RESUMO

OBJECTIVE: The number of participants in sports or some form of recreation globally has led to an increase in the incidence of anterior cruciate ligament (ACL) injuries and the number of surgeries performed. Although it does not belong to risky surgical interventions, this operation is accompanied by complications that slow down post-operative rehabilitation. The objective is to analyze the effects of intra-articular (IA) injection of tranexamic acid (TXA) on the reduction of post-operative drained blood volume, pain intensity, and incidence of hemarthrosis after ACL reconstruction. METHODS: This prospective research included 124 patients undergoing ACL reconstruction surgery, randomly divided into two groups. The TXA group received IA TXA, whereas an equal amount of placebo was administered using the same route in the control group. RESULTS: The research has shown that IA injection of TXA effectively reduces post-operative blood loss (TXA group 71.29 ± 40.76 vs. control group 154.35 ± 81.45), reducing the intensity of post-operative pain (p < 0.001) and the incidence of hemarthrosis. CONCLUSION: The application of TXA significantly reduced post-operative bleeding and pain intensity, which accelerated the post-operative period.

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OBJETIVO: El mayor número de participantes en deportes o alguna forma de recreación en todo el mundo ha llevado a un aumento en la incidencia de lesiones del ligamento cruzado anterior (LCA) y de las cirugías realizadas. Aunque no es una intervención quirúrgica de riesgo, esta operación va acompañada de complicaciones que ralentizan la rehabilitación posoperatoria. El objetivo es analizar los efectos de la inyección intraarticular de ácido tranexámico (TXA) sobre la reducción del volumen sanguíneo drenado posoperatorio, la intensidad del dolor y la incidencia de hemartrosis tras la reconstrucción del LCA. MÉTODO: Esta investigación prospectiva incluyó 124 pacientes sometidos a cirugía de reconstrucción del LCA, divididos aleatoriamente en dos grupos: uno recibió TXA intraarticular y otro (grupo de control) una cantidad igual de placebo por la misma vía. RESULTADOS: La investigación ha demostrado que la inyección intraarticular de TXA reduce efectivamente la pérdida de sangre posoperatoria (grupo TXA 71.29 ± 40.76 vs. grupo control 154.35 ± 81.45), reduciendo la intensidad del dolor posoperatorio (p < 0.001) y la incidencia de hemartrosis. CONCLUSIONES: La aplicación de TXA redujo significativamente el sangrado posoperatorio y la intensidad del dolor, lo que aceleró el posoperatorio.

Assuntos

Reconstrução do Ligamento Cruzado Anterior , Antifibrinolíticos , Dor Pós-Operatória , Ácido Tranexâmico , Humanos , Ácido Tranexâmico/uso terapêutico , Ácido Tranexâmico/administração & dosagem , Reconstrução do Ligamento Cruzado Anterior/métodos , Antifibrinolíticos/uso terapêutico , Antifibrinolíticos/administração & dosagem , Feminino , Masculino , Estudos Prospectivos , Injeções Intra-Articulares , Adulto , Dor Pós-Operatória/prevenção & controle , Dor Pós-Operatória/tratamento farmacológico , Adulto Jovem , Hemorragia Pós-Operatória/prevenção & controle , Hemartrose/prevenção & controle , Hemartrose/etiologia , Lesões do Ligamento Cruzado Anterior/cirurgia , Método Duplo-Cego , Adolescente , Perda Sanguínea Cirúrgica/prevenção & controle

RESUMO

Background: Real-world data assessing treatment outcomes in patients with hemophilia A in routine clinical practice are limited. Objective: To evaluate the effectiveness and safety of octocog alfa in patients with moderate/severe hemophilia A receiving treatment in clinical practice. Design: The international Antihemophilic Factor Hemophilia A Outcome Database study is an observational, noninterventional, prospective, multicenter study. Methods: This planned interim data read-out was conducted following 7 years of observation of patients receiving octocog alfa (cut-off, 30 June 2020). The primary endpoint was joint health status, assessed by the Gilbert Score. Secondary endpoints included annualized bleeding rates (ABRs), Hemophilia Joint Health Score (HJHS), health-related quality of life, consumption, and safety. This post hoc analysis stratified data by hemophilia severity at baseline [moderate, factor VIII (FVIII) 1-5%; severe, FVIII <1%]. Results: Of the 711 patients in this analysis, 582 (82%) were receiving prophylaxis with octocog alfa at enrollment, and 498 (70%) had severe disease. Median Gilbert Scores were higher with on-demand therapy versus prophylaxis and scores were comparable in moderate and severe disease. In patients receiving prophylaxis, there was an improvement in HJHS Global Gait Score over 7 years of follow-up overall and in patients with severe disease. ABRs and annualized joint bleeding rates were low across all 7 years. An ABR of zero was reported in 34-56% of prophylaxis patients versus 20-40% in the on-demand group. ABRs were similar in severe and moderate disease. In total, 13/702 (1.9%) patients experienced 18 treatment-related adverse events. Conclusion: These data demonstrate the long-term effectiveness and safety of octocog alfa in patients with moderate and severe hemophilia A, especially in those receiving prophylaxis. The high number of patients receiving on-demand treatment experiencing zero bleeds could be due to selection bias within the study, with patients with less severe disease more likely to be receiving on-demand treatment. Trial registration: ClinicalTrials.gov: NCT02078427.

