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Objectives: This study aimed to assess the reliability of the Hemophilia Early Arthropathy Detection with Ultrasound in China (HEAD-US-C) scale for the knees of severe hemophilia A (SHA) patients and to determine its diagnostic accuracy for assessments of knee-joint lesions in comparison with magnetic resonance imaging (MRI)., Methods: We collected data from 32 knee joints of 21 patients diagnosed with SHA. The knees were evaluated based on the HEAD-US-C scale and the results were compared with the International Prevention Study Group (IPSG) scale. The HEAD-US-C scale was applied independently by two trained ultrasonographers blinded to the MRI results. The IPSG scale was applied independently by two radiologists blinded to the clinical data and ultrasound (US) results., Results: The IPSG and HEAD-US-C scales exhibited good to excellent inter-rater reliability. Additionally, there was good to excellent agreement between the US and MRI results for the detection of knee lesions in SHA patients. The sensitivities of US for joint effusion, synovial hyperplasia, cartilage loss, and bone-surface irregularities in the knees of patients were 92.59, 100, 95.45, and 87.50%, respectively. The HEAD-US-C scale was positively correlated with the IPSG scale., Conclusions: US is important for evaluating knee lesions in patients with SHA and may potentially replace MRI., (© 2023 American Institute of Ultrasound in Medicine.)

Background: Ultrasound (US) has been proven to be reliable in the assessment of early haemophilic arthropathy in the adult haemophilic population, however few studies so far focused on the reliability of US specifically in the paediatric haemophilic population. We were interested if the changing appearance of the growing bone hinders the ultrasonographic evaluation of the pathologic processes caused by haemophilic arthropathy. The aim of the study was to assess the reliability of US for evaluation of haemophilic arthropathy in children in comparison to magnetic resonance imaging (MRI)., Patients and Methods: The study included all children aged 6 years or more with severe haemophilia in the country (n = 10). We assessed their elbows, knees, and ankles bilaterally by US and compared the results to the MRI as the reference standard. Pearson correlation coefficient (r) was used to analyse correlation., Results: The correlation with MRI for the US for the total score was excellent for all joints (r = 0.849 for the elbows, r = 1 for knees, r = 0.842 for ankles). The correlation of scores for specific joint components showed fair, moderate, or excellent correlation for all joint components in all joints. The correlation was the lowest for the evaluation of cartilage and bone in the ankles (r = 0.546 and r = 0.478) and bone in the elbows (r = 0.499)., Conclusions: Our study proved that US using the HEAD-US method performed by paediatric radiologists is a reliable tool for detection and quantification of haemophilic arthropathy in children in comparison to MRI., (© 2022 Domen Plut, Barbara Faganel Kotnik, Luka Pusnik, Peter Slak, Ziga Snoj, Vladka Salapura, published by Sciendo.)

Background: Certain hemophilia patients are unable to cooperate with or afford magnetic resonance imaging (MRI) examinations. The purpose of our study was to explore the value of multislice spiral computed tomography (MSCT) in evaluating hemophilic arthropathy (HA)., Methods: Thirty-eight patients with 73 joints of HA were consecutively selected from January 2016 to May 2018 for this prospective study. All 73 joints were examined by X-ray, CT, and MRI within 2 days. The MRI scores of the joints were determined by the International Prophylaxis Study Group (IPSG) standard. The CT findings were quantified according to the IPSG standard, except for cartilage injury, which was quantified by joint space narrowing using the X-ray Pettersson score. The CT and MRI scores were compared by the paired Wilcoxon signed-rank test. The correlations between the CT score of joint space narrowing and MRI score of cartilage injury and the total CT and MRI scores were analyzed by Spearman rank correlation. The kappa test was used to compare the consistency of CT and MRI scores., Results: MRI was superior to CT based on the scores for small amount of effusion (P < 0.05), synovial hypertrophy and hemosiderin deposition in the mild groups (P < 0.05). The CT and MRI scores were not significantly different for moderate and massive effusion, synovial hypertrophy, and hemosiderin deposition in the moderate and severe groups, bone erosion or cystic changes (P > 0.05), and there was a high degree of consistency between the two scores (kappa > 0.81). The consistency between the Pettersson scores of joint space narrowing on CT and the IPSG scores of cartilage injury on MRI was high (kappa = 0. 774, P < 0.05)., Conclusion: The image scores of MSCT are generally consistent with MRI except for mild synovitis, which can be used as an alternative for the evaluation of HA.

