Stromal cell-derived factor 1 alpha (SDF-1alfa) and cartilage oligomeric matrix protein (COMP): Two potential signature biomarkers of radiological detectable hemophilic arthropathy.

INTRODUCTION: Hemophilia is a rare constitutional bleeding disorder due to a deficiency in Factor VIII or Factor IX. Recurrent hemarthroses, one of the major complications of the disease, lead to hemophilic arthropathy, a disabling condition that requires early diagnosis. Traditionally, clinical examination and plain film radiography have been used to diagnose hemophilic arthropathy. Magnetic resonance imaging (MRI) and ultrasound can be more useful for diagnosing soft-tissue changes. However, but each of these methods has limitations and diagnosis of arthropathy can be delayed. AIM: The aim of this project was to assess plasmatic biomolecules indicative of osteo-cartilaginous damage in patients with hemophilia with or without known arthropathy, in order to improve the diagnosis of this major complication of the disease. METHODS: In this monocentric retrospective study, 40 patients with hemophilia A or B, for whom a plasma sample was available, provided informed consent for further analyses (multiplex immunoassays and ELISA) and collection of relevant clinical information in their medical files. Correlations were sought for between biomarkers of interest and the severity of joint lesions assessed according to Pettersson's radiologic score. RESULTS: Two biomarkers were identified, respectively SDF-1α and COMP. Their plasmatic levels were significantly increased in patients with arthropathy compared to controls and patients without arthropathy. These values correlated significantly with the Pettersson score in patients under regular prophylaxis. CONCLUSION: Two plasma biomarkers have been identified that could help assess the presence and severity of hemophilic arthropathy.

The utility of standing knee radiographs for detection of lipohemarthrosis: comparison with supine horizontal beam radiographs.

OBJECTIVES: Lipohemarthrosis is a key finding in acute trauma patients and indicates an intra-articular fracture. The horizontal beam lateral radiography with supine position is known to be the best technique to demonstrate knee lipohemarthrosis. Our main purpose was to compare the sensitivity of supine and standing lateral knee radiographs to detect lipohemarthrosis. METHODS: In our retrospective study, consecutive patients with lipohemarthrosis on computed tomography of the knee between October 2019 and September 2021 were included. Fractured bone, the presence of lipohemarthrosis, and image quality in both standing and supine anteroposterior and lateral knee radiographs were evaluated. Interobserver reliability of the three observers was calculated. Fisher exact chi-square and z-proportion tests were used to compare lateral and anteroposterior knee radiographs. Krippendorff's Alpha and Kappa coefficients were used for inter-observer agreement. RESULTS: A total of 61 patients (38 men [62.3%], 23 women [37.7%]; mean age, 43 years ± 17 [standard deviation]) were included. The most common type of fracture was isolated tibial fractures (n = 32; 52.5%). The sensitivity of showing lipohemarthrosis of standing lateral knee radiographs (95.5%) was higher than supine lateral radiographs (38.5%) (p < 0.001). While non-optimal image quality did not affect lipohemarthrosis detection on lateral radiographs (p > 0.99), it caused a significant decrease in the diagnosis of lipohemarthrosis on anteroposterior radiographs (p = 0.036). We found a good-excellent interobserver agreement in lipohemarthrosis detection. CONCLUSIONS: Standing lateral radiographs have higher sensitivity than supine lateral radiographs in detecting lipohemarthrosis and are beneficial for detecting lipohemarthrosis which indicates the presence of occult-evident intraarticular fracture in patients with knee trauma. CLINICAL RELEVANCE STATEMENT: Standing lateral knee radiographs offer a useful method for reducing the misdiagnosis of the occult intra-articular fractures by showing the fat-fluid leveling more clearly. Its advantages may be more prominent when the advanced imaging modalities are limited. KEY POINTS: • Fat-fluid level (lipohemarthrosis) is an important radiographic sign to assess patients with acute trauma. It almost always indicates an intra-articular fracture. • Our retrospective study results support that lipohemarthrosis sign could be observed more frequently in standing lateral knee radiographs than in supine lateral radiographs. • Knee trauma patients, when available, should be evaluated with standing lateral radiographs for the diagnosis of lipohemarthrosis.

Haemophilic arthropathy: basic protocols for clinical examination and imaging.