RESUMO

Objective: To describe the efficacy of using viscosupplementation in patients with hemophilic arthropathy (HA), on pain, limb functionality, and quality of life. Methods: A systematic review of the literature was performed following the PRISMA guidelines without limitations of language or year of publication. The search was performed on the following medical databases: PubMed, Cochrane Library, EMBASE, BVS/BIREME, Scopus, Web of Science, EBSCOhost, and PROQUEST in April 2020. The search used the following word: (hemophilia AND joint diseases) OR (haemophilic arthropathy OR hemophilic arthropathy) AND viscosupplementation. Results: The systematic review identified 127 articles, 10 of which were selected for data extraction and qualitative analysis. The 10 selected articles included 297 joints with HA in 177 hemophilic subjects. Our review showed positive results in alleviating pain and improving functional capacity, and quality of life. No major adverse effects were observed. Conclusion: There is a lack of scientific evidence regarding viscosupplementation with hyaluronic acid, but the results presented in this research suggest that it is an effective and safe therapeutic option to alleviate pain and improve functional capacity in patients with HA. Level of Evidence II, Systematic Review.

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Objetivo: Descrever o uso da viscossuplementação com ácido hialurônico em pacientes com artropatia hemofílica (HA), sua eficácia na dor, a funcionalidade do membro e a qualidade de vida após sua aplicação. Métodos: Revisão sistemática da literatura (RSL) que seguiu as diretrizes PRISMA, sem limitação de idioma ou ano de publicação. A pesquisa foi realizada em abril de 2020 nas seguintes bases de dados médicas: PubMed, Cochrane Library, EMBASE, BVS/BIREME, Scopus, Web of Science, EBSCOhost e ProQuest. A estratégia de pesquisa foi: (hemofilia AND joint disease) OR (artropatia hemofílica OU artropatia hemofílica) E viscossuplementação. Resultados: A RSL identificou 127 artigos, dos quais 10 foram selecionados para extração de dados e análise qualitativa. Os 10 artigos selecionados incluíram 297 articulações com AH em 177 indivíduos hemofílicos. Nossa revisão mostrou resultados positivos na melhora da dor, na capacidade funcional e na qualidade de vida. Não foram observados efeitos adversos importantes. Conclusão: A evidência científica atual a respeito da viscossuplementação com ácido hialurônico é escassa, mas os resultados apresentados nesta pesquisa sugerem que é uma opção terapêutica eficaz e segura para diminuir a dor e melhorar a capacidade funcional em pacientes com AH. Nível de Evidência II, Revisão Sistemática.

RESUMO

ABSTRACT Objective: To describe the efficacy of using viscosupplementation in patients with hemophilic arthropathy (HA), on pain, limb functionality, and quality of life. Methods: A systematic review of the literature was performed following the PRISMA guidelines without limitations of language or year of publication. The search was performed on the following medical databases: PubMed, Cochrane Library, EMBASE, BVS/BIREME, Scopus, Web of Science, EBSCOhost, and PROQUEST in April 2020. The search used the following word: (hemophilia AND joint diseases) OR (haemophilic arthropathy OR hemophilic arthropathy) AND viscosupplementation. Results: The systematic review identified 127 articles, 10 of which were selected for data extraction and qualitative analysis. The 10 selected articles included 297 joints with HA in 177 hemophilic subjects. Our review showed positive results in alleviating pain and improving functional capacity, and quality of life. No major adverse effects were observed. Conclusion: There is a lack of scientific evidence regarding viscosupplementation with hyaluronic acid, but the results presented in this research suggest that it is an effective and safe therapeutic option to alleviate pain and improve functional capacity in patients with HA. Level of Evidence II, Systematic Review.