Background: Outcomes of total knee replacement in cases of hemophilic patients are worse than in patients who undergo operations due to osteoarthritis. Previous publications have reported varying rates of complications in hemophilic patients, such as infection and an unsatisfactory range of motion, which have influenced the survival of prostheses. Our retrospective study evaluated the data of hemophilic patients regarding changes in the development of the range of motion., Methods: The data and clinical outcomes of 72 total knee replacements in 45 patients with hemophilia types A and B were reviewed retrospectively. Patients were operated between 1998 and 2013. All of the patients were systematically followed up to record the range of motion and other parameters before and after surgery., Results: The mean preoperative flexion contracture was 17° ± 11° (range, 0°-40°), and it was 7° ± 12° (range, 0°-60°) postoperatively. The mean flexion of the knee was 73° ± 30° (range, 5°-135°) before the operation and 80° ± 19° (range, 30°-110°) at the last follow-up. The mean range of motion was 56° ± 34° (range, 0°-130°) before the operation and 73° ± 24° (range, 10°-110°) at the last follow-up., Conclusions: Statistical analysis suggested that the range of motion could be improved until the 9th postoperative week. The patient should be operated on until the flexion contracture reaches 22° to obtain a contracture < 15° postoperatively or until the contracture reaches 12° to obtain less than 5°. The operation generally does not change the flexion of the knee in cases of hemophilic patients, but it reduces the flexion contracture and therefore improves the range.

Purpose: Increased articular 99m Tc MDP uptake on blood pool imaging (BPI) of patients with rheumatologic conditions is indicative of active inflammatory changes, and has been suggested as a strong predictor of response to radiosynoviorthesis (RSO). In this study, we aimed to assess the value of pretreatment BPI positivity (i.e. scintigraphic-apparent hyperemia) for successful RSO in hemophilic arthropathy., Methods: Thirty-four male patients with painful hemophilic arthropathy underwent RSO after failure of conservative treatment. Treated joints comprised the knee in eight, elbow in five, and ankle in 21 patients. Pretreatment triple-phase bone scintigraphy showed hyperemic joints (pathologic BPI) in 17 patients, whereas 17 patients had no increased tracer uptake on BPI. Response to RSO was evaluated 6 months post-treatment by measuring changes in intensity of arthralgia according to the visual analog scale (VAS), bleeding frequency, and range of motion. The association between hyperemia (pathologic BPI) and treatment outcome was examined using nonparametric tests for independent samples., Results: Clinically evident pain relief occurred in 26 patients (76.5 %), and the mean VAS decreased from 7.7 ± 1.1 to 4.6 ± 2.7 (p < 0.001). Joint bleeding frequency (hemarthrosis) decreased from 4.5 ± 0.6 to 2.1 ± 0.4 during the first 6 months after RSO (p < 0.001). For both parameters (pain relief and bleeding frequency), patients experienced a similar benefit from RSO regardless of pretreatment BPI: arthralgia (p = 0.312) and frequency of hemarthrosis (p = 0.396). No significant improvement was observed for range of motion, but it was significantly more restricted in hyperemic joints both before (p = 0.036) and after treatment (p = 0.022)., Conclusions: Hemophilic arthropathy can be effectively treated with RSO regardless of pre-therapeutic BPI. Patients in whom articular hyperemia is not detectable by scintigraphy may have similar (outstanding) outcomes, and thus should not be excluded from treatment.

Introduction and Objectives: Intra-articular corticosteroid injections are standard of care for managing joint pain secondary to osteoarthritis or rheumatoid arthritis but are rarely used in haemophilic arthropathy. We have introduced and evaluated the efficacy and safety of ultrasound-guided corticosteroid injections for pain relief in patients with haemophilic arthropathy., Patients and Methods: Ultrasound-guided intra-articular injections performed on haemophilia patients at UCSD between March 2012 and January 2016 were analysed. Needle placement and injection (40 mg triamcinolone; 3-5 mL lidocaine) were performed with musculoskeletal ultrasound and Power Doppler. Analysis included patient demographics, joint-specific parameters such as tissue hypervascularity and effusions, pain relief, and procedure-associated complications., Results: Forty-five injections (14 ankles, 13 elbows, 18 knees) were administered in 25 patients. Advanced arthropathy with hypervascularity and/or effusions was present in 91% and 61% of joints, respectively. Ninety-one per cent of injections resulted in pain relief which was significant in 84% (>30% reduction). Median pain score was reduced from 7 of 10 to 1 of 10 (P < 0.001), usually within 24 h. Median duration of pain relief was 8 weeks (range 1-16 weeks). Haemophilia B patients experienced longer periods of relief, and high Pettersson scores were associated with shorter duration of relief. There were no procedure-associated complications. Repeat ultrasound of eight joints within 4 weeks of injection demonstrated nearly complete resolution of hypervascularity., Conclusions: Point-of-care ultrasound enabled intra-articular corticosteroid injections that provided highly effective, safe, and relatively long-lasting pain relief in haemophilic arthropathy. This approach should be used to improve pain management in haemophilic arthropathy., (© 2016 John Wiley & Sons Ltd.)

