RESUMEN
INTRODUCTION: Hemophilia is an inherited bleeding disorder. Bleeding, and in particular joint hemorrhage results in chronic arthropathy and disability. Acute and chronic pain are frequent and limit activity and participation and result in decreased health-related quality of life. Remarkable progress has been made in the diagnosis and treatment of hemophilia but bleeding continues to prove recalcitrant to currently available treatments and joint disease remains problematic. Physiotherapy and pain management are mainstays of current multidisciplinary integrated care of people with hemophilia (PWH). The focus of this review is on preservation of joint health in the era of new and innovative therapies. AREAS COVERED: A search of the PubMed Central was conducted on 1 February 2024 using the MeSH Major Topic terms identified as keywords for the manuscript. This review will highlight what is known and unknown about joint bleeding and arthropathy, including insights on pain as a related complication. EXPERT OPINION: Recent advances in therapeutic interventions aimed at promoting healthy joints in PWH will be discussed, including both the pharmacological treatment landscape and related strategies to promote joint health.
Asunto(s)
Hemofilia A , Humanos , Hemofilia A/terapia , Hemofilia A/complicaciones , Manejo del Dolor/métodos , Dolor/etiología , Calidad de Vida , Hemartrosis/terapia , Hemartrosis/etiología , Hemartrosis/diagnóstico , Artropatías/terapia , Artropatías/etiología , Artropatías/diagnósticoRESUMEN
INTRODUCTION: The therapeutic approach to pain in hemophilia should be multimodal. Intra-articular injections are a good option when joint lesions do not respond to hematological treatment or rehabilitation and orthopedic surgery is not yet indicated. Performing these procedures under ultrasound guidance has been shown to improve their accuracy and efficacy. AREAS COVERED: This article provides a practical overview of the most frequently employed ultrasound-guided intra-articular procedures on the joints of people with hemophilia. The article describes the key elements for performing the technique on the elbow, knee and ankle as the most affected joints. The particularities of the most frequent indications, arthrocentesis, synoviorthesis and analgesic injections with various products are detailed. EXPERT OPINION: Current hematological treatments have made it possible to incorporate new therapeutic tools for pain relief for people with hemophilia, including ultrasound-guided joint procedures, which offer excellent results.
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Hemofilia A , Ultrasonografía Intervencional , Humanos , Hemofilia A/complicaciones , Ultrasonografía Intervencional/métodos , Inyecciones Intraarticulares , Hemartrosis/etiología , Hemartrosis/terapia , Artropatías/cirugía , Artropatías/terapiaRESUMEN
Gene therapy and universal use of safer, more effective, and personalised prophylactic regimens (factor, and nonfactor) are expected to prevent joint bleeding and promote joint health in persons with haemophilia (PwH). Growing evidence suggests that subclinical bleeding, with active and inactive synovial proliferation, continues and haemophilic arthropathy remains a major morbidity in PwH despite early institution of joint prophylaxis. Joint health assessment is evolving with physical examination scores complementing imaging scores. Point-of-care ultrasound is emerging as a safe, cost-effective, and readily available tool for acute determination of musculoskeletal abnormalities, serial evaluation of joints for sonographic markers of haemophilic arthropathy, and in providing objective insight into the efficacy of new therapies. In acute haemarthrosis, arthrocentesis expedites recovery and prevent the vicious cycle of bleed-synovitis-rebleed. When synovial proliferation develops, a multidisciplinary team approach is critical with haematology, orthopaedics, and physiotherapy involvement. Synovectomy is considered for patients with chronic synovitis that fail conservative management. Non-surgical and minimally invasive procedures should always be offered and considered first. Careful patient selection, screening and early intervention increase the success of these interventions in reducing bleeding, pain, and improving joint function and quality of life. Chemical synovectomy is practical in developing countries, but radioactive synovectomy appears to be more effective. When surgical synovectomy is considered, arthroscopic/minimally invasive approach should be attempted first. In advanced haemophilic arthropathy, joint replacement and arthrodesis can be considered. While excited about the future of haemophilia management, navigating musculoskeletal challenges in the aging haemophilia population is equally important.