In haemophilia, screening protocols in the prevention and treatment of common lesions still require unification of criteria. Patients with haemophilia seek medical consultation exclusively for two reasons: because they have requested an appointment for a routine check-up (1-2 times a year in case of severe haemophilia) or because they have developed acute bleeding that requires treatment. The purpose of this paper is to emphasize the importance of an early differential diagnosis of joint damage and to review the techniques that allow an effective evaluation. The World Federation of Haemophilia recommends the 'Primary Prophylaxis' treatment modality, and today, severe haemophilia patients adhering to that factor VIII/IX therapy have significantly reduced common injuries: haematomas, haemarthrosis, synovitis, and haemophilic arthropathy. The basic protocols and minimum data for the control of musculoskeletal health are described. In summary, the primary goal of the haematologist-led multidisciplinary care team treating patients with haemophilia is likely to restore and/or preserve joint and musculoskeletal health, which is essential to promoting quality of life. Appropriate factor replacement regimens are required to prevent bleeding, these should be combined with physical activity and a physiotherapy program, in accordance with the recommendations of the World Health Organization for the general population.

18 F-FDG PET/CT imaging for haemophilic arthropathy compared with clinical, radiological and power Doppler sonographic characteristics of 20 haemophilia patients.

INTRODUCTION AND OBJECTIVES: Repeated joint bleeding in haemophilia patients may lead to haemophilic arthropathy with marked inflammation and synovitis. This study investigated the potential of 18 F-fluorodeoxyglucose positron emission tomography-computed tomography (18 F-FDG PET/CT) as a novel diagnostic method for haemophilic arthropathy. MATERIALS AND METHODS: We recruited 20 adult haemophilia patients who reported history of hemarthroses in the shoulder, elbow, hip, knee, or ankle joints. All joints were assessed by power Doppler ultrasonography and radiography, and graded by hyperaemia score and Pettersson score, respectively. Joint pain was evaluated by visual analogue score (VAS). Range of motion (ROM), Haemophilia Joint Health Score (HJHS) and annual joint bleeding rate (AJBR) were recorded. Finally, all participants had whole-body 18 F-FDG PET/CT, and maximum standardized uptake value (SUVmax) of the joints being studied was measured. RESULTS: Thirteen patients had severe haemophilia, and seven had moderate haemophilia. The mean age was 36 years. PET SUVmax showed significant correlations with VAS, ROM, Pettersson score, hyperaemia score, HJHS score and AJBR in all large joints except hip. Joints with pain, hyperaemia and radiographic changes were found to have higher SUVmax than those without these features. Using Youden's index, the optimal cut-off value for early radiographical arthropathy was found to be between 1.9 and 2.0. CONCLUSION: Our study indicates that 18 F-FDG PET/CT imaging correlated well with various conventional diagnostic techniques. It also demonstrated high sensitivity and specificity for early radiographic arthropathy. 18 F-FDG PET/CT imaging may quantitatively evaluate disease activity of most large joints in a single examination and help detect early haemophilic arthropathy.

The Correlation Between the HEAD-US-C Score and HJHS in Hemophilic Arthropathy of the Knee.

OBJECTIVES: We aimed to discuss the correlation between the Hemophilia Early Detection Ultrasound in China (HEAD-US-C) score and the Hemophilia Joint Health Score version 2.1 (HJHS 2.1) of the knee joint in patients with hemophilia. METHODS: We included 70 male patients with hemophilia admitted to The Second Hospital of Shanxi Medical University; the patients' bilateral knee joints were evaluated using the HEAD-US-C score and HJHS. We analyzed factors influencing hemophilia arthropathy of the knee and examined the correlation between the HEAD-US-C score and HJHS. RESULTS: The joint injury severity was positively correlated with age and the number of bleeds (P < .001). Further, the HEAD-US-C score and HJHS differed according to the severity (both P < .001), but not type (P = .163 and P = .283, respectively), of hemophilia. There was a significant correlation between the HEAD-US-C score and HJHS (P < .001). CONCLUSIONS: Overall, all joint lesions observed on ultrasound corresponded to clinical joint functional abnormalities. Therefore, the HEAD-US-C is important for hemophilic arthropathy evaluation and is useful in explaining abnormal joint function.

The value of radiosynoviorthesis for treatment of chronic synovitis in hemophilic joint disease.