Joint hemorrhages represent the most common type of bleeding episode in persons with hemophilia, and recurrent hemarthrosis triggers chronic arthropathy, which is the most frequent chronic complication in these patients. In recent years, in the frame of a comprehensive care approach, a growing attention has been given to the periodic assessment of the joint status in hemophilia patients with the aim to identify early arthropathic changes and to prevent the development of a clinically overt arthropathy. Besides clinical examination, X-ray and magnetic resonance imaging (MRI) are currently used to evaluate joint status and to monitor the disease progression in hemophilia. Considering the limitations of X-ray and MRI, growing interest has been given to ultrasound (US) as a possible tool to assess joint status and identify early arthropathic changes in hemophilia patients. In the present review, we summarize major literature evidence on the use of joint US for the evaluation of markers of disease activity (joint effusion and synovial hypertrophy) and of degenerative damages (osteochondral changes) in patients with hemophilia. On the whole, being able to identify the presence of intra- or extra-articular fluid, US examination is the fastest and most reliable technique to identify acute conditions, such as hemarthrosis. In addition, the information on joint involvement provided by US in the patient follow-up may influence treatment decisions on a personalized basis. The use of US as part of a routine clinical examination by hemophilia experts may optimize the diagnostic workflow, avoiding additional costs and long waiting lists for patients referred to imaging departments. In the frame of a comprehensive care approach, US might represent a strategy to early detect and monitor synovial hypertrophy and osteochondral changes in hemophilia, thus extending the clinical examination and helping identify joints to be studied with a second-level examination such as MRI., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Although effective therapies for haemophilia have been available for decades, the prevention and treatment of joint disease remain major clinical concerns for all haemophilia patients. Early identification of joint disease is vital to initiate or modify treatment, and prevent arthropathy. However, there remains a need for more sensitive and accurate methods, which may also detect improvement in patient outcome with new therapies or different prophylaxis regimens. These topics were explored at the Ninth Zürich Haemophilia Forum. A summary of our shared views on the limitations of current assessment methods, and the potential advantages of more recently developed tools, is provided. Ultrasonography enables more frequent routine monitoring and the early detection of joint disease. In addition, serological markers may provide suitable biomarkers of early arthropathy. To prevent arthropathy, in our opinion, prophylaxis is key to prevent joint bleeds and subsequent initiation of the 'vicious circle of joint disease'. However, issues remain, including when prophylaxis should be started, stopped, and if it is efficacious for inhibitor patients. Once joint bleeding has occurred, enhanced on-demand treatment should be considered. For more advanced stages of joint disease, the issues regarding the treatment options available are explored. Radiosynovectomy should be performed to treat chronic synovitis, and may prevent the need for elective orthopaedic surgery (EOS). Ultimately, however, EOS can be considered once all other treatment options have been explored. While, bypassing agents have facilitated the use of EOS in inhibitor patients, a multidisciplinary approach and careful surveillance is required for good patient outcome., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Little objective information exists about musculoskeletal bleeding patterns in haemophilic arthropathy. Bleeding is assumed to be the cause of painful joints or muscles. Clotting factor treatment is provided empirically, but often does not alleviate pain. We hypothesized that perception of pain aetiology is unreliable, and introduced point-of-care high-resolution musculoskeletal ultrasound (MSKUS) to differentiate intra-articular bleeds vs. joint inflammation, and intra-muscle bleeds vs. other regional pain syndromes. To assess painful musculoskeletal episodes in adult haemophiliacs, we used rapid MSKUS, employing grey scale and power Doppler examination. Forty episodes in 30 adult haemophiliacs were evaluated. Thirty three of the 40 episodes were patient-reported as 'bleeding', five as 'arthritis-type' pain and two as 'undecided'. Of the 33 bleeding reports, only 12 were confirmed by MSKUS; the other episodes revealed other pathology. In contrast, three of five perceived arthritis flares were reclassified as bleeds. Similarly, physician assessment was incorrect in 18 of 40 instances. Swelling and warmth were present in approximately half of confirmed bleeding and non-bleeding episodes, and therefore not useful clinically. Few of the painful episodes were symptom controlled at the time of MSKUS. Management changed based on objective imaging findings in >70% of episodes, which resulted in symptom improvement >60% of the time. Significant discrepancies exist between MSKUS findings and patient/physician-perceived pain classification as bleeding or other musculoskeletal symptoms. Current practice of prescribing clotting factor or conservative measures based on pain perception seems inadequate and suggests that point-of-care imaging should be included into modern haemophilia care., (© 2013 John Wiley & Sons Ltd.)

The aim of this study was to develop a simplified ultrasound scanning procedure and scoring method, named Haemophilia Early Arthropathy Detection with UltraSound [HEAD-US], to evaluate joints of patients with haemophilic arthropathy. After an initial consensus-based process involving a multidisciplinary panel of experts, three comprehensive and evidence-based US scanning procedures to image the elbow, knee and ankle were established with the aim to increase sensitivity in detection of early signs of joint involvement while keeping the technique easy and quick to perform. Each procedure included systematic evaluation of synovial recesses and selection of a single osteochondral surface for damage analysis. Based on expert consensus, a simplified scoring system based on an additive scale was created to define the joint status and, in perspective, to offer a tool to evaluate disease progression and monitor the result of treatment in follow-up studies.