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Artritis , Hemofilia A , Sinovitis , Humanos , Hemofilia A/complicaciones , Hemofilia A/terapia , Hemofilia A/diagnóstico , Calidad de Vida , Hemartrosis/diagnóstico , Hemartrosis/etiología , Hemartrosis/terapia , Sinovitis/diagnóstico , Sinovitis/etiología , Sinovitis/terapia , Envejecimiento , ArtrodesisRESUMEN
INTRODUCTION: Transformational advances have occurred in the management of haemophilia in the last decade leading to much better outcomes. However, a detailed and critical examination of its assessment and reporting show gaps in many aspects. These are discussed in this review. METHODS: The relevant literature related to different aspects of management of haemophilia was reviewed to identify gaps which need to be addressed. These include detection and diagnosis of haemophilia, documentation and reporting of joint bleeding, its management and methods of reporting in clinical trials and practice, aspects of personalizing care as well as access to therapeutic products and the need for and organization of comprehensive care. RESULTS: Current diagnostic approaches have more than doubled the identified number of persons with haemophilia (PWH) over the last 25 years but still constitute only â¼30% of the expected number. Joint bleeding is the primary indicator of disease severity and treatment efficacy, but there is lack of consistency and standardization in the way it is recorded and reported. Its continued use as an efficacy measure of modern treatments which maintain steady state factor levels or equivalence of >5% will lack sensitivity. The treatment of acute haemarthrosis has focussed on haemostasis and pain control, ignoring the role of inflammation in joint damage. Phenotypic heterogeneity of severe haemophilia has recognized clinical and laboratory variations based on haemostasis but not differences in local response to blood in the joint. At the organizational level, IU/capita provides a relevant measure of access to therapeutic products when the detection rate is â¼100% but is fallaciously low when detection rates are very low. With highly effective modern therapies for haemophilia and nearly no bleeding, the concept of comprehensive care team will need modifications. CONCLUSION: As haemophilia care advances, a deeper dive is needed into the details of various aspects its management to ensure consistency and contemporary relevance.
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Hemofilia A , Humanos , Hemofilia A/terapia , Hemofilia A/tratamiento farmacológico , Hemartrosis/terapia , Hemorragia/etiología , Hemorragia/terapia , Manejo del Dolor/métodos , Resultado del TratamientoRESUMEN
Hemophilia A and B are rare X-linked genetic bleeding disorders due to a complete or partial deficiency in the coagulation factors VIII or IX, respectively. The main treatment for hemophilia is prophylactic and based on coagulation factor replacement therapies. These treatments have significantly reduced bleeding and improved the patients' quality of life. Nevertheless, repeated joint bleedings (hemarthroses), even subclinical hemarthroses, can lead to hemophilic arthropathy (HA). This disabling condition is characterized by chronic pain due to synovial inflammation, cartilage and bone destruction requiring ultimately joint replacement. HA resembles to rheumatoid arthritis because of synovitis but HA is considered as having similarities with osteoarthritis as illustrated by the migration of immune cells, production of inflammatory cytokines, synovial hypertrophy and cartilage damage. Various drugs have been evaluated for the management of HA with limited success. The objective of the review is to discuss new therapeutic approaches with a special focus on the studies that have investigated the potential of using mesenchymal stromal cells (MSCs) in the management of HA. A systematic review of the literature has been made. Most of the studies have focused on the interest of MSCs for the delivery of missing factors VIII or IX but in some studies, more insight on the effect of MSC injection on synovial inflammation or cartilage structure were provided and put in perspective for possible clinical applications.
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Hemofilia A , Hemofilia B , Trasplante de Células Madre Mesenquimatosas , Humanos , Hemartrosis/etiología , Hemartrosis/terapia , Hemofilia A/complicaciones , Hemofilia A/terapia , Trasplante de Células Madre Mesenquimatosas/métodos , Células Madre Mesenquimatosas , Hemofilia B/complicaciones , Hemofilia B/terapiaRESUMEN
BACKGROUND: Hemophilia is a congenital coagulopathy characterized by degenerative joint damage. Self-induced myofascial therapy aims to decrease pain and improve tissue mobility, functionality and proprioception. AIM: The aim of this study was to evaluate the safety and efficacy of self-induced myofascial release in patients with hemophilic knee arthropathy. DESIGN: This is a randomized clinical study. SETTING: This study was carried out in different patient associations. POPULATION: Fifty-two patients with hemophilia were included in the study. METHODS: Patients were randomized to the experimental group (daily home protocol of foam roller-based self-induced myofascial therapy for 8 weeks) or the control group (no intervention). The variables were the frequency of hemarthrosis (self-reporting), pain intensity (visual analog scale), range of motion (goniometry) and muscle strength (dynamometry). All variables were evaluated at baseline, post-treatment and after a 10-week follow-up. RESULTS: The patients included in the experimental group showed significant improvements in terms of a decrease in frequency of hemarthrosis (mean difference [MD]=-0.61; 95% confidence interval [CI]: -0.81; -0.41) and pain intensity (MD=-0.33; 95% CI: -0.48, -0.18), increased range of motion (MD=0.88; 95% CI: 0.39; 1.37), strength in quadriceps (MD=0.88; 95% CI: 0.39; 1.37). (MD=12.39; 95% CI: 3.44; 21.34) and hamstrings (MD=7.85; 95% CI: 0.60; 15.11). There were intergroup differences in the frequency of hemarthrosis (F=14.51; P<0.001), pain intensity (F=9.14; P<0.001) and range of motion (F=13.58; P<0.001). CONCLUSIONS: Self-induced myofascial therapy can be an effective complementary technique in the treatment of patients with hemophilic arthropathy. Self-induced myofascial therapy can reduce the frequency of knee hemarthrosis in patients with hemophilia. This technique can improve pain intensity and range of motion in patients with hemophilic knee arthropathy. CLINICAL REHABILITATION IMPACT: Hemophilic knee arthropathy is characterized by chronic pain, decreased range of motion, and periarticular muscle atrophy. Foam roller-based self-induced myofascial therapy can reduce the frequency of hemarthrosis and pain intensity and improve range of motion in patients with hemophilic arthropathy. Foam roller-based self-induced myofascial therapy is safe and effective in the treatment of patients with hemophilia. The inclusion of self-induced myofascial therapy exercises in the approach to degenerative joint pathologies may be an effective and safe treatment option.
