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Objectives: To evaluate joint health, pain and health-related quality of life (HRQoL) in patients with moderate/severe haemophilia A in Europe., Design: Multinational, cross-sectional survey, with retrospective data collection. Data were taken from the Adelphi Real World Haemophilia Disease Specific Programme Wave II, using surveys completed by physicians and patients between February 2020 and May 2021., Setting: Haematologists/haemato-oncologists and their patients in France, Germany, Italy, Spain and the UK., Participants: Males aged ≥18 years with moderate or severe haemophilia A (baseline clotting factor level ≤5%), without existing inhibitors and currently receiving prophylaxis. Patients were grouped into those with or without haemophilia-affected joints (HAJs) based on bleeding, radiographic, surgical, mobility and joint pain data., Primary Outcome Measure: Characterisation of humanistic and clinical outcomes in patients with or without HAJs., Results: A total of 120 physicians provided data for 351 eligible patients; 209 (59.5%) patients had HAJs and 142 (40.5%) had no HAJs. Pain/discomfort was significantly different (p=0.01) and reported more frequently in the HAJ (85.7%) vs non-HAJ group (53.3%). Pain medication use was significantly higher in the HAJ versus non-HAJ group (73.2% vs 60.6%; p=0.01). Up to half of the patients with HAJs had synovitis (49.8%) or arthropathy (48.4%), and one-third had undergone joint surgery (35.4%). Overall health status was significantly worse in the HAJ versus non-HAJ group (mean (SD) EuroQol Visual Analogue Scale score: 65.5 (19.3) vs 81.1 (14.6); p=0.01)., Conclusions: In this multinational real-world study, nearly two-thirds of adults with moderate/severe haemophilia A without inhibitors experienced HAJs despite prophylaxis. Individuals with HAJs had higher rates of pain and pain medication use, and lower HRQoL compared with those without HAJs. These data indicate that HAJs represent a clinically relevant burden and early identification/monitoring and management of affected joints should be an important consideration to help prevent long-term joint morbidity., Competing Interests: Competing interests: HDlC-R reports consultancy/advisory, honoraria, expert testimony fees and support for meeting attendance from Bayer, CSL-Behring, Novo Nordisk, Pfizer, Roche, Sobi and Takeda. LB, ZH and NK are employees of and shareholders in Sobi. NB, SO and KG are employees of Adelphi Real World., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Aim: Joint damage due to haemarthrosis can be effectively monitored with point-of care ultrasound using the Haemophilia Early Arthropathy Detection with US (HEAD-US) scoring system. A post hoc comparative analysis of the joint status of patients with severe haemophilia A (HA) or B (HB) was performed., Methods: The databases of two observational, cross-sectional studies that recruited patients with HA or HB from 12 Spanish centres were analysed to compare the status of the elbows, knees and ankles in patients with severe disease according to treatment modality. The HEAD-US score was calculated in both studies by the same trained operators., Results: Overall, 95 HA and 41 HB severe patients were included, with a mean age of 35.2 ± 11.8 and 32.7 ± 14.2 years, respectively. The percentage of patients who received prophylaxis, over on-demand (OD) treatment, was much higher in HA (91.6%) than in HB (65.8%) patients. With a similar number of target joints, the HEAD-US score was zero in 6.3% HA and 22.0% HB patients (p < .01), respectively. The HA population showed significantly worse HEAD-US scores. Whilst osteochondral damage occurred more frequently in patients OD or tertiary prophylaxis, our data suggest that articular damage is less prominent in primary/secondary prophylaxis, regardless of the type of haemophilia. These latter treatment modalities were also associated with a lower prevalence of synovial hypertrophy, particularly in HB patients., Conclusion: This post hoc analysis indicates that joint status seems to be significantly influenced by haemophilia type (HA or HB) and treatment modality in these severe Spanish populations with severe disease. Continuing HEAD-US monitoring for the early detection and management of intra-articular abnormalities, as well as more efficiently tailored therapies should be warranted., (© 2024 John Wiley & Sons Ltd.)