Evaluation of prophylactic treatment of haemophilia requires sensitive methods. To design and test a new magnetic resonance imaging (MRI) scale for haemophilic arthropathy, two scales of a combined MRI scoring scheme were merged into a single scale which includes soft tissue and osteochondral subscores. Sixty-one joint MRI's of 46 patients with haemophilia were evaluated by four radiologists using the new and older scales. Forty-six of the joints were evaluated using two X-ray scales. For all MRI scores, interreader agreement and correlations with X-ray scores and lifetime number of haemarthroses were analysed. The interreader agreement intraclass correlation coefficient was 0.82, 0.89 and 0.88 for the soft tissue and osteochondral subscores and the total score, as evaluated according to the new MRI scale, compared to 0.80 and 0.89 as for the older scales. The total score and osteochondral subscore according to the new scale, as well as scores according to the older scales were correlated (P < 0.01) with number of haemarthroses (Spearman correlation 0.35-0.68) and with the X-ray scores (Spearman correlation 0.40-0.76), but no correlation (P > 0.05) was found between the soft tissue subscore of the new MRI scale and the X-ray scores. The new MRI scale is simpler to apply than the older and has similar reader reliability and correlation with lifetime number of haemarthroses, and by separating soft tissue and osteochondral changes it gives additional information. The new scale is useful for analyses of early and moderate stages of arthropathy, and may help to evaluate prophylactic haemophilia treatment., (© 2012 Blackwell Publishing Ltd.)

The functional independence score in haemophilia (FISH) is a performance-based instrument used to assess musculoskeletal function in patients with haemophilia. We aimed to evaluate the functional independence of haemophilia A adolescents and its correlation to radiological joint scores. A cross-sectional study was carried out on 50 adolescent haemophilia A patients. Musculoskeletal function was assessed using the FISH and individual joints were assessed radiologically using the Pettersson score and MRI scale. The mean age of our patients was 16 ± 1.1 with a mean FISH of 23.32 ± 4.69 (range 13-28) and a mean Pettersson score of 2.32 ± 3.09 (range 0-13) for the knees, 1.86 ± 2.67 (range 0-11) for ankles and 1.42 ± 2.17 (range 0-10) for elbows. The mean MRI score for the knees was 3.92 ± 2.74 (range 0-10) while that for ankles was 3.16 ± 2.64 (range 0-10) and for elbows was 2.34 ± 2.63 (range 0-10). There was highly significant correlation between both radiological joint scores and FISH and between degree of factor VIII deficiency and each of FISH, Pettersson score and MRI score. MRI was superior to conventional radiography in detection of subchondral cyst formation and erosions at joint margins. Given the highly significant correlation with both radiological joint scores, FISH appears to be a reliable tool for assessment of functional independence in adolescents with haemophilia A. MRI is more sensitive than conventional radiography in detection of early joint abnormalities., (© 2011 Blackwell Publishing Ltd.)

Haemarthrosis triggers haemophilic arthropathy (HA) because bleeding starts synovitis immediately, damages cartilage and leads to loss of function and disability. The aim of our study was to investigate the capacity of ultrasonography (US) in detecting bleeding and joint damage in HA. The joints of 62 patients (pts) with haemophilia A or haemophilia B were consecutively evaluated and scored (score ranging from 0 to 21) for effusion (E), bone remodelling (BR), cartilage damage (CD), synovial hypertrophy (SH), haemosiderin (H), osteophytes (O), haemarthrosis (Hae), erosion (Er) and fibrotic septa (FS) with US. X-rays [Pettersson Score (PXS)] were performed in 61 patients and clinical evaluation [World Federation Haemophiliac orthopaedic score (WFHO)] was performed in all patients. A total of 20 healthy subjects and 20 patients affected by Rheumatoid Arthritis (RA) were used as controls. Power Doppler US (PDUS) was performed in all patients on the knee, ankle and elbow joints. A total of 83 joints were studied (50 knees; 12 elbows and 21 ankles). US showed effusion in 57 joint, bone remodelling in 62, cartilage damage in 64, synovial hypertrophy in 45, haemosiderin in 39, osteophytes in 30, haemarthrosis in 24, erosion in 5 and fibrotic septa in 3. The X-rays score showed remodelling in 47 joints, narrowing joint space in 44, displacement/angulation in 39, osteoporosis in 42, subchondral irregularity in 44, subchondral cyst formation in 37, osteophytes in 36 and erosions in 25. The US score in healthy subjects was always ≤ 5 (range 0 to 4). In haemophiliacs, 34 of 83 joints showed US score ≤ 5, and 49 US score > 5. Joints with US score ≤ 5 had a low PXS (SRCC = 0.375, P < 0.01) and joints with US score > 5 showed a high PXS (SRCC = 0.440, P < 0.01). A significant correlation between US score and PXS for bone remodelling [Spearman's rho Correlation Coefficient (SRCC) = 0.429, P < 0.01] and for osteophytes (SRCC = 0.308, P < 0.05) was found. The correlation between the US score and number of bleedings in 83 joints was very significant (SRCC = 0.375, P < 0.01). A total of 24 bleeding joints were identified and verified with aspiration of haematic fluid. US may detect bone and cartilage alterations and synovitis. Indeed, PDUS identified bleeding also in asymptomatic joints and was able to show different entity of haemarthrosis. US may be a feasible and reliable tool to evaluate joint modifications in HA., (© 2010 Blackwell Publishing Ltd.)