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Hemofilia A , Humanos , Hemofilia A/complicaciones , Hemartrosis/terapia , Hemartrosis/complicaciones , Método Simple Ciego , Articulación de la Rodilla , DolorRESUMEN
INTRODUCTION: It is important to know the current status of hemophilic arthropathy diagnoses, treatments, complications, and outcomes in developed countries. AREAS COVERED: A bibliographic search in PubMed for articles published from 1 January 2019 through 12 June 2023 was performed. EXPERT OPINION: In developed countries with specialized hemophilia treatment centers, primary hematological prophylaxis (started before the age of 2 years and after no more than one joint bleed) has almost completely eliminated the joint-related problems of the disease. The ideal goal of zero hemarthroses can be achieved only with intense and well-dosed prophylaxis: intravenous infusion of coagulation factor - standard half-life or extended half-life; periodic or subcutaneous injections of nonfactor products (emicizumab or fitusiran). However, hemophilic arthropathy continues to occur due to subclinical joint hemorrhages. In one study, 16% of the joints without reported hemarthroses showed signs of previous subclinical bleeding (hemosiderin deposits with/without synovial hypertrophy on magnetic resonance imaging were deemed signs of previous subclinical bleeding), rendering evidence for subclinical bleeding in people with severe hemophilia with lifelong prophylaxis treatment. Subclinical joint hemorrhages can be averted only by employing accurate and tailored prophylaxis.
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Hemofilia A , Sinovitis , Humanos , Preescolar , Hemartrosis/diagnóstico , Hemartrosis/etiología , Hemartrosis/terapia , Hemofilia A/complicaciones , Hemofilia A/diagnóstico , Hemorragia/diagnóstico , Hemorragia/etiología , Hemorragia/terapia , Sinovitis/etiología , Sinovitis/patología , Sinovitis/prevención & control , Factores de Coagulación Sanguínea/uso terapéuticoRESUMEN
Introduction: Hemophilia A and B are rare congenital X-linked recessive diseases caused by lack or deficiency of the coagulation factors VIII (FVIII) or IX (FIX), respectively. The primary therapeutic approach is to replace the deficient coagulation factor, which can be achieved with factors derived from human plasma or recombinants. However, despite having a therapeutic approach, most severe cases are symptomatic and may have complications, mainly in the muscles and joints. One example of such disorder is hemarthrosis. This manifestation tends to affect mainly the knee, ankle, or elbow joints in about 80% of cases. Objective: to describe the primary forms of treatment for joint bleeding in patients with severe hemophilia. Methods: This is a qualitative research of the integrative review type meant to identify productions on topics associated with hemarthrosis and severe hemophilia. The articles were searched through the databases PubMed, Scientific Electronic Library Online (Scielo) and Virtual Health Library (BVS) with the following search descriptors: "hemarthrosis and hemophilia"; "joint diseases and Hemophilia" and corresponding terms in Portuguese. The inclusion criteria were as follows: a) scientific articles b) available in full-text c) studies available in Portuguese, English, or Spanish d) randomized clinical trials e) articles published between 2016 and 2021 f) articles containing hemarthrosis caused by severe hemophilia. As exclusion criteria, texts that had no relation to the theme, did not answer the guiding question, other types of articles that did not include randomized clinical trials and/or presented duplicates were discarded. Results: In total, 42 articles were found in the selected databases; eight were duplicated, and 25 were excluded for not being randomized clinical trials or because they did not contemplate the theme. After careful reading, nine articles that met the inclusion and exclusion criteria were identified. Of the eligible studies, one reported factor replacement, and eight reported physiotherapeutic treatment. Conclusion:Factor replacement for hemophilic patients is essential and, based on the information obtained, early replacement is ben-eficial for the patient to avoid joint complications. Prophylaxis is indicated in severe hemophilia and its main objective is to prevent recurrent hemarthrosis, which can cause permanent functional deformities. Some physiotherapeutic interventions are indicated to prevent joint damage in severe hemophilic patients. The findings show diversity in the physical therapy modalities employed. The complete prevention of joint damage is still a challenge. A combination of treatments and a multi-disciplinary team follow-up is necessary to ensure health and quality of life of patients. (AU)