Chronic synovitis is contributing to the development of arthropathy in hemophilia A and B. In most patients with severe and moderate hemophilia, during lifetime, joint damage progresses despite early prophylaxis and intense treatment with coagulation factor concentrates. Recurrent hemorrhages into the joints and subclinical bleeding lead to chronic inflammation of the synovium, neoangiogenesis and remodeling, sustaining a vicious circle of bleeding-remodeling-bleeding and progression of osteochondral damage. Imaging techniques including ultrasound and MRI are able to early visualize synovitis and osteochondral changes. Early detection and sustained therapy of synovitis are important preconditions to prevent further deterioration of joint status. Chronic synovitis requires intensified substitution of coagulation factors and concomitant analgetic, antiphlogistic and physical therapy. The value of early radiosynoviorthesis (RSO) as effective method to control ongoing synovitis is discussed here. RSO is recommended as first choice therapy in case of persistant chronic synovitis, recorded in both national and international guidelines.

Patient-derived assessment tool using musculoskeletal ultrasound for validation of haemarthrosis.

INTRODUCTION: Haemophilia patients experience painful joint episodes which may or may not be associated with haemarthrosis. We sought to validate a questionnaire developed by the Canadian Haemophilia Society using point-of-care musculoskeletal ultrasound (POC MSKUS) to confirm haemarthrosis. METHODS: The questionnaire comprised of 20 questions (10 each associated with haemarthrosis and arthritis pain) and was administered to adult haemophilia patients reporting to the Haemophilia Treatment Centre (University of California San Diego). We confirmed the presence (or absence) of haemarthrosis using POC MSKUS [Joint Activity and Damage Exam (JADE)]. We fitted univariate and multivariate generalized estimating equations to identify symptoms associated with haemarthrosis. RESULTS: We evaluated 79 painful episodes in 32 patients [median age = 38 years (range 21-74)]. POC MSKUS detected haemarthrosis in 36 (46%) episodes. The strongest predictor for haemarthrosis pain was 'like a balloon swelling with water' (odds ratio [OR] 2.88 [CI .68;12.10]); 'no feeling of sponginess with movement' (OR .24[CI .07;.76]) was the strongest for arthritic pain. We identified four questions with the strongest OR for differentiating haemarthrosis pain from arthritic pain to develop an algorithm for haemarthrosis prediction. Answering these questions in "yes/no" fashion yielded estimates of the probability of haemarthrosis CONCLUSION: Objective diagnosis of haemarthrosis by MSKUS facilitated the development of a symptom-based prediction tool for diagnosis of haemarthrosis. The tool requires further validation and will be particularly helpful in situations where MSKUS is not readily available.

Comparison of the clinical and ultrasound evaluation of haemophilic arthropathy in children.

BACKGROUND: Joint ultrasound examination using the HEAD-US method in the detection of early arthropathy is poorly studied in our country. OBJECTIVE: To compare the clinical and ultrasound evaluation of the joints in haemophilia. METHOD: Longitudinal, prospective and descriptive study with paediatric patients with haemophilia A and B evaluated with the HJHS 2.1 scale and ultrasound with a linear transducer of 8 to 12 MHz. Elbows, knees and ankles joints were evaluated bilaterally, with HEAD-US protocol. RESULTS: 69 paediatric patients were included of which 48 with severe haemophilia A (weight: 40.1 kg). On the HJHS scale, a greater involvement was observed in the left knee (0.49), and less in the right ankle (0.05). With the HEAD-US scale, the most affected was the right knee (0.78). There is a significant relationship in the involvement of the right knee evaluated with the HEAD-US scale in the presence of inhibitor. CONCLUSIONS: Weight above the 50th percentile is an independent risk factor for joint bleeding complications, while age and type of haemophilia do not appear to be related. The HEAD-US method is a useful and accessible tool for early detection of arthropathy and hemarthrosis.