To evaluate the inter-observer reliability of radiological assessment systems for haemophilic arthropathy, three senior orthopaedic surgeons with expertise in haemophilia independently evaluated a total of 527 joint radiographs of adult haemophilia patients, without any knowledge of the clinical data. This study was the largest study to evaluate the reliability of radiological assessment systems. As for the results, the Arnold-Hilgartner staging system showed moderate reliability (kappa value: κ = 0.44, P = 0.000), and the De Palma grading system and the Pettersson scoring system showed fair reliability (κ = 0.40, P = 0.000) and slight reliability (κ = 0.12, P = 0.000) respectively. As for the reliability of the eight findings in the Pettersson scoring system, three findings, which were 'narrowing of joint space' (κ = 0.70 P = 0.000), 'irregular subchondral surface' (κ = 0.58, P = 0.000) and 'erosion of joint margins' (κ = 0.56, P = 0.000), showed substantial or moderate reliability. Other findings showed fair or less reliability. The traditional radiological assessment systems showed poor inter-observer reliability. Both progressive scales showed higher reliability than the additive scale, and the three findings in the Pettersson scoring system showed good reliability. These results suggested that the progressive scale, including the three reliable radiological findings, might be a more reliable radiological assessment system., (© 2010 Blackwell Publishing Ltd.)

Of the methods used to evaluate haemophilic arthropathy, clinical joint score can only detect advanced and not minor changes, which usually occur in younger patients. In addition, the currently used clinical scores are poorly correlated with the MRI and X-rays scales. In an attempt to address these shortcomings, a modification of Stockholm clinical scale was designed in which elements of clinical information were included. This new scale was applied in 165 joints of 40 patients with haemophilia A and B and the results were statistically analyzed for its capacity to detect early joints alterations. Furthermore, the adjusted score was compared with Pettersson's radiological score and Denver's MRI score, which can detect early signs of arthropathy. The adjusted scale (a) revealed the earliest abnormalities of haemophilic arthropathy and its correlation with the Pettersson and Denver scores is better than those of Stockholm's scale, (b) provided prediction of the Denver score and (c) was simple and safe to be performed and it could easily be repeated.

Although the severity of haemophilic arthropathy is commonly assessed using established radiographic scoring systems, there is limited available information about their inter- and intra-observer reliability. The purpose of the present study was to establish the inter-observer reliability (IEOR) and intra-observer reliability (IAOR) of three different methods available for the classification of haemophilic arthropathy, including the Arnold and Hilgartner classification, a modification to the Arnold and Hilgartner system described by Luck et al., and the classification described by Pettersson et al. Antero-posterior and lateral radiographs of 54 haemophilic joints were included for the analysis. To determine the IEOR for each one of the three radiographic systems, the radiographs were randomly evaluated by four observers, including two orthopaedic surgeons, one orthopaedic resident and one haematologist. For the determination of IAOR, all four reviewers repeated the assessment in a similar fashion, after a period of at least 2 weeks. IEOR and IAOR for the three classification systems was established using kappa (kappa) statistics. A Spearman rank correlation was used to determine the similarities between each reviewer's own interpretative scales. The IEOR was low for the Arnold and Hilgartner system (kappa = 0.35, P < or = 0.001) and the Luck system (kappa = 0.38, P < or = 0.001), but even lower for the Pettersson system (kappa = 0.06, P = 0.1). For the Pettersson system, particularly low kappa values were observed for the presence or absence of osteoporosis (kappa = 0.11, P = 0.0027), enlarged epiphysis (kappa = 0.10, P = 0.0039), erosion of joint margins (kappa = 0.11, P = 0.0018), and joint deformity (kappa = 0.16, P = 0.00001). However, a relatively high Spearman rank correlation for all three scales [r(s) = 0.75 (P < 0.001) for Arnold and Hilgartner system, r(s) = 0.74 (P < 0.001) for the Luck system and r(s) = 0.81 (P < 0.001) for Pettersson system] indicated an overall, general agreement among the reviewers with regard to the severity of the haemophilic arthropathy. There was a moderate IAOR value for both, the Arnold and Hilgartner system (kappa = 0.57, P = 0.00001) and the Luck system (kappa = 0.62, P = 0.00001) with a low IAOR value for the Pettersson system [kappa = 0.22, P = 0.00001). Currently available radiographic scoring systems for haemophilic arthropathy have low inter- and intra-observer reliability rates. Improvements, either through education or modification of the scoring systems, are critical in an era where correlations between clinical and radiographic scores have received significant attention.