Introdução: As hemofilias A e B são doenças congênitas raras, recessivas ligadas ao X, causadas por falta ou deficiência de fator de coagulação VIII (FVIII) ou IX (FIX), respectivamente. A terapêutica tem como conduta principal a reposição do fator de coagulação deficiente, podendo ser feita com fatores derivados de plasma humano ou recombinantes. Porém, apesar de possuir uma terapêutica, grande parte dos casos graves são sintomáticos e podem ter complicações, na sua maioria, nos músculos e nas articulações. Uma dessas desordens é a hemartrose. Essa manifestação tende a acometer principalmente articulações do joelho, tornozelo ou cotovelo em cerca de 80% dos casos. Objetivo: descrever as principais formas de tra-tamento para sangramento articular em pacientes com hemofilia grave. Método: Trata-se de uma pesquisa qualitativa do tipo revisão integrativa para identificação de produções sobre temas associados a hemartrose e hemofilia grave. A busca dos artigos foi através das bases de dados PubMed, Scientific Electronic Library Online (SciELO) e Biblioteca Virtual em Saúde (BVS) com os seguintes descritores de busca: "hemarthrosis and hemophilia"; "joint diseases and hemophilia" e termos correspondentes no português. Os critérios de inclusão foram os seguintes: a) artigos b) estar disponível em texto completo c) estudos disponíveis nos idiomas português, inglês ou espanhol d) ensaios clínicos randomizados e) artigos publicados entre 2016 e 2021 f) artigos que contemplem hemartrose por hemofilia grave. Resultados: No total, foram encontrados 42 artigos nas bases de dados selecionadas; oito estavam duplicados e 25 foram excluídos por não serem ensaios clínicos randomizados ou por não contemplarem a temática. Após leitura cuidadosa, foram identificados 9 artigos que atenderam aos critérios de inclusão e exclusão. Dos trabalhos elegíveis, um relatou sobre reposição de fator e oito artigos relataram sobre tratamento fisioterapêutico. Conclusão: A reposição de fatores para pacientes hemofílicos é essencial e, com base nas informações obtidas, a reposição precoce é benéfica para o paciente, evitando complicações articulares. A profilaxia está indicada na hemofilia grave e seu principal objetivo é prevenir a hemartrose recorrente, que pode causar deformidades funcionais permanentes. Algumas intervenções fisioterapêuticas são indicadas para prevenir danos articulares em pacientes hemofílicos graves. Os achados mostram diversidade nas modalidades de fisioterapia empregadas. A prevenção total dos danos articulares ainda é um desafio. É necessária uma combinação de tratamentos e acompanhamento por equipe multi-disciplinar de forma a garantir a saúde e qualidade de vida dos pacientes. (AU)
Asunto(s)
Humanos , Anomalías Congénitas , Hemartrosis/terapia , Hemofilia A/terapiaRESUMEN
INTRODUCTION: Haemarthrosis is a clinical feature of haemophilia leading to haemarthropathy. The ankle joint is most commonly affected, resulting in significant pain, disability and a reduction in health-related quality of life. Footwear and orthotic devices are effective in other diseases that affect the foot and ankle, such as rheumatoid arthritis, but little is known about their effect in haemophilia. AIMS: To review the efficacy and effectiveness of footwear and orthotic devices in the management of ankle joint haemarthrosis and haemarthropathy in haemophilia. METHODS: A systematic literature review was conducted. Two review authors independently screened studies for inclusion and appraised methodological quality using Joanna Briggs Institute Critical Appraisal checklists. A narrative analysis was undertaken. RESULTS: Ten studies involving 271 male participants were eligible for inclusion. All studies were quasi-experimental; three employed a within-subject design. Two studies included an independent comparison or control group. A range of footwear and orthotic devices were investigated. Limited evidence from non-randomised studies suggested that footwear and orthotic devices improve the number of ankle joint bleeding episodes, gait parameters and patient-reported pain. CONCLUSION: This review demonstrates a lack of robust evidence regarding the efficacy and effectiveness of footwear and orthotic devices in the management of ankle joint haemarthrosis and haemarthropathy in haemophilia. Methodological heterogeneities and limitations with the study designs, small sample sizes and limited follow-up of participants exist. Future studies utilising randomised designs, larger sample sizes, long-term follow-up and validated patient-reported outcome measures are needed to inform the clinical management of ankle joint haemarthrosis and haemarthropathy.
