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Covalent Tolerance Induction to Factor VIII-Prediction of Inhibitors in Hemophilia

10:05 EDT 23rd May 2013 | BioPortfolio

Summary

To correlate the HLA type and genetic defect with hemophilia A.

Description

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

Study Design

Observational Model: Case-Only, Time Perspective: Prospective

Conditions

Hemophilia A

Location

The Univeristy of Texas Health Science Center at Houston
Houston
Texas
United States
77030

Status

Recruiting

Source

The University of Texas Health Science Center, Houston

Results (where available)

View Results

Links

Medical and Biotech [MESH] Definitions

Hemophilia B

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)

Hemophilia A

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.

Factor Xi Deficiency

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.

Hemarthrosis

Bleeding into the joints. It may arise from trauma or spontaneously in patients with hemophilia.

Factor Xi

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.

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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.

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PubMed Articles [ 352 Associated PubMed Articles listed on BioPortfolio]

Pseudotumor of the Mandible as First Presentation of Hemophilia in a 2-Year-Old Male: A Case Report and Review of Jaw Pseudotumors of Hemophilia.

Pseudotumor of hemophilia (PTH) is a rare complication seen in approximately 1-2% of cases of hemophilia. Although much more common in long bones, the pelvis, and small bones of the hands and feet tha...

Assessment of blood-induced arthritis causes friction.

Is a reduced intensity treatment with Rituximab effective in acquired haemophilia A?

Bone Status of Children With Hemophilia A Assessed With Quantitative Ultrasound Sonography (QUS) and Dual Energy X-ray Absorptiometry (DXA).

Recent studies report reduced bone mineral density (BMD) even among young adults and children with hemophilia. Our aim was to assess bone status in children and adolescents with hemophilia with 2 meth...

Another role for the VW molecule.

Summary.  My comments on the implication of the vW molecule in down-regulating the immunogenicity of factor VIII.

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