Unlabelled: Very limited data are available in the literature on the doses of unwanted radiation that patients receive following treatment with radiosynoviorthesis (RSO)., Objective: The aim of this study was to assess the radiation exposure after RSO with (186)Re colloid in hemophiliacs., Methods: This study involved 12 hemophiliacs who were treated for hemophilic joint disease with 14 RSOs by using (186)Re colloid. Whole-body scintigrams were performed 1, 6, and 24 hours and 3 and 7 days after RSO. Measurements, using a whole-body counter, were done immediately after scintigraphy, with the treated joint protected with a lead shield. The cumulative activity of (186)Re in the body and in the lymph nodes was calculated. The distribution of (186)Re in the body was determined by using the values for small colloids as proposed by the International Commission on Radiological Protection (ICRP) Publication 53. The computer code, OLINDA/EXM (Vanderbilt University, Nashville, TN), was used for the calculation of the internal dose. A constant distance of 1 m between the ankle joint and body organs, and of 0.33 m between the elbow or shoulder joint and body organs, was used to calculate the contribution of gamma radiation to the effective radiation dose., Results: The mean effective dose received by hemophiliacs after RSO with (186)Re colloid was 28 +/- 9 microSv/MBq of the activity injected into the joint. The patients received 0.8-3.7 mSv (1.9 +/- 0.8 mSv) owing to the leakage of (186)Re from the treated joint and its retention in the body. The highest doses were established in the spleen (26.0 +/- 10.7 mGy), the liver (17.6 +/- 7.2 mGy), and red marrow (3.0 +/- 0.8 mGy). The contribution of gamma radiation to the effective dose was less than 0.1 mSv in RSO of the ankle, 0.4 mSv in the elbow, and 0.6 mSv in the shoulder-joint treatment. The activity of (186)Re in the regional lymph nodes was noted in 4 of the 14 treatments. In these cases, the estimated average dose received by individual lymph nodes was 14.7 +/- 1.9 Gy., Conclusions: RSO with (186)Re colloid is a safe treatment method. The effective dose received by patients after RSO by using (186)Re colloid is low, as are the radiation doses to the most exposed organs. If (186)Re is retained in the regional lymph nodes, the lymph node radiation dose would be high.

Arthropathies and joint deformities in patients with severe hemophilia result in prolonged immobilization, reduced physical activity, and predispose them for osteoporosis. This can lead to an increasing tendency of bone fragility and fractures in patients after trivial trauma. The aim of this study was to find out (i) the prevalence of osteoporosis in hemophilia patients and (ii) the association of osteoporosis with hemophilic arthropathy and related restricted physical activity. In this case-control study, 50 consecutive severe hemophilia patients aged between 20 and 50 years were evaluated for osteoporosis with measurement of bone mass density (BMD) by a DEXA scan and values were compared with that of 50 sex matched normal healthy controls. Major joints of the limbs were evaluated to determine the extent of joint damage and related disability. Forty-two patients had severe hemophilia A and 8 patients severe hemophilia B (efficient factor activity < 0.01 U/ml). BMD values (gms/cm(2)) of lumbar spine and left hip of the patients were significantly lower than that of controls (0.825 vs. 0.939; P < 0.0001 and 0.725 vs. 0.938; P < 0.0001, respectively). The incidence of osteoporosis (T score: -2.5 or more) was significantly higher in hemophiliacs. Incidence of fractures in adult life was also significantly higher in hemophiliacs compared to controls (12% vs. 0%). There was statistically significant correlation between joint evaluation scores and BMD of hip, but not with that of the lumbar spine. There was no correlation between Hepatitis-C virus status and BMD of any site. This shows that development of osteoporosis is a significant problem in patients with severe hemophilia in this country. Hence appropriate preventive measures such as early treatment and adequate mobilization, exercises, encouragement to participate in sporting activities, early assessment of bone density, and administration of anti-osteoporotic therapy is recommended., ((c) 2007 Wiley-Liss, Inc.)

Hemophilia is a coagulation disorder characterized by acute hemorrhages into the musculoskeletal system, leading eventually to arthropathy and disability. We investigated the functional loss, namely disability, in hemophiliacs. The clinical and radiological characteristics of joint involvement were also evaluated. There were 31 patients between the ages of 3 and 18 years and 65 involved joints. The knees were the most commonly affected joints followed by the elbows and ankles. There was a positive correlation between the radiological and clinical evaluation scores; however, the clinical evaluation score did not correlate with age. The radiological score increased in conjunction with increasing age of the patients. We observed a significant relationship between the disability score and the clinical evaluation and radiological scores. These observations suggest that hemophilia is a life-long condition, with a high potential for functional disability if not promptly and adequately controlled. The main principle in the treatment of hemophilic arthropathy is the restoration of the patient's lifestyle and mobility with a comprehensive multidisciplinary approach.