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Hemartrosis , Hemofilia A , Tobillo , Articulación del Tobillo , Femenino , Hemartrosis/etiología , Hemartrosis/terapia , Hemofilia A/complicaciones , Hemofilia A/terapia , Humanos , Masculino , Aparatos Ortopédicos , Dolor , Calidad de VidaRESUMEN
OBJECTIVE: To evaluate the effect of fascial therapy on the perceived quality of life, pain intensity, and joint health in people with hemophilia. DESIGN: Single-blind randomized controlled trial. SETTING: Hemophilia Patient Associations. PARTICIPANTS: Patients with hemophilia (N=69) were randomly allocated to an experimental group (fascial therapy protocol) or to a control group (no intervention whatsoever). INTERVENTIONS: The fascial therapy intervention lasted 3 consecutive weeks with one 45-minute weekly session. MAIN OUTCOME MEASURES: The variables evaluated were perceived quality of life (Short Form Health Survey-36), pain intensity (visual analog scale) and joint health (Hemophilia Joint Health Score) at baseline and at 3 and 12 weeks. RESULTS: The mean values of the physical (38.64±9.41 and 41.92±12.16) and mental (45.77±6.25 and 55.02±9.73) components of the Short Form Health Survey-36 improved after the intervention in the experimental group, as well as those for pain intensity and joint health (P<.001). There were differences (P<.001) in the intergroup effect in the physical role variables (F=49.22), emotional role (F=229.71), mental component (F=9.86), intensity of pain (F=24.74), and joint health (F=55.31). CONCLUSIONS: A fascial therapy protocol for patients with hemophilic elbow arthropathy can improve their perceived quality of life. Elbow pain intensity improved in patients treated with fascial therapy. This technique can improve elbow joint health.
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Articulación del Codo , Hemofilia A , Codo , Hemartrosis/complicaciones , Hemartrosis/terapia , Hemofilia A/complicaciones , Humanos , Calidad de Vida , Método Simple Ciego , Resultado del TratamientoRESUMEN
Roughly 37% of Americans 60 years of age and older experience chronic pain due to osteoarthritis (OA) of the knee. After conservative treatment (pharmacologic, physical therapy, and joint injections) fails, patients often require total knee arthroplasty to alleviate pain and regain knee function. Given the high economic burden of surgery paired with its invasive nature, many patients with this degenerative joint disease seek alternative treatment. Moreover, many patients with severe knee OA who also have comorbidities that preclude surgery-most often morbid obesity-are left without options. Geniculate artery embolization (GAE) is a minimally invasive intra-arterial intervention that was originally developed for the treatment of knee hemarthrosis that has recently been adapted for symptomatic knee OA. Through selective embolization of geniculate branches corresponding to the site of knee pain, GAE inhibits the neovascularity that contributes to the catabolic and inflammatory drive of OA. Preliminary trials over the past decade have demonstrated promising clinical results, including decreased pain and improved function and quality of life after treatment. Given such success, GAE provides another minimally invasive treatment option for knee OA to patients who feel reluctant to undergo or are ineligible for surgery. The authors review the radiographic manifestations and current standard of treatment of OA and hemarthrosis of the knee. Procedural technique, embolic selection, and clinical evidence for GAE in the treatment of OA and hemarthrosis of the knee are also explored. The online slide presentation from the RSNA Annual Meeting is available for this article. ©RSNA, 2021.
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Artroplastia de Reemplazo de Rodilla , Osteoartritis de la Rodilla , Arterias , Artroplastia de Reemplazo de Rodilla/efectos adversos , Hemartrosis/etiología , Hemartrosis/terapia , Humanos , Osteoartritis de la Rodilla/complicaciones , Osteoartritis de la Rodilla/diagnóstico por imagen , Osteoartritis de la Rodilla/terapia , Calidad de VidaRESUMEN
OBJECTIVES: Hemophilic arthropathy is the most common complication of severe hemophilia A. This study aims to investigated joint status and related risk factors in patients with severe hemophilia A (PWSHA). METHODS: This single-center study included 31 patients. Six index joints (both elbows, knees, and ankles) were evaluated using the Hemophilia Early Arthropathy Detection with UltraSound in China (HEAD-US-C) and Hemophilia Joint Health Score (HJHS). Treatment adherence was measured using the Validated Hemophilia Regimen Treatment Adherence Scale-Prophylaxis (VERITAS-Pro). We analyzed the influence of age, treatment delay (the interval between diagnosis and the initiation of treatment), prophylaxis, and treatment adherence on joint outcomes. RESULTS: All patients were male (median age, 22 years). The median age at diagnosis was 1 year; that at initial treatment was 5 years. All patients experienced joint bleeding. HEAD-US-C and HJHS scores were positively correlated (R = 0.70, P < 0.0001). Median [range] HEAD-US-C and HJHS scores were 15 [0-36] and 32 [2-49], respectively. Age was positively correlated with both HEAD-US-C (P = 0.002) and HJHS scores (P < 0.0001). The difference of HEAD-US-C scores between groups with ≤1 year and >1 year treatment delay was close to significant (P = 0.055). HJHS scores were significantly different between these two groups (P = 0.03). Joint assessment scores were not significantly different between on-demand and low-dose prophylaxis groups. VERITAS-Pro scores were correlated with both HEAD-US-C and HJHS scores (P = 0.046 and P = 0.005, respectively). CONCLUSIONS: Hemophilic arthropathy was pervasive in PWSHA. Age and poor adherence were significantly correlated with joint damage. Prompt treatment and adherence improvement may reduce severity.