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RESUMO Objetivo: Descrever o uso da viscossuplementação com ácido hialurônico em pacientes com artropatia hemofílica (HA), sua eficácia na dor, a funcionalidade do membro e a qualidade de vida após sua aplicação. Métodos: Revisão sistemática da literatura (RSL) que seguiu as diretrizes PRISMA, sem limitação de idioma ou ano de publicação. A pesquisa foi realizada em abril de 2020 nas seguintes bases de dados médicas: PubMed, Cochrane Library, EMBASE, BVS/BIREME, Scopus, Web of Science, EBSCOhost e ProQuest. A estratégia de pesquisa foi: (hemofilia AND joint disease) OR (artropatia hemofílica OU artropatia hemofílica) E viscossuplementação. Resultados: A RSL identificou 127 artigos, dos quais 10 foram selecionados para extração de dados e análise qualitativa. Os 10 artigos selecionados incluíram 297 articulações com AH em 177 indivíduos hemofílicos. Nossa revisão mostrou resultados positivos na melhora da dor, na capacidade funcional e na qualidade de vida. Não foram observados efeitos adversos importantes. Conclusão: A evidência científica atual a respeito da viscossuplementação com ácido hialurônico é escassa, mas os resultados apresentados nesta pesquisa sugerem que é uma opção terapêutica eficaz e segura para diminuir a dor e melhorar a capacidade funcional em pacientes com AH. Nível de Evidência II, Revisão Sistemática.

RESUMO

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)

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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)

Assuntos

Humanos , Anormalidades Congênitas , Hemartrose/terapia , Hemofilia A/terapia

RESUMO

SUMMARY OBJECTIVE: Postoperative bleeding is one of the most important factors affecting clinical and functional results in total knee arthroplasty. Therefore, many studies have been conducted on bleeding in arthroplasty patients. However, there are very few reports investigating the effect of patellar surface replacement on bleeding in knee arthroplasty. We, therefore, aimed to investigate the effect of patellar surface replacement on postoperative bleeding. METHODS: In this retrospective study, 30 with patellar resurfacing were compared with 39 without patellar resurfacing among patients who had undergone total knee replacement due to primary osteoarthritis. Demographic data, amount of transfusion, preoperative and postoperative hemoglobin and hematocrit values, and total, visible, and hidden blood loss values were recorded. RESULTS: No statistical difference was found between the two groups in terms of demographic values. There was no significant difference between the groups in terms of the amount of blood in the drain, total blood loss, hidden blood loss, and blood transfusion in patients who had and had not undergone patellar resurfacing. A positive significant correlation was found between postoperative drainage volume and total blood loss. CONCLUSION: Patellar component application in patients who had undergone total knee arthroplasty does not change the blood loss of the patients.

RESUMO

ABSTRACT Introduction: The radiosynovectomy (RS) is one treatment option for recurrent hemarthrosis in patients with hemophilia (PWH). A prospective cohort study was designed to evaluate the effects of the RS on the synovial membrane volume in the ankles and knees of PWH and patient characteristics related to the RS outcome. Methods: In a one-year follow-up, 25 joints of 22 PWH who presented 3 bleeds or more in the same joint over the last 6 months (target joints) were subjected to the RS. Two groups were compared: those who retained target joints following the RS and those who did not (less than 3 bleeds/6 months after the RS). The groups were analyzed according to age, hemophilia type/severity, joint, body mass index (BMI), inhibitor and Hemophilia Joint Health Score 2.1 (HJHS). The magnetic resonance images (MRI) of six ankles and six knees were acquired prior to, and 6 months after, the RS. The synovial membrane volume and arthropathy MRI scale were accessed and volumes were compared and correlated with the Yttrium-90 dose injected. Results: Patients with a mean age of 12 years and a mean HJHS of 6.7 (p < 0.05) retained target joints after the RS. The inhibitor, joint, type/severity of disease and BMI showed no significant differences between groups. The synovial membrane volume had a significant reduction after the RS (p = 0.03), but no correlation with the Yttrium-90 dose. In proportion to the synovial membrane volume, doses injected to the ankles were larger than those injected to the knees. Conclusion: The synovial membrane volume is reduced after the RS, regardless of the effective 90Y dose.

