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To correlate the HLA type and genetic defect with hemophilia A.
One of the most serious complications of treatment in patients with hemophilia A or hemophilia B is the development of an inhibitor, which is an antibody that neutralizes the factor VIII or IX coagulant activity. Up to one fourth of patients with severe hemophilia A develop an inhibitor but at present it is not possible to predict which patients will develop such antibody. The ability to predict an inhibitor development at an individual level would greatly improve therapeutic approach to this serious problem
Observational Model: Case-Only, Time Perspective: Prospective
The Univeristy of Texas Health Science Center at Houston
The University of Texas Health Science Center, Houston
Published on BioPortfolio: 2014-08-27T03:50:33-0400
To identify the causative mutations in previously untreated patients with hemophilia A enrolled in the ReFacto® clinical safety and efficacy study CTN 93-R833-0XX/C9741-28, using two esta...
To collect and analyze data on females with hemophilia so as to better define the difference between the study population and the male population with hemophilia.
To determine the risk factors associated with inhibitor formation in hemophilia A and to study the mechanism of tolerance in the murine hemophilia A model.
To collect and analyze data on female carriers of severe and moderate hemophilia A and B.
To assess efficacy and safety of BeneFix® for prophylaxis in "Short-term" therapy and on demand therapy for all bleeding episodes of subjects with hemophilia B.
The mainstay of treatment of hemophilia A and B is the replacement of the congenitally deficient coagulation factor through the intravenous infusion of specific concentrates (factor VIII, FVIII, in he...
In Norway, boys with hemophilia usually begin treatment after their first bleeding episode. Boys with severe hemophilia usually start prophylactic treatment around 18-24 months. Health professionals a...
Individual pharmacokinetic assessment is a critical component of tailored prophylaxis for hemophilia patients. Population pharmacokinetics allows using individual sparse data, thus simplifying individ...
Hemophilia B (HB) is an inherited bleeding disorder caused by the absence or dysfunction of coagulation factor IX (FIX). A subset of patients who have HB develop neutralizing alloantibodies (inhibitor...
The dental surgical management of a patient with a bleeding disorder can present a challenge to the dental provider. This article reviews the clinical presentation of hemophilia and describes the mana...
A deficiency of blood coagulation factor IX inherited as an X-linked disorder. (Also known as Christmas Disease, after the first patient studied in detail, not the holy day.) Historical and clinical features resemble those in classic hemophilia (HEMOPHILIA A), but patients present with fewer symptoms. Severity of bleeding is usually similar in members of a single family. Many patients are asymptomatic until the hemostatic system is stressed by surgery or trauma. Treatment is similar to that for hemophilia A. (From Cecil Textbook of Medicine, 19th ed, p1008)
The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
A hereditary deficiency of blood coagulation factor XI (also known as plasma thromboplastin antecedent or PTA or antihemophilic factor C) resulting in a systemic blood-clotting defect called hemophilia C or Rosenthal's syndrome, that may resemble classical hemophilia.
Bleeding into the joints. It may arise from trauma or spontaneously in patients with hemophilia.
Stable blood coagulation factor involved in the intrinsic pathway. The activated form XIa activates factor IX to IXa. Deficiency of factor XI is often called hemophilia C.
Within medicine, nutrition (the study of food and the effect of its components on the body) has many different roles. Appropriate nutrition can help prevent certain diseases, or treat others. In critically ill patients, artificial feeding by tubes need t...