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Hemartrosis/epidemiología , Hemartrosis/etiología , Hemofilia A/complicaciones , Hemofilia A/epidemiología , Adolescente , Adulto , Niño , Preescolar , Estudios Transversales , Diagnóstico Tardío , Manejo de la Enfermedad , Susceptibilidad a Enfermedades , Hemartrosis/terapia , Hemofilia A/diagnóstico , Hemofilia A/terapia , Humanos , Masculino , Medición de Riesgo , Factores de Riesgo , Adulto JovenRESUMEN
Background: Hemophilic arthropathy is characterized by loss of function and chronic pain. Fascial therapy mobilizes the connective tissue and is thus involved in the condition of the injured fascial complex and the surrounding tissues.Objective: To evaluate the safety of a physiotherapy program using fascial therapy in patients with hemophilic elbow arthropathy.Methods: Fourteen adult patients with hemophilia were randomly assigned to a control group and an intervention group. The intervention consisted of three 45-min sessions of fascial therapy over a 3-week period. Assessment was carried out at baseline, after treatment, and at follow-up. The study variables were bleeding frequency using a self-registration of bleeding; joint pain using the visual analog scale; range of motion with a universal goniometer; and joint status assessed with Hemophilia Joint Health Score.Results: None of the patients developed joint bleeding during the experimental period. Joint pain in the experimental group decreased by 1.43 out of 2.43 (95% CI 0.52 to 2.33) and 2.14 out of 2.57 (95% CI 0.18 to 4.10) in right and left elbow, respectively, more than the control group by 3 weeks. Flexion increased by 3.57 degrees out of 129.14 (95% CI 5.48 to 1.65) in right elbow and joint condition improved by 1.14 points out of 6.0 (95% CI 0.01 to 2.26) more than the control group by 3 weeks.Conclusion: Fascial therapy does not appear to produce elbow hemarthrosis in patients with hemophilia. This treatment can improve joint pain, range of motion, and elbow status in patients with hemophilia.
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Codo , Hemofilia A , Adulto , Hemartrosis/diagnóstico , Hemartrosis/etiología , Hemartrosis/terapia , Hemofilia A/complicaciones , Hemofilia A/diagnóstico , Hemofilia A/terapia , Humanos , Proyectos Piloto , Rango del Movimiento Articular , Resultado del TratamientoRESUMEN
INTRODUCTION: Arthropathy is the characteristic injury of hemophilia, primarily occurring in the elbows, knees, and ankles. The aim of this review is a teaching approach devoted to hemophilia treaters in under-development countries. AREAS COVERED: Current major challenges include the absence of the following therapeutic tools for all people with hemophilia (PWH) worldwide: hematological prophylaxis; well-coordinated multidisciplinary teams in specialized centers; joint aspiration of acute hemarthrosis; point-of-care ultrasonography (POC-US); exercise; treatment of synovitis by means of radiosynovectomy; treatment of mild hemophilic arthropathy with analgesics, anti-inflammatory agents, and rehabilitation medicine; intra-articular injections of corticosteroids, hyaluronic acid, platelet-rich plasma, and mesenchymal stem cells; and treatment of severe hemophilic arthropathy by surgical treatment. The future scenario in under-development countries should include all the aforementioned treatment tools plus the possibility of performing total joint arthroplasty in PWH with inhibitors; concomitant use of bypassing agents with emicizumab for PWH with inhibitors undergoing orthopedic surgery; and telemedicine. EXPERT OPINION: Primary hematological prophylaxis is now the gold standard for the management of hemophilia. Acute hemarthrosis needs intense hematological management and articular aspiration; the articular situation should be scrutinized by POC-US. Synovitis can be controlled by radiosynovectomy. In cases of severe articular degeneration, surgical procedures might be required.
