Hemophilia A, B are X-linked recessive bleeding disorder that typically results from a deficiency of clotting factor VIII (FVIII) and IX (FIX). The severity the disease is determined according to FVIII FIX levels. A have similar symptoms both characterized by bleeding, particularly in large joints such as ankles, knees, elbows. Recurrent eventually causes progressive hemophilic arthropathy. Lif...

Hemophilic arthropathy is the most common chronic complication in patients with hemophilia. The pathogenesis of hemophilic arthropathy involves the inflammatory processes associated with rheumatoid arthritis (RA). Determining the severity and/or progression of joint damage is crucial when evaluating the effect of treatment modalities. Identifying reliable biomarkers in the peripheral blood of p...

Abstract Background Hemophilia B is an X-linked inherited disease, mainly caused by deficiency of factor IX. Severity the disease manifested IX in blood. Case presentation This paper presents our experience cochlear implantation with hemophilia 11-month child profound sensorineural hearing loss. The patient had normal hematological parameters pre-operatively and surgery was uneventful. After 36...

A diagnosis of hemophilia A or hemophilia B begins with clinical assessment of the patient and is facilitated by laboratory testing. The influence of the latter on a diagnosis of hemophilia A or hemophilia B is clear-a diagnosis cannot be made without laboratory confirmation of a deficiency of factor FVIII (FVIII) or factor IX (FIX), respectively. Moreover, the degree of hemophilia severity is ...

Hepatitis C has a negative effect on health-related quality of life (HRQoL). It is not clear whether hepatitis C affects HRQoL of patients with hemophilia. The objective of this study was to assess the effect of hepatitis C virus (HCV) infection on HRQoL in patients with hemophilia. A cross-sectional study was performed among all registered hemophilia patients in the Netherlands. HRQoL was dete...

BACKGROUND AND OBJECTIVES In patients with factor XI(FXI) deficiency the bleeding tendency is poorly correlated with plasma factor levels. The purpose of this study was to evaluate whether or not this discrepancy is also present in a large series of patients from Iran, a previously unexplored ethnic group. DESIGN AND METHODS In 28 FXI - deficient patients bleeding symptoms and their relation ...

Although many examples of unrelated hemophilia A patients carrying identical point mutations in the factor VIII (FVIII) gene have been reported, the clinical phenotype is not always the same among patients sharing the same molecular defect. Possible explanations for this discrepancy include undetected additional mutations in the FVIII gene or coinheritance of mutations at other genetic loci tha...

This study was undertaken to assess the frequency of decreased bone mineral density and its risk factors as well as its impact on the quality of life during childhood among hemophiliac patients. Materials and Methods: Thirty seven children with severe hemophilia A and B, referred to Mofid Children’s Hospital during 2010, were selected. For all patients the joint score, body mass indexes, bone...

this study was undertaken to assess the frequency of decreased bone mineral density and its risk factors as well as its impact on the quality of life during childhood among hemophiliac patients. materials and methods: thirty seven children with severe hemophilia a and b, referred to mofid children’s hospital during 2010, were selected. for all patients the joint score, body mass indexes, bone m...