We tested the reliability and construct validity of the Compatible magnetic resonance imaging (MRI) scale for the evaluation of haemophilic knees and ankles and compared the diagnostic performance of MRI and plain film radiographs. Sagittal and coronal gradient-echo 1.5-T MR images of knees (n=22) and ankles (n=23) were obtained from boys (age range 4-16 years; mean 11 years) in two centres (Toronto, n=26; Europe, n=19). The MR images were independently read by four blinded radiologists on two occasions. Number of previous joint bleedings and laboratory level of severity of haemophilia were the reference standards for imaging assessment. Both components of the MRI scale demonstrated high inter- and intrareader intraclass correlation coefficients (progressive (P) scale, 0.91 and 0.94; additive (A) scale, 0.81 and 0.92 respectively). The correlation between the osteochondral domain of the MRI scale and patient's age was moderate. Otherwise, correlations between A- and P-scales and clinical laboratory measurements were weak. The areas under the curve (AUCs) used for discrimination of disease severity were similar for the A- and P-scales (AUCs used for mild disease, A-scale, 0.72+/-0.07; P-scale, 0.69+/-0.08; P=0.23; AUCs for severe disease, A-scale, 0.93+/-0.05; P-scale, 0.87+/-0.08; P=0.05). No differences were noted between the AUCs of the MRI and radiographic scales used for discrimination of late osteoarticular changes; MRI scales performed better for discrimination of early changes. In conclusion, both MRI scales demonstrated excellent reliability, poor convergent validity, and moderate and excellent validity for discrimination of mild and severe diseases respectively. Compared with radiographic scores, the MRI scales performed better for discrimination of early osteoarticular changes.

To evaluate joints alterations, we performed clinical examination, X-rays and magnetic resonance imaging (MRI) (Denver score) in 165 joints of 40 children with severe (n, 32) or moderate (n, 8) haemophilia A or B. All investigated joints had a history of more than three bleeds. At evaluation, 25 of 40 haemophilic patients were on prophylaxis for the last 1-8 years (mean: 3.5 years). MRI revealed chronic synovitis in 55.4% and 50% of joints, which were diagnosed, as normal by the clinical scale and plain radiography respectively. Moreover, MRI unmasked more profound alterations than those observed by plain radiography in 70% of cases. Statistical analysis showed that the clinical and Pettersson scores in contrast to the Denver score provide an underestimation of arthritic changes. Besides, Denver score did not provide resolution in differentiating stages of arthropathy, because of its inherent nature; however, a score of 6 expressing severe synovitis seemed to be the cut-off value for the distinction of severe cases. Based on MRI findings we intensified prophylaxis in nine children and initiated it in another nine children. Five children, who were already on prophylaxis complied with our recommendations and eliminated haemorrhages. Finally, three boys with severe haemophilic arthropathy in knees underwent successful chemical synovectomy with rifampicin.

Although prophylactic treatment is advised for all children with severe haemophilia, the optimal regimen is still under discussion. Should all joint bleeds be prevented, or can a limited amount of arthropathy be tolerated in adulthood without loss of quality of life? To answer this question, the effect of haemophilic arthropathy on health-related quality of life (HRQoL) needs to be quantified. In a retrospective study, the effect of arthropathy on HRQoL and socio-economic parameters was assessed in a single-centre cohort of 96 patients with severe and moderate haemophilia with a minimum age of 13 years. Arthropathy was measured by the radiological Pettersson score of the elbows, knees and ankles (maximum: 78 points). HRQoL was assessed by the Short Form 36 (SF36), measuring eight domains of health. Labourforce participation and medical consumption were assessed using a separate questionnaire. Patients were studied at a mean age of 28.6 years (range: 13-54), the mean time between evaluation and the last Pettersson score was 0.4 years (SD: 1.1). The overall median Pettersson score was 13 (range: 0-78). There was a trend towards lower quality of life with increasing Pettersson scores and age, especially in the physical domains of the SF36. An age-adjusted analysis showed that arthropathy had a small but significant effect on HRQoL in the domain of 'physical function' of the SF36, but not on its other domains, or on labourforce participation and medical consumption. Thus suggesting that the SF36 can be used to assess the effects of haemophilic arthropathy, especially in the domain of 'physical function'.

Hemophilia leads to recurrent bleeding episodes in joints resulting in a severe destructive arthropathy in many cases. Staging of hemophilic joint disease is important for identifying the site of bleeding and planning appropriate therapy. However, classification systems based on plain X-ray findings have been insensitive at detecting the earlier, possibly reversible, changes of synovial hyperplasia. They may also underestimate the severity of cartilage damage. Magnetic resonance imaging (MRI) has been shown to more accurately assess a hemophilic joint. Tentative hemophilic arthropathy scales based on MRI findings are being developed. Hopefully, use of these scales will result in more consistent assessment of hemophilic joints and facilitate development of more targeted treatment and thus prevent or delay the later, destructive changes in the articular cartilage and bone.