ANTECEDENTES: La exploración articular por ultrasonido mediante el método HEAD-US en la detección de la artropatía temprana ha sido poco estudiada en nuestro país. OBJETIVO: Comparar la evaluación clínica y por ultrasonido de las articulaciones en niños con hemofilia. MÉTODOS: Estudio longitudinal, prospectivo y descriptivo con pacientes pediátricos con hemofilia A y B valorados con la escala HJHS 2.1 y ultrasonido con transductor lineal de 8 a 12 MHz. Se evaluaron las articulaciones de codos, rodillas y tobillos de forma bilateral, con el método HEAD-US. RESULTADOS: Se incluyeron 69 pacientes; de ellos, 48 con hemofilia A grave (peso: 40.1 kg). En la escala HJHS se observó mayor afectación en la rodilla izquierda (0.49) y menor en el tobillo derecho (0.05). Con la escala HEAD-US, la más afectada fue la rodilla derecha (0.78). Existe una relación significativa en la afectación de la rodilla derecha evaluada con la escala HEAD-US en presencia de inhibidor. CONCLUSIONES: El peso superior al percentil 50 es un factor de riesgo independiente de complicaciones por sangrado articular, mientras que la edad y el tipo de hemofilia no parecen relacionados. El método HEAD-US es una herramienta útil y accesible para la detección temprana de artropatía y hemartrosis.

Value of Contrast-enhanced Ultrasound in Evaluating Synovitis and Predicting Recurrent Joint Bleeding of Hemophilia.

OBJECTIVE: Contrast-enhanced ultrasound (CEUS) is advantageous for evaluating microcirculation, and has been applied to assess arthritis in previous studies. However, CEUS examinations have not been studied for hemophilia arthritis. Hemophilia arthritis is different from other arthritis, because it is induced by spontaneous joint bleeding. Hence, CEUS may have special value in evaluating hemophilia arthritis. The present study assessed the value of CEUS in evaluating synovial hypertrophy and predicting recurrent joint bleeding in severe hemophilia A patients. METHODS: From August 2016 to January 2017, 81 severe hemophilia A patients, who were referred to our hospital for ultrasound joint assessment with conventional ultrasound, were enrolled. Among these 81 patients, 46 patients consented for CEUS examinations on the same day. RESULTS: Compared to color Doppler flow imaging (CDFI), four more joints presented with a blood flow signal under CEUS mode. In addition, the synovial hypertrophy measured by CEUS was thicker than that measured by conventional ultrasound. The ultrasound scores (including the total grey-scale ultrasound score, joint effusion/hemarthrosis, synovial hypertrophy, CDFI semi-quantitative score, and CEUS semi-quantitative score) were significantly higher in the joint bleeding group than in the no joint bleeding group (P<0.05). Furthermore, these ultrasound scores were positively correlated with the joint bleeding frequency, and had the highest correlation with the CEUS score (r=0.620, P<0.05). CONCLUSION: CEUS can more accurately assess the degree of synovial hypertrophy and vascularization, and diagnose synovitis, when compared to conventional ultrasound. In addition, CEUS appears to be essential for evaluating the possibility of recurrent joint bleeding, and providing more reliable evidence for individualized treatment.

Joint status of patients with nonsevere hemophilia A.

BACKGROUND: Joint bleeding in hemophilia may eventually lead to joint damage. In nonsevere hemophilia, joint bleeds occur infrequently. Currently, knowledge on the joint status of patients with nonsevere hemophilia using objective imaging is limited. OBJECTIVE: To investigate the joint status in patients with nonsevere hemophilia A. METHODS: This cross-sectional study included patients with nonsevere hemophilia A aged 24-55 years. Joint status was assessed by magnetic resonance imaging (MRI) of the elbows, knees, and ankles and International Prophylaxis Study Group (IPSG) scores were calculated. Lifetime joint bleeding history was collected from medical files. The contribution of factors to joint outcome was explored using multivariable linear regression analysis. RESULTS: In total, 51 patients were included, of whom 19 (37%) had moderate and 32 (63%) had mild hemophilia. Patients had a median age of 43 years (interquartile range [IQR] 32-50), a median factor VIII activity of 10 IU/dl (IQR 4-16) and a median annual joint bleeding rate (AJBR) of 0.0 (IQR 0.0-0.2). Soft-tissue changes (IPSG subscore > 0) in the elbows, knees, and ankles were present in 19%, 71%, and 71% of patients, respectively. Osteochondral changes (IPSG subscore > 0) in the elbows, knees, and ankles were present in 0%, 20%, and 35% of patients, respectively. In 14% of bleed-free joints, hemosiderin depositions were observed. Age and AJBRs were most strongly associated with the IPSG score. CONCLUSION: This study demonstrates that a substantial proportion of adults with nonsevere hemophilia has joint changes on MRI despite low joint bleeding rates.