Asunto(s)
Articulación del Codo , Hemofilia A , Sinovitis , Hemartrosis/prevención & control , Hemartrosis/terapia , Hemofilia A/tratamiento farmacológico , Hemofilia A/terapia , Humanos , Articulaciones , Sinovitis/complicaciones , Sinovitis/terapiaRESUMEN
Hemophilia A and B are rare X-linked inherited bleeding disorders caused by complete or partial deficiency in or the absence of coagulation factors VIII and IX. Recurrent joint bleeding (hemarthrosis) is the most frequent clinical manifestation of severe hemophilia. Unless appropriately managed, even subclinical hemarthrosis can lead to the development of hemophilic arthropathy, a disabling condition characterized by joint remodelling, chronic pain, and a reduced quality of life, and eventually requires joint replacement. Given the lack of specific treatments to reduce blood-induced synovitis, the prevention of bleeding is pivotal to the maintenance of joint health. Prophylactic coagulation factor replacement therapy using extended half-life recombinant drugs has significantly improved patients' quality of life by reducing the burden of intravenous injections, and the more recent introduction of nonreplacement therapies such as subcutaneous emicizumab injections has improved treatment adherence and led to the greater protection of patients with hemophilia A. However, despite these advances, chronic arthropathy is still a significant problem. The introduction of point-of-care ultrasound imaging has improved the diagnosis of acute hemarthrosis and early hemophilic arthropathy, and allowed the better monitoring of progressive joint damage, but further research into the underlying mechanisms of the disease is required to allow the development of more targeted treatment. In the meantime, patient management should be based on the risk factors for the onset and progression of arthropathy of each individual patient, and all patients should be collaboratively cared for by multidisciplinary teams of hematologists, rheumatologists, orthopedic surgeons, and physiotherapists at comprehensive hemophilia treatment centers.
Asunto(s)
Hemofilia A , Sinovitis , Factor VIII , Hemartrosis/diagnóstico , Hemartrosis/etiología , Hemartrosis/terapia , Hemofilia A/complicaciones , Hemofilia A/diagnóstico , Hemofilia A/tratamiento farmacológico , Humanos , Calidad de Vida , Sinovitis/terapiaRESUMEN
PURPOSE: To investigate the safety and efficacy of genicular artery embolization for treatment of refractory hemarthrosis following total knee arthroplasty. MATERIAL AND METHODS: Patients who underwent genicular artery embolization with spherical embolics between January 2010 and March 2020 at a single institution were included if they had undergone total knee arthroplasty and subsequently experienced recurrent hemarthrosis. Technical success was defined as the significant reduction or elimination of the hyperemic blush. Clinical success was defined as the absence of clinical evidence of further hemarthrosis. Clinical follow-up was performed 7-14 days after the procedure and at 3-month intervals thereafter via a telephone interview. A total of 117 embolizations, comprising 82 initial, 28 first repeat, and 7 second repeat, were performed. RESULTS: An average of 2.5 arteries was treated per procedure. The superior lateral genicular artery was the most frequently embolized. The most utilized embolic size was 100-300 µm. Follow-up was available for all patients, with a median duration of 21.5 months. 65.9%, 25.6%, and 8.5% of patients underwent 1, 2, and 3 treatments, respectively. Complications occurred following 12.8% of treatments, of which the most common was transient cutaneous ischemia. Technical success was achieved in all cases. Clinical success was achieved in 56%, 79%, and 85% of patients following the first, second, and third treatment, respectively. 83% of patients reported being either satisfied or very satisfied with the overall result. CONCLUSIONS: Targeted genicular artery embolization with spherical embolics is an effective treatment for recurrent hemarthrosis with infrequent serious complications. Repeat embolization should be considered in cases of recurrence following initial therapy.
Asunto(s)
Artroplastia de Reemplazo de Rodilla , Hemartrosis , Arterias , Artroplastia de Reemplazo de Rodilla/efectos adversos , Hemartrosis/etiología , Hemartrosis/terapia , Humanos , Medición de Resultados Informados por el Paciente , RecurrenciaRESUMEN
In people with hemophilia, hematological prophylaxis during childhood and adolescence could elude the occurrence of musculoskeletal complications (in joints and muscles) if the concentration of the defective factor is averted from decreasing under 1% of normal. Prompt management is of capital significance as the juvenile skeleton is hypersensitive to the adverse events of the disease; intense structural defects might appear rapidly. Important, articular bleeds and inveterate hypertrophy of the articular synovial membrane must be treated vigorously to preclude joint degeneration (hemophilic arthropathy). At the moment that extreme joint disease is in place with intense affliction, the goal must be to reestablish activity whilst at the same time reducing the peril to the patient. Arthroscopic articular debridement is an efficacious surgical technique to accomplish this goal, particularly around the knee or ankle, and maybe contemplated to be a backup to ankle arthrodesis or ankle or knee replacement in patients of younger age. Eventually, joint replacement can commonly reestablish both articular mobility and function in an unhealthy articulation.