Extracorporeal shock waves are high-intensity mechanical waves (500-1000 bar) of a microsecond duration with a morphology characterized by a rapid positive phase followed by a negative phase., Background: Extracorporeal shock waves have been used for pain treatment for various sub-acute and chronic musculoskeletal (MSK) problems since 2000. The aim of this article is to update information on the role of extracorporeal shock wave therapy (ESWT) in the treatment of various pathologies that cause MSK pain., Methods: Given that in the last two years, articles of interest (including systematic reviews and meta-analyses) have been published on less known indications, such as low back pain, nerve entrapments, osteoarthritis and bone vascular diseases, a literature search was conducted in PubMed, the Cochrane Database, EMBASE, CINAHL and PEDro, with the aim of developing a narrative review of the current literature on this topic. The purposes of the review were to review possible new mechanisms of action, update the level of evidence for known indications and assess possible new indications that have emerged in recent years., Results: Although extracorporeal shock waves have mechanical effects, their main mechanism of action is biological, through a phenomenon called mechanotransduction. There is solid evidence that supports their use to improve pain in many MSK pathologies, such as different tendinopathies (epicondylar, trochanteric, patellar, Achilles or calcific shoulder), plantar fasciitis, axial pain (myofascial, lumbar or coccygodynia), osteoarthritis and bone lesions (delayed union, osteonecrosis of the femoral head, Kienbock's disease, bone marrow edema syndrome of the hip, pubis osteitis or carpal tunnel syndrome). Of the clinical indications mentioned in this review, five have a level of evidence of 1+, eight have a level of evidence of 1-, one indication has a level of evidence of 2- and two indications have a level of evidence of 3., Conclusions: The current literature shows that ESWT is a safe treatment, with hardly any adverse effects reported. Furthermore, it can be used alone or in conjunction with other physical therapies such as eccentric strengthening exercises or static stretching, which can enhance its therapeutic effect.

In addition to receiving the medical treatment they need, people with hemophilia or other coagulopathies must acquire the strategies required to deal with the physical, emotional and social challenges they will encounter at the different stages of their lives. To this end, it is necessary to offer support for any uncertainty that may arise for the patient or parents upon diagnosis of the disease, and to help them develop a healthy response pattern to bleeding episodes. First years and decades of life are essential because self-image is built and the foundations for socialization are laid, making it necessary to foster communication and self-esteem. However, in later life, the social impact of the consequences of hemophilia, including disability and dependency, require the introduction of technical and human adaptations and aids. Hematological, rehabilitative, orthopedic psychological and social approaches focus on improving the individual's ability to carry out daily activities independently and facilitate social integration. Patient associations play a key role in minimizing the social impact of hemophilia. Finally, recognition of disability helps to compensate to some extent for the difficulties faced by people with hemophilia., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

The Metaverse is 'an integrated network of 3D virtual worlds.' It incorporates digitally created realities into the real world, involves virtual copies of existing places and changes the physical reality by superimposing digital aspects, allowing its users to interact with these elements in an immersive, real-time experience. The applications of the Metaverse are numerous, with an increasing number of experiences in the field of musculoskeletal disease management. In the field of medical training, the Metaverse can help facilitate the learning experience and help develop complex clinical skills. In clinical care, the Metaverse can help clinicians perform orthopedic surgery more accurately and safely and can improve pain management, the performance of rehabilitation techniques and the promotion of healthy lifestyles. Virtualization can also optimize aspects of healthcare information and management, increasing the effectiveness of procedures and the functioning of organizations. This optimization can be especially relevant in departments that are under significant care provider pressure. However, we must not lose sight of the fundamental challenges that still need to be solved, such as ensuring patient privacy and fairness. Several studies are underway to assess the feasibility and safety of the Metaverse.