Agreement Between Clinical Examination and Magnetic Resonance Imaging in Acute Knee Trauma With Hemarthrosis.

OBJECTIVE: Hemarthrosis after knee trauma often indicates serious joint injury. Few studies have evaluated agreement between clinical examination and findings from magnetic resonance imaging (MRI). We aimed to describe the agreement between acute clinical examination and subacute MRI findings after acute knee trauma with hemarthrosis and the importance of the subspecialty of the examiner. DESIGN: Longitudinal cohort study. Agreement with MRI findings was evaluated by logistic regression. SETTING: Helsingborg hospital. PATIENTS: Thousand one hundred forty-five consecutive patients with hemarthrosis after knee trauma. INTERVENTIONS: Clinical examination and MRI. MAIN OUTCOME MEASURES: agreement between clinical examination and findings from MRI. We considered the radiologist's report as the gold standard. RESULTS: Median time (25th, 75th percentile) from injury to clinical examination was 2 (1, 7) days, and from injury to imaging was 8 (5, 15) days. The overall sensitivity and specificity of clinical examination versus MRI for major ligament injury or lateral patella dislocation (LPD) were 70% [95% confidence interval 67-73) and 66% (61-72), respectively. Orthopedic subspecialist knee had the highest agreement with anterior cruciate ligament rupture (adjusted odds ratios were 1.7 (95% confidence interval 1.2-2.3), 1.9 (1.2-3.0) and 5.9 (3.7-9.5) for orthopedic trainees, orthopedic subspecialists other, and orthopedic subspecialist knee, respectively]. For other ligament injuries and LPD, we did not find statistically significant differences. CONCLUSIONS: Clinical diagnosis after acute knee injury is relatively unreliable versus MRI findings even when performed by orthopedic specialists. However, the agreement is improved when the examination is performed by an orthopedic knee subspecialist.

Clinical assessment and point of care ultrasonography: How to diagnose haemophilic synovitis.

BACKGROUND: The ability of clinical tools to identify early joint changes is limited. Synovitis is a fundamental finding in understanding haemophilia activity and the response to its therapies; thus, there is a need for sensitive methods to better diagnose subclinical synovitis early. PURPOSE: Our aim was to compare the frequency with which clinical assessment and ultrasound detected synovial hypertrophy in the most frequently affected joints in patients with haemophilia (elbows, knees and ankles). METHODS: We analysed patients with haemophilia older than 16 years who came to the haemophilia centre for routine follow-up. From the clinical assessment carried out in the consultation, the swelling, pain and history of haemarthrosis were evaluated and compared with the findings of synovial hypertrophy detected by ultrasound. This comparison was also analysed independently for elbows, knees and ankles. RESULTS: A total of 203 joints of 66 patients with haemophilia (mean age 34 years), most of them on secondary, tertiary prophylaxis or on demand treatment, were included. In joints with swelling, pain and history of haemarthrosis, 78% of the joints showed synovial hypertrophy on ultrasound. However, in joints with no swelling, no pain and no history of haemarthrosis, 40% presented subclinical synovial hypertrophy on ultrasound. This percentage was higher in elbows than in knees and ankles. CONCLUSION: In adults with haemophilia, physical examination and point-of-care ultrasound study provide complementary data on their joint disease. However, without ultrasound, the ability to detect subclinical synovitis is considerably reduced, especially in the elbows.

Treatment Individualization Using Pharmacokinetic Studies and Joint Ultrasound Imaging in Pediatric Patients With Hemophilia.