The majority of bleeding episodes in hemophilic patients occur within the joints. Of these hemarthroses, the knees, elbows, and ankles account for almost 80%. Should the bleeding persist, the synovium starts to hypertrophy and a vicious cycle of chronic synovitis develops, leading to joint destruction. In immature articulation, synovitis causes hypertrophy of the epiphyseal growth plates and significant structural deficiencies may rapidly develop. This stimulus to the growth plates results in bone hypertrophy, leg length discrepancy, and angular deformities. In mature articulation, hemophilia has a major detrimental effect on the joint cartilage. As it progressively exacerbates, joint function deteriorates. Loss of joint space is the most important radiographic finding related to range of motion. As the synovium becomes increasingly scarred, there is gradual conversion from friable hyperemic tissue to fibrotic scar tissue. This process is the natural course of hemophilic arthropathy. A phenomenon in adult hemophilic patients is that their joints, which radiographically appear severely destroyed, seem to function reasonably well for many years.

Hemophilia is a rare disorder, whereby recurrent bleedings into the joint can result in osteoarthropathy. Radiological changes consist of osteoporosis, enlargement of the epiphyses, irregularity of the subchondral bone surface, narrowing of the joint space, cysts, erosions, joint incongruence and joint deformity. The earlier and the more frequent bleedings have occurred that have not been treated adequately, the more of the changes mentioned above are present. In children, osteoarthropathy of the elbow is present in only about 50% of cases, and in the remaining cases the degree is mostly minimal or moderate. Differential diagnosis consists of juvenile rheumatoid arthritis in children, and rheumatoid arthritis and osteoarthritis in adults.

Total knee arthroplasty (TKA) for hemophilic (factor VIII deficiency) arthropathy is a complex and demanding procedure with a high complication rate. However, the long-term benefits have not previously been reported. This study reviews 19 TKAs performed for hemophilic arthropathy that were followed for a minimum of 5.5 years and an average of 9.5 years. At present, 13 knees have good or excellent results, and six knees rate as poor or failures. Those patients with excellent results have maintained good pain relief and function. Four of the six failures were among the first seven arthroplasties performed, when only 80% factor VIII coverage was used during the perioperative period. Since the use of 100% factor VIII coverage was instituted, the failure rate has declined. Ten of the 19 knees suffered complications, including one deep infection, six superficial skin necroses, three nerve palsies, seven postoperative bleedings, and one transfusion reaction. Six of the seven knees operated on under 80% factor VIII coverage had complications. Once 100% factor VIII coverage was instituted, the only complications included one skin necrosis and three postoperative bleedings. The roentgenographic failure rate has remained high with progressive roentgenographic lucencies in 13 of 19 tibial components, associated with component shift in three knees. While these roentgenographic findings have not necessarily correlated with clinical results, they are disturbing and may portend future failures. However, pain relief and improved function are maintained at longer follow-up times. The best results were obtained under 100% factor VIII coverage using a posterior stabilized prosthesis and patellar resurfacing.

This study deals with the distribution and extent of hemophilic arthropathy (HA) in 52 adult patients suffering from severe hemophilia A. A clinical and radiological evaluation was undertaken and the results were compared. The limitations of the currently used radiological scoring system are discussed.

Three patients with cutaneous and/or synovial hemangiomas demonstrated roentgenographic alterations in the knees, simulating hemophilic arthropathy. In 2 cases, unilateral synovial hemangiomas appeared to be present; in the third patient, who had bilateral abnormalities, the Kasabach-Merritt syndrome was evident, resulting in a consumption coagulopathy and hemorrhagic tendency. The pathogenesis of this unusual arthropathy is discussed.

This study deals with the distribution and extent of hemophilic arthropathy in 52 adult patients suffering from severe hemophilia A. A clinical and radiological score-evaluation of knee, ankle and elbow joints was undertaken, 4.7 of 6 examined joints proved to be affected in the third life-decade in the average, the extent of destruction being most pronounced at the knee joints. A rather close correlation of clinical and radiological signs was found to exist only related to the knee joints.

Single or multiple destruction of joints arises from many causes, ranging from the acute destructive process (usually monarticular) in septic joint disease to the chronic, slowly evolving destruction of cartilage and adjacent bone that occurs in osteoarthritis. Medical treatment of a few of these conditions (including rheumatoid arthritis) is reasonably satisfactory if it is initiated early. On the other hand, surgical procedures are indicated if physical and X-ray examination of the involved joints discloses the presence of a significant destructive process involving both articular cartilage and bone. Several very satisfactory such operative techniques, including synovectomy (in the relatively early phases) and prosthetic joint replacement, have been recently developed. These are especially favorable in destructive disease of the hip, knee, or small joints of the hand. The various differential diagnostic, radiographic medical, and surgical procedures are discussed in some detail in this conference.