Assuntos

Humanos , Criança , Adolescente , Adulto , Sinovite , Imageamento por Ressonância Magnética , Sinovectomia , Hemartrose , Hemofilia A , Artropatias

RESUMO

INTRODUCTION: Radiosynovectomy (RS) with 90Y-hydroxyapatite (90Y-HyA) aims to control knee hemarthrosis in hemophiliac patients to prevent secondary arthropathy. However, knee RS using 153Sm-hydroxyapatite (153Sm-HyA) is considered less suitable due to the lower average soft tissue range and energy of 153Sm for large joints, such as the knees. PURPOSE: The objective of this investigation was to assess the efficacy and safety of knee RS with 153Sm-HyA, compared to 90Y-HyA. METHODS: Forty patients were prospectively assigned to undergo knee RS with 153Sm-HyA (n = 19) or with 90Y-HyA (n = 21). The frequency of hemarthrosis episodes before and after treatment were compared. RESULTS: After six months of knee RS, 153Sm-HyA and 90Y-HyA promoted a similar reduction of hemarthrosis episodes (50% and 66.7%, respectively). However, after 12 months of knee RS, the reduction of hemarthrosis episodes was significantly (p = 0.037) higher using 153Sm-HyA (87.5%) compared to 90Y-HyA (50.0%). This discrepancy was more pronounced (p = 0.002) for 153Sm-HyA compared to 90Y-HyA in adults/adolescents. CONCLUSION: Knee radiosynovectomy with 153Sm-HyA is safe, reduces hemarthrosis episodes after 12 months of treatments, especially in adults/adolescents and even with grades III/IV arthropathy, similar to 90Y-HyA. 90Y-HyA seems to promote better hemarthrosis control in small children.

Assuntos

Durapatita/química , Hemartrose/radioterapia , Articulação do Joelho/efeitos da radiação , Radioisótopos/química , Samário/química , Radioisótopos de Ítrio/química , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Radioisótopos/efeitos adversos , Radioisótopos/uso terapêutico , Medição de Risco , Samário/efeitos adversos , Samário/uso terapêutico , Fatores de Tempo , Resultado do Tratamento , Radioisótopos de Ítrio/efeitos adversos , Radioisótopos de Ítrio/uso terapêutico

RESUMO

INTRODUCTION: The radiosynovectomy (RS) is one treatment option for recurrent hemarthrosis in patients with hemophilia (PWH). A prospective cohort study was designed to evaluate the effects of the RS on the synovial membrane volume in the ankles and knees of PWH and patient characteristics related to the RS outcome. METHODS: In a one-year follow-up, 25 joints of 22 PWH who presented 3 bleeds or more in the same joint over the last 6 months (target joints) were subjected to the RS. Two groups were compared: those who retained target joints following the RS and those who did not (less than 3 bleeds/6 months after the RS). The groups were analyzed according to age, hemophilia type/severity, joint, body mass index (BMI), inhibitor and Hemophilia Joint Health Score 2.1 (HJHS). The magnetic resonance images (MRI) of six ankles and six knees were acquired prior to, and 6 months after, the RS. The synovial membrane volume and arthropathy MRI scale were accessed and volumes were compared and correlated with the Yttrium-90 dose injected. RESULTS: Patients with a mean age of 12 years and a mean HJHS of 6.7 (p < 0.05) retained target joints after the RS. The inhibitor, joint, type/severity of disease and BMI showed no significant differences between groups. The synovial membrane volume had a significant reduction after the RS (p = 0.03), but no correlation with the Yttrium-90 dose. In proportion to the synovial membrane volume, doses injected to the ankles were larger than those injected to the knees. CONCLUSION: The synovial membrane volume is reduced after the RS, regardless of the effective 90Y dose.

RESUMO

Objetivo: avaliar a força muscular periférica em adultos portadores de hemofilia, por meio da dinamometria manual, e verificar a diferença da força muscular de acordo com a classificação de gravidade da hemofilia. Método: Participaram da pesquisa 20 homens divididos em 2 grupos, um com hemofílicos e um controle; foi aplicada uma ficha avaliativa seguida do teste de preensão palmar. Resultados: Quando comparados os dois grupos, foi observada diferença estatística significativa nas variáveis estatura (p=0,007) e força de preensão dos lados dominante (p=0,04) e não dominante (0,002), favorecendo o grupo controle; quando comparada a força de preensão dos hemofílicos com a doença leve e grave, houve diferença significativa para o lado não dominante (p=0,01). Conclusão: Pode-se associar a diminuição de força de preensão para o grupo de hemofílicos à sua condição de doença crônica hereditária. Entretanto, como o número de participantes foi pequeno, esses resultados sugestivos, mostram a necessidade de mais estudos sobre o tema

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Objective: to evaluate peripheral muscle strength in adults with hemophilia, through manual dynamometry, and to verify the difference in muscle strength according to the classification of hemophilia severity. Method: Twenty men participated in the research, divided into 2 groups, one with hemophiliacs and one control; an evaluation form was applied, followed by the handgrip test. Results: When the two groups were compared, a statistically significant difference was observed in the variables height (p=0.007) and grip strength of the dominant (p=0.04) and non-dominant (0.002) sides, favoring the control group; when comparing the grip strength of hemophiliacs with mild and severe disease, there was a significant difference for the non-dominant side (p=0.01). Conclusion: The decrease in grip strength for the group of hemophiliacs can be associated with their condition of hereditary chronic disease. However, as the number of participants was small, these suggestive results show the need for more studies on the subject.