Hemophilia is characterized by bleeding diathesis, primarily affecting the joints. Prophylactic use of missing factor aims at limiting the number of bleeds and, in the long term, the risk of permanent joint damage. However, standard prophylactic regimens are usually applied empirically, not adjusting for variations in bleeding phenotype or drug metabolism. Aim of the present study was to evaluate the need for individualizing prophylaxis, with guidance of pharmacokinetic (PK) studies and joint ultrasound in a setting of everyday clinical practice. To evaluate adequacy of applied regimens, joint status was assessed using the Hemophilia Joint Health Score as well as ultrasound imaging, while PK studies were performed using the Web-Accessible Population Pharmacokinetic Service-Hemophilia. Imaging results were consistent with early joint damage in a large proportion of pediatric patients, whereas PK measures were indicative of inadequate prophylaxis in many cases-despite the limited number of bleeds reported by patients. The study revealed the need for prophylaxis adjustment in the majority of patients. Real world data confirm that traditional prophylaxis is often unable to achieve therapeutic goals, while an individualized approach, guided by the use of novel modalities, may be of great benefit to young hemophilia patients.

Extra-Articular Nonhemophilic Hemosiderotic Synovitis of the Hand: A Case Report.

Hemosiderotic synovitis is a rare distinctive type of synovial proliferative disorder. Hemosiderotic synovitis develops in men with hemophilia who are at risk of hemarthrosis, especially of the knees, associated with severe degenerative articular cartilage disease. It appears to be exceedingly rare in individuals without hemophilia and has been reported in case reports. We describe a case of trauma-related nonhemophilic hemosiderotic synovitis, located in the hand in an otherwise healthy young male construction worker, with no systemic illness, inflammatory arthritis, or any bleeding disorder. We report the first case of nonhemophilic hemosiderotic synovitis located in the hand. To the best of our knowledge, a case of extra-articular nonhemophilic hemosiderotic synovitis has not been previously described in the literature.

Treatment of Recurrent Hemarthrosis Following Total Knee Arthroplasty Using Surgical Interventions.

BACKGROUD: Recurrent hemarthrosis following total knee arthroplasty (TKA) is a rare complication. Its pathophysiology and standard treatments have not yet been established. In this study, we report 7 cases of recurrent hemarthrosis after TKA in which failure of the initial conservative treatment was followed by angiographic embolization; in 1 of the 7 cases, arthroscopic electrocauterization was also performed after treatment failure with selective embolization. METHODS: From January 2015 to May 2018, 7 patients visited our hospital due to recurrent hemarthrosis after TKA. Their medical records and serologic test results were reviewed to check for the presence of any bleeding disorder and history of anticoagulant use. Implant malalignment and instability were checked using X-ray. In all cases, the conservative treatment failed, so interventional angiography with selective embolization was performed, which was also followed by arthroscopic electrocauterization if the outcome was unsatisfactory. RESULTS: The interval between TKA and the onset of hemarthrosis ranged from 3 to 76 months (average, 34.1 months). There was no coagulopathy and instability. All patients underwent conservative treatment at an interval of 4.3 months and the rate of relapse was 3.1 on average. On the interventional angiography, 6 cases showed vascular blush, and 1 case had pulsatile bleeding. The average duration for interventional angiography was 90.9 minutes. The average length of follow-up was 38.8 months. Embolization was successfully performed in 4 cases. In 2 of 3 failed cases, the symptoms improved without further treatment. In the remaining 1 failed case, the patient had a relapse of hemarthrosis, so an arthroscopic procedure was performed, which led to identification of the suspicious bleeding point by using preoperative angiographic findings. Electrocauterization was performed and active bleeding was stopped. All cases with recurrent hemarthrosis achieved improvement. CONCLUSIONS: Interventional angiography was used to aid in the diagnosis of recurrent hemarthrosis, and therapeutic selective embolization provided satisfactory clinical results. Even if selective embolization fails, interventional angiography may be helpful for further surgical procedures because it reveals vascular blush of a bleeding site. Therefore, interventional angiography and selective embolization should be considered to be a useful treatment for recurrent hemarthrosis after TKA.

Quantitative measurements of haemophilic joint tissues by point-of-care musculoskeletal ultrasound: Associations with clinical and functional joint outcome parameters.

