Background: Heterogeneous mutations in the human coagulation factor IX gene lead to an X-linked recessive bleeding disorder known as hemophilia B. The disease is distributed worldwide with no ethnic or geographical priority. Materials and Methods: The aim of this study was to characterize the factor IX gene mutations in 28 unrelated Iranian hemophilia B patients. Polymerase chain reaction (PCR)...

OBJECTIVE The aim was to overview the oral health aspects in hemophilia patients. MATERIAL AND METHODS An electronic search of Medline (Pub Med), Cochrane, SSCI (Social Citation Index), SCI (Science Citation Index) databases from 1982 to the present, using the following search words: hemophilia, oral health, dental caries, dental caries prevalence, gingivitis, periodontitis, primary dentition...

Hemophilia A and B are severe Xlinked inherited bleeding disorders caused by deficiency of blood coagulation factors (F) VIII and FIX, respectively. Currently, hemophilia is treated with protein replacement therapy using either plasma-derived or recombinant coagulation factors. Although replacement therapy is extremely effective and has significantly enhanced the quality and life expectancy of ...

This work presents a case report of a generalized form of hereditary gin­ gival fibromatosis with hemophilia B as an accompanying disease. In the family of proband, consisting of 28 members, fibromatosis was present in 9 (4 males and 5 females). The pedigree analysis confirmed that gingival fibro­ matosis was transmited through three generations as an autosomal dominant trait. Neither proband, ...

Factor IX (F.IX) is a vitamin K-dependent plasma protein, a deficiency of which results in hemophilia B. A canine model of hemophilia B exists; attempts to use this model for gene transfer experiments or characterization of the hemophilic defect require elucidation of normal canine F.IX structure. We report the isolation and characterization of the coding region for canine F.IX cDNA. Canine F.I...

The X-linked genetic bleeding disorder caused by deficiency of coagulator factor IX, hemophilia B, is a disease ideally suited for gene therapy with genome editing technology. Here, we identify a family with hemophilia B carrying a novel mutation, Y371D, in the human F9 gene. The CRISPR/Cas9 system was used to generate distinct genetically modified mouse models and confirmed that the novel Y371...

A longstanding goal for the treatment of hemophilia B is the development of a gene transfer strategy that can maintain sustained production of clotting factor IX (F.IX) in the absence of an immune response. To this end, we have sought to use lentiviral vectors (LVs) as a means for systemic gene transfer. Unfortunately, initial evaluation of LVs expressing F.IX from hepatocyte-specific promoters...

Hemophilia B is a rare X-linked disorder that may cause dramatic bleeding. Women account for only 3.2% of those clinically affected. The X-linked inheritance frequently delays the diagnosis in women and may expose the patient to an increased risk of adverse events. There is limited experience with these patients during labor and delivery. A 28-year-old primiparous woman with hemophilia B (bleed...

Hemophilia A & B are congenital bleeding disorders affecting 1:10-20,000 population and 1:20 to 40,000 population respectively. Hemophilia represents the prototype of high cost low volume disease. Eighty persons of world hemophilia population lives in financially poor developing countries, where <2% of GDP is usually spent for total health care. In India, with a population of more than 1 billio...