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Background: Invasive orthopaedic interventions (IOI) are often used to control recurrent haemarthrosis, pain and loss of joint function, in males with haemophilia (Factor VIII and Factor IX deficiency). Aim: Identify risk factors associated with IOIs in males with haemophilia enrolled in the Universal Data Collection (UDC) surveillance program from 2000 until 2010. Methods: Data were collected on IOIs performed on patients receiving care in 130 haemophilia treatment centers in the United States annually by health care providers using standardized forms. IOIs included in this study are as follows: 1) synovectomy and 2) arthrodesis or arthroplasty (A/A). Information about potential risk factors was obtained from the preceding UDC visit if available, or from the same visit if not. Patients with no reported IOI at any of their UDC visits were the reference group for the analysis. Multivariate analyses were conducted to identify independent risk factors for synovectomies and arthrodesis/arthroplasty. Results: Risk factors significantly associated with the two IOI categories were age, student status, haemophilia severity, number of joint bleeds within the last 6 months, HIV or hepatitis C (HCV) status. Multivariate analyses showed patients on continuous prophylaxis were 50% less likely to have had a synovectomy and were 40% less likely to have an A/A. Conclusions: This study shows modifiable risk factors, including management of bleeding episodes with a continuous prophylactic treatment schedule are associated with a decreased likelihood of IOIs in males with haemophilia. [ABSTRACT FROM AUTHOR]

Introduction Health-related quality of life ( HRQoL) is reduced among persons with haemophilia. Little is known about how HRQoL varies with complications of haemophilia such as inhibitors and joint disease. Estimates of preference-based HRQoL measures are needed to model the cost-effectiveness of prevention strategies. Aim We examined the characteristics of a national sample of persons with severe haemophilia A for associations with two preference-based measures of HRQoL. Methods We analysed utility weights converted from EuroQol 5 Dimensions ( EQ-5D) and the Short Form 6 Dimensions ( SF-6D) scores from 1859 males aged ≥14 years with severe haemophilia A treated at 135 US haemophilia treatment centres in 2005-2011. Bivariate and regression analyses examined age-group-specific associations of HRQoL with inhibitor status, overweight/obesity, number of bleeds, viral infections, indicators of liver and joint disease, and severe bleeding at the time of the first HRQoL measurement. Results Overall mean HRQoL utility weight values were 0.71 using the SF-6D and 0.78 using the EQ-5D. All studied patient characteristics except for overweight/obesity were significantly associated with HRQoL in bivariate analyses. In a multivariate analysis, only joint disease was significantly associated with utility weights from both HRQoL measures and across all age groups. After adjustment for joint disease and other variables, the presence of an inhibitor was not significantly associated with HRQoL scores from either of the standardized assessment tools. Conclusion Clinically significant complications of haemophilia, especially joint disease, are strongly associated with HRQoL and should be accounted for in studies of preference-based health utilities for people with haemophilia. [ABSTRACT FROM AUTHOR]

Haemophilia and inherited bleeding disorders in newborns and their carrier mothers pose unique challenges. The pattern of bleeding and the causes and risk factors for bleeding are decidedly different than an older child or an adult with haemophilia/inherited bleeding disorder. This document outlines the needs for further research and education, summarizes the state of the art background information and provides guidance regarding research, education and access to care issues in this population. [ABSTRACT FROM AUTHOR]

Background: Before the implementation of donor screening and the development of effective virus-inactivation procedures, persons with hemophilia (PWHs) were at risk of infection with HBV and HCV transmitted through clotting factor concentrates.Study Design and Methods: Data collected from the medical records of a cohort of 2,772 males with hemophilia who resided in six states of the United States were used to examine relations between demographic and clinical characteristics and laboratory markers of past or present infection with HBV and HCV using logistic regression.Results: Test results were available for 60 percent of the cohort. Among those tested, 30 percent were positive for markers of HBV infection and 64 percent for HCV infection. Factors associated with increased odds of positive HBV markers and HCV infection were greater severity of hemophilia, larger amounts of factor use, and HIV infection. Markers of HBV infection persisted in birth cohorts as late as 1992 and those of HCV infections in birth cohorts through 1991. Compared to same-age US males, PWHs born between 1987 and 1989 were more likely to have markers of HBV and HCV infection.Conclusion: PWHs who received clotting factor concentrates before 1990 may be at risk for infection with hepatitis B or hepatitis C and should be tested. [ABSTRACT FROM AUTHOR]