BACKGROUND: Painful arthropathy is a long-term complication in patients with hemophilia (PWH), affecting mobility and quality of life. A major barrier for the appraisal of joint health is the absence of point-of-care (POC) imaging modalities to promptly identify and manage arthropathic changes. Accordingly, we developed the Joint tissue Activity and Damage Exam (JADE) POC musculoskeletal ultrasound (MSKUS) protocol. JADE is validated for haemophilic joint tissue recognition with high intra/inter-rater and inter-operator reliability. AIMS: Evaluate associations of JADE with clinical (Hemophilia Joint Health Score, [HJHS]) and functional (total arc [combined flexion and extension range of motion [ROM]]) parameters. METHODOLOGY: In this multi-centre prospective study, we recruited PWH A or B with at least one arthropathic joint. We evaluated joint health (both elbows, knees, and ankles) by comparing JADE measurements (soft tissue and cartilage thickness, and osteochondral alterations) with HJHS and total arc. RESULTS: Of 44 PWH, most had hemophilia A (35/44), were severe (36/44) and had a median age of 36 years. Increasing HJHSs and declining total arc, indicating worsening arthropathy, were associated with JADE measurements in the expected direction, including (1) increasing length of osteochondral alterations, (2) diminished cartilage thickness, and (3) greater soft tissue expansion. The ankles had the highest proportion of joints without measurable (missing) cartilage. In multivariable models MSKUS measurements explained 68% and 71% of the variation in HJHS and total arc respectively for the elbow, 55% and 29% respectively for the knee, and 50% and 73% for the ankle. CONCLUSIONS: This study highlights the associations of direct intra-articular ultrasonography measurements using the JADE protocol with clinical and functional parameters. Our findings underscore the clinical value of POC MSKUS using the JADE protocol as a complementary instrument for the diagnosis and management of haemophilic arthropathy.

Acquired multi-segment foot kinematics in haemophilic children, adolescents and young adults with or without haemophilic ankle arthropathy.

INTRODUCTION AND AIM: The ankle joint remains vulnerable in children with haemophilia and is the primary joint affected. The purpose of this study was to dynamically characterize the segmental foot and ankle kinematics of male children, adolescents and young adults with or without ankle arthropathy. METHODS: The barefoot multi-segment foot kinematics of 70 ankles from 35 haemophilia subjects between 6 and 20 years old were captured with the Rizzoli Multi-Segment Foot Model. Joint damage of the tibiotalar and subtalar joints was scored using the IPSG-MRI score. The feet of patients with or without evidence of ankle arthropathy were compared with those of matched typically developing boys via a nonpaired comparison. The differences between the affected and nonaffected sides of patients with unilateral ankle arthropathy were assessed using a paired comparison. RESULTS: Subjects without arthropathy demonstrated a nonsignificant trend towards a higher frontal plane range of motion (RoM) at the midfoot upon loading response and a lower sagittal plane RoM at the midfoot during midstance. No differences were observed between the affected side group and their matched control group. The affected side of unilaterally affected subjects exhibited a nonsignificant tendency towards a higher frontal plane RoM at the ankle joint upon loading response and terminal stance compared to the healthy side. CONCLUSION: Most patients maintained physiological rocker function of the ankle and had no (mal)adaptive motion patterns in the more distal joints of the foot. Therefore, established structural lesions may remain subclinical with respect to moderate functional activities like walking.

Embolization for intractable spontaneous hemarthrosis of the knee joint in the elderly patient: Case report.

RATIONALE: Several case reports about the diagnostic and therapeutic approaches of hemarthrosis after total knee arthroplasty using angiogram have been reported, owing to the probability of bleeding caused by vascular injuries. However, there were only few cases of spontaneous hemarthrosis of the knee joint in the elderly patient that have not undergone total knee arthroplasty that have been previously reported. PATIENTS CONCERNS: An 82-year-old male presented to our outpatient department with acute left knee pain. He had no history of trauma. The patient had under gone several times of therapeutic arthrocentesis for treatment of left knee joint effusion at a local clinic. DIAGNOSIS: Arthroscopic examination was performed at the local clinic and was not able to reveal any focus of intra-articular bleeding. We consulted this case with the department of radiology to angiographically find out abnormalities of the genicular arteries. Angiographs showed hyper vascularity of the superior and inferior lateral genicular artery, and superior medial genicular artery. INTERVENTION: One-step embolization using micro-catheter and 50 to 150 µm gelfoam particles was conducted. The hypervascular findings shown on angiogram were markedly subsided after embolization. OUTCOMES: Until 1 year after embolization, there were no signs of recurrence on outpatient follow-up sessions LESSONS:: Degenerative changes of the genicular arteries may be a cause of spontaneous knee joint hemarthrosis in the elderly patients. Angiographic diagnosis and treatment may be effective for such cases.