Hemophilia B Leyden is an X chromosome-linked bleeding disorder characterized by an altered developmental expression of blood coagulation factor IX. This form of hemophilia B has been found to be associated with a variety of single point mutations in the factor IX promoter region. We now describe a novel point mutation, T-->G at position -21, in two related patients with the hemophilia B Leyden...

BACKGROUND Patients with hemophilia A have low bone density than healthy controls. It is now widely recognized that physical activity and sports are beneficial for patients with hemophilia. OBJECTIVE To compare the effects of mild and moderate intensity treadmill walking exercises on markers of bone metabolism and hand grip strength in male patients with moderate hemophilia A. MATERIAL AND ...

Hemophilia A is a worldwide disorder of coagulation system. It is a male disorder, yet females with hemophilia are rarely seen in communities with high rate of consanguineous marriages. The abnormalities in factor VIII gene transfer as an X-linked pattern in the family, affects as many as one-third of patients who had no family history of abnormality and thus the occurrence of a sporadic mutati...

Hemophilia is the most common congenital coagulative disorder which exposes the patients to many problems because of lack of coagulative factors, higher risk of viral infections and other complications. This study was designed to detect the prevalence of hepatitis B and C infection in hemophiliac patients referring to Kerman Samenalhojaj Center. For this purpose, 97 hemophiliac patients were st...

conclusions based on our results, it seems that there were no cases of obi among chronic hcv-infected patients with thalassemia and bleeding disorders, particularly hemophilia. however, to improve decisions concerning obi screening, especially in transfusion centers, and concerning the use of comprehensive screening methods, more original studies with more precise laboratory techniques and larg...

development of inhibitor to factor viii is the most serious complication of hemophilia therapy. to determine the prevalence of inhibitors in iran hemophilia a patients exposed to blood products, 1280 hemophilia a patients (age range 9 months-84 years) were evaluated. all patients received several blood products such as fresh frozen plasma (ffp), cryoprecipitate, and factor viii. 635 of 1280 pat...

Hemophilia is a common hereditary coagulation blood disorder due to the deficiency activity of clotting factors. divided into two, namely hemophilia A and B. Among all treatments, standard half-life (SHL) extended (EHL) factor replacement products are most commonly used. This study aimed review real-world evidence on comparison SHL EHL. literature search was conducted in PubMed google scholar p...

OBJECTIVES The aim of this study was to assess health-related quality of life in hemophilic children and adolescents, and describe the impact of health status on quality of life. METHODS The study included 45 patients with hemophilia A and B, their ages ranged from 4 to 16 years. Health-related quality of life was assessed by Hemophilia Quality of Life Questionnaire (Haemo-QOL) US-English lon...

Recombinant vectors based on a non-pathogenic human parvovirus, the adeno-associated virus (AAV), have gained attention as a potentially safe and useful alternative to the more commonly used retroviral and adenoviral vectors. AAV vectors are currently in use in Phase I/II clinical trials for gene therapy of a number of diseases such as cystic fibrosis, α-1 antitrypsin deficiency, muscular dystr...

Factor IX is a zymogen enzyme of the blood coagulation cascade. Inherited absence or deficit of the IX functional factor causes bleeding disorder hemophilia B, which requires constant protein replacement therapy. Reviewed herein are the current state in the manufacturing of FIX, improved variants of the recombinant protein for therapy, transgenic organisms for obtaining FIX, and the advances in...