Asunto(s)
Hemofilia A , Adolescente , Tobillo , Artrodesis , Hemartrosis/etiología , Hemartrosis/terapia , Hemofilia A/complicaciones , Hemofilia A/terapia , HumanosAsunto(s)
Consenso , Hemofilia A , Hemofilia B , Factores de Coagulación Sanguínea/efectos adversos , Factores de Coagulación Sanguínea/uso terapéutico , Diagnóstico Diferencial , Factor IX/análisis , Factor IX/inmunología , Factor VIII/análisis , Factor VIII/inmunología , Femenino , Pruebas Genéticas , Hemartrosis/diagnóstico , Hemartrosis/etiología , Hemartrosis/terapia , Hemofilia A/complicaciones , Hemofilia A/diagnóstico , Hemofilia A/genética , Hemofilia A/terapia , Hemofilia B/complicaciones , Hemofilia B/diagnóstico , Hemofilia B/genética , Hemofilia B/terapia , Hemostasis/genética , Humanos , Isoanticuerpos/análisis , Estilo de Vida , Masculino , México , Cuidados Preoperatorios/métodosRESUMEN
INTRODUCTION: The effects of manual therapy (MT) on joint health and functional level in haemophilic arthropathy (HA) have relatively under-explored. AIM: To investigate the effects of MT in HA of lower limb joints on musculoskeletal system, functional level, Functional Independence Score in Hemophilia (FISH), Hemophilia Joint Health Score (HJHS) and kinesiophobia. METHODS: Seventeen patients were randomized to the control group (CG = 9) and manual therapy group (MTG = 8). The number of haemarthrosis (NoH) was recorded from the diaries. Range of motion (ROM), strength and pain intensity were measured using goniometer, digital dynamometer and visual analogue scale, respectively. Joint health was evaluated with HJHS, functional independence with FISH, and kinesiophobia with Tampa Scale. Functional Reach (FR), Timed Up-Go (TUG) and 5-Times Sit-to-Stand (5*STS) tests were used to evaluate functional level. Home exercises were given to both groups. Additionally, myofascial release techniques and mobilizations were applied to MTG. Interventions were applied 3 d/weekly for 5 weeks. Assessments were performed at baseline and after treatment. RESULTS: ROM, strength, activity pain, HJHS and FISH were improved in MTG (pË0.05). Ankle NoH, 5*STS and FR were developed in both groups (pË0.05), but kinesiophobia, TUG and knee NoH were not significant. Although improvements were observed in favour of MTG in HJHS, FR, activity pain and ankle strength/ROM (pË0.05), no significant difference was found in FISH, kinesiophobia, TUG, 5*STS, resting pain and knee strength. CONCLUSION: Reduction in ankle NoH indicated that both interventions were safe. The use of MT in HA of lower limb joints was an effective physiotherapy approach to improve functional level, joint health and functional independence.
Asunto(s)
Hemofilia A , Manipulaciones Musculoesqueléticas , Articulación del Tobillo , Hemartrosis/etiología , Hemartrosis/terapia , Hemofilia A/complicaciones , Hemofilia A/terapia , Humanos , Proyectos Piloto , Adulto JovenRESUMEN
PURPOSE: To retrospectively review the safety and efficacy of genicular artery embolization procedures performed at our institution in patients presenting with recurrent knee hemarthrosis following total knee arthroplasty (TKA). MATERIALS AND METHODS: A total of 13 consecutive patients (average age: 68; range 51-84, 62% female) were identified who underwent 14 genicular artery embolization procedures after presenting with recurrent hemarthrosis after TKA. Patient charts were retrospectively reviewed for demographic information, pre-embolization investigations, and details of embolization procedure including complications, technical success, and clinical success. Each patient had failed initial conservative therapy and all patients had a diagnostic aspiration performed by the referring physician prior to the procedure. The average time between TKA and embolization in our cohort was 21 months. RESULTS: All procedures performed were technically successful, defined as elimination of periprosthetic hypervascular blush. An average of 3.6 genicular vessels were embolized in each patient; 355 to 500 µm polyvinyl alcohol (PVA) particles were used in each case. There were no cases of transient cutaneous ischemia, skin erythema, or skin necrosis. Clinical success was obtained in 85.7% of cases, defined by elimination of the presenting clinical symptoms (knee pain and swelling) during continued follow-up by the referring clinician. CONCLUSION: Particle embolization is a safe and effective treatment for recurrent hemarthrosis after arthroplasty and our experience suggests that utilizing particle sizes of greater than 300 µm appears to be important in order to avoid cutaneous ischemic complications.