Background: Primary prophylaxis with factor VIII concentrates is the therapeutic gold standard for severe hemophilia A. Although this approach will change substantially with the use of nonsubstitutive therapies, the long-term effects of primary prophylaxis remain unclear. We present information on joint health with tailored primary prophylaxis in a consecutive series at a single center., Methods: We retrospectively analyzed 60 patients who did not develop early inhibitors. The annual bleeding rate and annual joint bleeding rate, prophylaxis characteristics, physical activity, adherence, and development of inhibitors were compared between those with and without joint involvement at the end of follow-up. Joint involvement was defined as a Hemophilia Joint Health Score or Hemophilia Early Arthropathy Detection with an ultrasound score ≥1., Results: Among 60 patients with median follow-up of 113 ± 6 months after starting prophylaxis, 76.7% had no joint involvement at the end of the follow-up. Those without joint involvement started prophylaxis at a younger median age (1 [IQR 1-1] year vs 3 [IQR 2-4.3] years). They also had lower annual joint bleeding rate (0.0 [IQR 0-0.2] vs 0.2 [IQR 0.1-0.5]), were more often physically active (70% vs 50%), and had lower trough factor VIII levels. Adherence to treatment was not significantly different between groups., Conclusion: Initiation of primary prophylaxis at a younger age was the main factor associated with long-term preservation of joint status in patients with severe hemophilia A., (© 2022 The Authors.)

The main complications of surgical reconstruction of multiligament injuries of the knee joint are residual or recurrent instability, arthrofibrosis, popliteal artery injury, common peroneal nerve injury, compartment syndrome, fluid extravasation, symptomatic heterotopic ossification, wound problems and infection, deep venous thrombosis, and revision surgery.Careful surgical planning and execution of the primary surgical reconstruction of multiligament injuries of the knee joint can minimize the risk of the aforementioned complications.Careful postoperative follow-up is required to detect complications. Early recognition and prompt treatment are of paramount importance.To obtain good results in the revision surgery of failed multiligamentary knee reconstructions, it is crucial to perform a thorough and exhaustive evaluation to detect all the causes of failure.Addressing all associated injuries during revision surgery will lead to the best possible subjective and objective results, although functional outcomes are often modest.However, advanced age and high-energy injuries have been associated with the poorest functional outcomes after revision surgery of failed multiligament injuries of the knee joint. Cite this article: EFORT Open Rev 2021;6:973-981. DOI: 10.1302/2058-5241.6.210057., Competing Interests: ICMJE Conflict of interest statement: The authors declare no conflict of interest relevant to this work., (© 2021 The author(s).)

Introduction: The Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) system and scoring scale has proven to be an accurate and time-efficient imaging method for identifying joint damage in patients with haemophilia., Aim: Observational, multicentre, cross-sectional study conducted in 8 centres in Spain that assessed the joint status of adult patients with severe haemophilia A (SHA) using HEAD-US., Methods: Joint status of the elbow, knee and ankle was evaluated in adults with SHA receiving on-demand (OD) treatment, or primary (PP), secondary (SP), tertiary (TP) or intermittent (IP) prophylaxis., Results: Of the 95 patients enrolled, 87 received prophylaxis (6.3% PP, 38.9% SP, 43.2% TP and 3.2% IP). Mean age was 35.2 years, and 59% of patients had not undergone image testing in the last year. The HEAD-US score was 0 in all joints in 6.3% of patients. The ankle was the most affected joint, regardless of treatment regimen. Patients receiving OD treatment, TP or IP had the overall worst scores, mainly in the ankles and elbows; a similar but milder profile was observed in patients on SP; and patients on PP had the best score in all joints., Conclusion: Joint function may be effectively preserved in patients with SHA on PP, but OD treatment or later initiation of prophylaxis does not seem to prevent progression of arthropathy. Disease worsening was observed in patients OD, TP or IP, most often affecting ankles and elbows. Closer ultrasound imaging monitoring may improve management of these patients., (© 2021 John Wiley & Sons Ltd.)

