The Ca(2+)-phospholipid binding proteins in human placental tissue were investigated with the binding of a placental EDTA extract to liposomes composed of placental phospholipids. A new Ca(2+)-dependent phospholipid-binding protein different from calphobindin I (CPB I) and calphobindin II (CPB II) was isolated from the EDTA extract, and the purification procedure of this protein was established...