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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 9 (2003), S. 0 
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Medical imaging of haemophilic joints is important for detecting abnormalities, grading their severity and selecting the appropriate therapy. The plain-film scoring systems for staging joint disease that were developed prior to the availability of magnetic resonance imaging (MRI) are inadequate for planning modern prevention and treatment. MRI is capable of delineating all of the soft tissue findings long before they are evident on plain radiographs. In this paper, an MRI scoring system is presented along with examples of joint effusion, haemarthrosis, synovial hypertrophy, haemosiderin deposition, erosions, cysts and cartilage loss. MRI is a powerful tool in the diagnosis, staging and treatment of patients with haemophilic joint disease.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We hypothesized that magnetic resonance imaging (MRI) scans taken prior to radiosynoviorthesis may be predictive of response to the procedure in persons with haemophilia. Specifically, response would be inversely related to the severity of synovial hyperplasia. Radiosynoviorthesis was administered to 21 joints with recurrent haemorrhage (target joints). A detailed self-report of haemorrhage history, joint evaluation with scoring according to the World Federation of Haemophilia orthopaedic joint and pain scales, plain radiographs, and MRI studies of the joints were performed pre- and post-radiosynoviorthesis. To augment comparison of the MRI findings to those assessed using the Arnold-Hilgartner and Pettersson scales, a provisional MRI scale for evaluation of haemophilic arthropathy was designed. We found the MRI findings prior to the procedure were not predictive of clinical response; independent of the severity of synovial hyperplasia, most joints bled less and showed improvement by the WFH orthopaedic score. There was generally no change in the severity of synovial hyperplasia after the procedure. We conclude that MRI evaluation is not routinely indicated prior to radiosynoviorthesis.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Effective treatment of haemophilic arthropathy requires a detailed evaluation of joint integrity. Methodological assessment of magnetic resonance imaging (MRI) scores are needed to assure reproducibility of measurements when comparing results of clinical trials conducted in different centres. We compared the reliability of two MRI scoring systems for assessment of haemophilic arthropathy: one progressive system that displays the most severe change and one additive system that depicts osteochondral and soft tissue-related changes. A total of 47 1.5 T MRI examinations of knees (n = 21) and ankles (n = 26) of 42 haemophilic boys, age range, 22 months to 18 years, performed at different centres (Toronto, n = 20, Europe, n = 12 and Denver, n = 15) were independently reviewed by four radiologists at two occasions. Twenty-two examinations were from children 〈9 years and 25 from children ≥9. Sagittal and coronal gradient-echo (MPGR, 3D FLASH with fat saturation, GRASS) images were obtained. The MRI examinations of the ankle and knee studies presented with osteochondral abnormalities in 38.5% and 23.8% of the cases respectively. The two scoring systems demonstrated an excellent inter-reader [progressive, 0.88; additive (A, e, s and h components), 0.86] and intra-reader [progressive, 0.92; additive (A, e, s and h components), 0.93] reliability using intraclass correlation coefficients (ICCs). Although ICCs were slightly higher for knees when compared with ankles, and for older children when compared with younger children, all values fell within excellent inter- and intra-reader reliability categories. The two MRI scoring systems demonstrated a comparable reliability. This result constitutes the basis for further development of a combined MRI scoring system for assessment of haemophilic arthropathy, which incorporates progressive and additive components.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  The international MRI expert subgroup of the International Prophylaxis Study Group (IPSG) has developed a consensus for magnetic resonance imaging (MRI) scales for assessment of haemophilic arthropathy. A MRI scoring scheme including a 10 step progressive scale and a 20 step additive scale with identical definitions of mutual steps is presented. Using the progressive scale, effusion/haemarthrosis can correspond to progressive scores of 1, 2, or 3, and synovial hypertrophy and/or haemosiderin deposition to 4, 5, or 6. The progressive score can be 7 or 8 if there are subchondral cysts and/or surface erosions, and it is 9 or 10 if there is loss of cartilage. Using the additive scale, synovial hypertrophy contributes 1–3 points to the additive score and haemosiderin deposition contributes 1 point. For osteochondral changes, 16 statements are evaluated as to whether they are true or false, and each true statement contributes 1 point to the additive score. The use of these two compatible scales for progressive and additive MRI assessments can facilitate international comparison of data and enhance the accumulation of experience on MRI scoring of haemophilic arthropathy.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Routine infusions of factor VIII to prevent bleeding, known as prophylaxis, and other intensive therapies are being more broadly applied to patients with haemophilia. These therapies differ widely in replacement product usage, cost, frequency of venous access and parental effort. In order to address residual issues relating to recommendations, implementation, and evaluations of prophylaxis therapy in persons with haemophila, a multinational working group was formed and called the International Prophylaxis Study Group (IPSG). The group was comprised of haemophilia treaters actively involved in studies of prophylaxis from North America and Europe. Two expert committees, the Physical Therapy (PT) Working Group and the Magnetic Resonance Imaging (MRI) Working Group were organized to critically assess existing tools for assessment of joint outcome. These two committees independently concluded that the WFH Physical Examination Scale (WFH PE Scale) and the WFH X-ray Scale (WFH XR Scale) were inadequately sensitive to detect early changes in joints. New scales were developed based on suggested modifications of the existing scales and called the Haemophilia Joint Health Score (HJHS) and the International MRI Scales. The new scales were piloted. Concordance was measured by the intra-class correlation coefficient of variation. Reliability of the HJHS was excellent with an inter-observer co-efficient of 0.83 and a test-retest value of 0.89. The MRI study was conducted using both Denver and European scoring approaches; inter-reader reliability using the two approaches was 0.88 and 0.87; test-retest reliability was 0.92 and 0.93. These new PT and MRI scales promise to improve outcome assessment in children on early preventive treatment regimens.