The enormous pressure of the increasing case numbers experienced during the COVID-19 pandemic has given rise to a variety of novel digital systems designed to provide solutions to unprecedented challenges in public health. The field of algorithmic contact tracing, in particular, an area of research that had previously received limited attention, has moved into the spotlight as a crucial factor in containing the pandemic. The use of digital tools to enable more robust and expedited contact tracing and notification, while maintaining privacy and trust in the data generated, is viewed as key to identifying chains of transmission and close contacts, and, consequently, to enabling effective case investigations. Scaling these tools has never been more critical, as global case numbers have exceeded 100 million, as many asymptomatic patients remain undetected, and as COVID-19 variants begin to emerge around the world. In this context, there is increasing attention on blockchain technology as a part of systems for enhanced digital algorithmic contact tracing and reporting. By analyzing the literature that has emerged from this trend, the common characteristics of the designs proposed become apparent. An archetypal system architecture can be derived, taking these characteristics into consideration. However, assessing the utility of this architecture using a recognized evaluation framework shows that the added benefits and features of blockchain technology do not provide significant advantages over conventional centralized systems for algorithmic contact tracing and reporting. From our study, it, therefore, seems that blockchain technology may provide a more significant benefit in other areas of public health beyond contact tracing., (©Moritz Platt, Anton Hasselgren, Juan Manuel Román-Belmonte, Marcela Tuler de Oliveira, Hortensia De la Corte-Rodríguez, Sílvia Delgado Olabarriaga, E Carlos Rodríguez-Merchán, Tim Ken Mackey. Originally published in JMIR Public Health and Surveillance (http://publichealth.jmir.org), 06.04.2021.)

Introduction: We present the first registry of patients with congenital bleeding disorders and COVID-19. The study has been carried out in the Community of Madrid, which has the highest number of cases in Spain. The objective is to understand the incidence of COVID-19, the course of the disease if it occurs and the psychosocial and occupational impact on this population., Methods: We included 345 patients (246 of haemophilia, 69 of von Willebrand Disease, two rare bleeding disorders and 28 carriers of haemophilia). A telephone survey was used to collect the data., Results: Forty-two patients presented symptoms suggestive of infection by COVID-19, and in six cases, the disease was confirmed by RT-PCR. The cumulative incidence of our series was 1.73%. It is worth noting the complexity of the management of COVID-19 in two patients on prophylaxis with non-factor replacement therapy. Adherence to the prescribed treatment was maintained by 95.5% of patients. Although 94% were independent for daily living activities, 42.4% had a recognized disability and 58% required assistance, provided by the Madrid Haemophilia Association (Ashemadrid) in 75% of cases. Only 4.4% of consultations were held in person., Conclusions: Patients with congenital bleeding disorders infected with SARS-CoV-2 presented a mild course of the disease that did not require admission. Their identification and treatment by a specialist team from a Haemophilia Treatment Center are essential to make a correct assessment of the risk of haemorrhage/thrombosis. COVID-19 had a major impact on the psychosocial aspects of these patients which must be remedied with recovery plans., (© 2020 John Wiley & Sons Ltd.)

Aim: The use of musculoskeletal ultrasound (MSK-US) following protocols for haemophilic arthropathy and the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score can help standardize monitoring in haemophilia. This study evaluated the joint status (elbows, knees and ankles) of patients with haemophilia B (HB) in Spain using MSK-US and the HEAD-US score., Methods: Haemophilia B patients ≥14 years old were included in this observational, multicentre, cross-sectional study, regardless of their clinical condition, HB severity and treatment received. Two blinded observers were involved in image acquisition and scoring in each centre., Results: Eighty-two patients from 12 centres were enrolled: 27% mild HB, 23% moderate, 50% severe HB. Mean age was 38.9 ± 16.4 years, 60% were treated on demand (OD) and 40% were on prophylaxis. HEAD-US was zero in all joints in 28.6% OD patients and 36.4% on prophylaxis. Mean scores significantly worsened with HB severity, except for the left knee. Patients on primary and secondary prophylaxis had significantly better joint health vs OD patients in all joints, except the right ankle. Among OD patients, those with severe disease presented significantly worse scores in all HEAD-US items related to permanent damage., Conclusion: Joint status of HB patients in Spain is influenced by severity and treatment modality, related to the development of arthropathy, which appears prevalent in OD patients with severe HB. Routine assessment with an imaging tool such as ultrasound and HEAD-US system may help to improve joint health by personalizing and adjusting treatment in this population., (© 2018 John Wiley & Sons Ltd.)