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford UK : Blackwell Publishing Ltd
    Haemophilia 4 (1998), S. 0 
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background/objectives: haemophilia is a lifelong bleeding disorder associated with significant morbidity. Because of this for almost 25 years there has been a national network of specialized haemophilia treatment centres (HTCs). Despite this, there is little published information about HTC utilization. We chose to study utilization and satisfaction with care received from the Colorado HTC as compared with that received at other nonspecialized sites.Research design: a survey was designed in collaboration between Colorado Department of Public Health and Environment (CDPHE) and the Denver Mountain States Regional Hemophilia Center personnel for telephone administration by CDPHE personnel to all persons with haemophilia (pwh) residing in Colorado in 1994.Subjects: one hundred forty-five persons with haemophilia (77% of those eligible) participated in the survey.Results: the majority of respondents received care from the HTC. Persons less than 21 years of age and those with severe disease were significantly more likely to do so. Satisfaction with care received at the HTC was greater than that received at other sites (P 〈 0.01). Issues of concern were insurer restricted access to the HTC and the lack of haemophilia knowledge of non-HTC providers.Conclusions: if HTCs and other specialty centres are to survive in an increasingly managed care environment, in addition to increased patient satisfaction, data documenting improved patient outcome with specialty centre directed care will be necessary to facilitate referral.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The clinical, plain X-ray and magnetic resonance imaging (MRI) findings were studied in 13 haemophilic joints previously treated with radiosynoviorthesis. 32P had been injected into the joints at a median of 16 years earlier in an attempt to halt recurrent haemorrhage. Prior to 32P injection, the majority of joints demonstrated bone damage evident on plain X-ray, secondary to recurrent haemorrhage. At the follow-up evaluation we found plain X-rays were adequate to identify cysts, erosions and cartilage loss in these very damaged joints. MRI was superior to clinical examination and plain X-ray in identifying synovial hyperplasia and effusions.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford UK : Blackwell Science Ltd
    Haemophilia 6 (2000), S. 0 
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: With the heightened interest in protocols to prevent or treat complications of haemophilia related to recurrent haemarthroses, there is a need for sensitive joint-evaluation tools. The World Federation of Haemophilia (WFH) Physical Joint Examination instrument, which was developed for persons with haemophilia worldwide, is not sensitive enough to detect early structural or functional abnormalities. Therefore, we have expanded the WFH instrument to detect more subtle abnormalities of joint structure and function, and in addition, developed a new scale specifically tailored to the dynamic growth and gait development of children. We compared the original and three new instruments in 43 children with haemophilia. The three new scales all showed better correlation with the WFH pain instrument than did the original WFH physical examination instrument (P 〈 0.01 for each of the new instruments vs. P 〉 0.05 for the WFH instrument). In addition, results of the new child physical examination instrument best conformed to a normal distribution (P=0.35) and this instrument had better overall statistical performance. This instrument should be studied further in prospective, longitudinal clinical trials of young children.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We hypothesized that persons with HIV who perceive themselves as having waning health status may participate in fewer sexual behaviours than HIV-infected persons without HIV-related health problems. The object-ives of this study were to compare health care provider responses with participant responses for health status and for sexual activity and to examine the relationship of HIV-related health status to the sexual behaviour of HIV-seropositive adolescents and young men with haemophilia.A detailed questionnaire designed by researchers from 11 participating US haemophilia treatment sites and CDC personnel was administered to 306 HIV-seropositive young men with haemophilia aged 12–25 years. A health care provider from the haemophilia treatment centre also completed a questionnaire on each respondent.Self-assessment of HIV-related health status was similar to provider assessment of health status. Providers accurat-ely assessed participant sexual activity (overall Kappa = 0.62). Participation in vaginal intercourse and condom use was independent of health status.Following extensive educational efforts, most HIV-seropositive adolescents and young men with haemophilia are aware of the relationship between their HIV seropositivity and health status. Waning health status does not reduce participation in penetrative sexual behaviours or increase use of condoms. HIV prevention efforts should continue for this population.
    Type of Medium: Electronic Resource
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