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Objetivo: El objetivo del estudio fue evaluar la fuerza muscular periférica en adultos con hemofilia, mediante dinamometría manual, y verificar la diferencia en la fuerza muscular según la clasificación de severidad de la hemofilia. Método: Participaron de la investigación 20 hombres, divididos en 2 grupos, uno con hemofílicos y otro control; Se aplicó un formulario de evaluación, seguido de la prueba de agarre. Resultados: Al comparar los dos grupos, se observó una diferencia estadísticamente significativa en las variables altura (p = 0,007) y fuerza de agarre en los lados dominantes (p = 0,04) y no dominantes (0,002), favoreciendo al grupo control; al comparar la fuerza de agarre de los hemofílicos con la enfermedad leve y grave, hubo una diferencia significativa para el lado no dominante (p = 0,01). Conclusión: La disminución de la fuerza de prensión del grupo de hemofílicos puede estar asociada a su condición de enfermedad crónica hereditaria. Sin embargo, como el número de participantes fue pequeño, estos sugerentes resultados muestran la necesidad de más estudios sobre el tema.

Assuntos

Humanos , Hemofilia B , Força Muscular , Hemofilia A , Dinamômetro de Força Muscular , Hemartrose

RESUMO

BACKGROUND: Spontaneous bleedings occurring into joints (hemarthrosis) are the most common manifestations of hemophilia and causes severe joint damage ultimately resulting in joint disfunction known as hemophilic arthropathy. Among available therapeutic options for reducing recurrent hemarthrosis-associated damage, radiosynoviorthesis (RS) has proven effective in improving joint function. AIM: To assess the impact of RS with Yttrium(90) citrate (C-Y(90)) on frequency of hemarthroses and joint function in a group of pediatric patients. METHODS: Between November 1998 and February 2017, we evaluated 27 pediatric patients with mild, moderate or severe hemophilia with haemophilic arthropathy. Overall, RS was applied in 60 joints. Some patients received more than one single intra-articular injection with C-Y(90). RESULTS: During the follow-up, one patient showed joint bleeding 15 months after RS, one patient after 12 months and one patient after 45 days. The episodes of hemarthrosis were reduced and joint function significantly improved in all patients. CONCLUSION: RS with C-Y(90) is a simple and safe treatment for reducing the frequency of hemarthroses in patients with hemophilia. It decreases the use of factor VIII / IX and improves joint function.

Assuntos

Hemartrose , Hemofilia A , Criança , Hemartrose/etiologia , Hemartrose/radioterapia , Hemofilia A/complicações , Humanos

RESUMO

INTRODUCTION: Joint affection is a frequent complication of hemophilia. Virtual reality programs offer the patient a dynamic plan with maximum functional benefit. The objective of this work is to evaluate the effect of a rehabilitation program combined with conventional therapy and virtual reality on joint function, in patients with hemophilic arthropathy and the impact on the gait. MATERIAL AND METHODS: Eleven pediatric patients with hemophilia and history of hemophilic arthropathy grade I or II were evaluated. An 8-week rehabilitation program was carried out with Xbox KinectTM and mechanotherapy. Movement analysis laboratory to evaluate the gait pattern and the Joint Health Scale was performed at the beginning and end of the program. RESULTS: A reduction of 50% (p = 0.000), 68% (p = 0.016) and 48% (p = 0.000) were observed in the Total Joint Health score (STA), Global Gait Score (PMG) and Total Score of the Joint Health Scale (P-HJHS-T) respectively. There is a decrease in the support phase and right stride, and increase in cadence, step length, left stride and swing phase. CONCLUSIONS: The overall improvement of the patients makes us conclude that this pilot study opens a window of opportunity to continue the study of an integral rehabilitation program based on the use of virtual reality in pediatric patients with hemophilic arthropathy.

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INTRODUCCIÓN: La afección articular es una complicación frecuente de la hemofilia. Los programas de realidad virtual ofrecen al paciente pediátrico un esquema dinámico con máximo beneficio funcional. El objetivo de este trabajo es evaluar el efecto de un programa de rehabilitación combinado con terapia convencional y realidad virtual sobre la función articular en pacientes con artropatía hemofílica y el impacto sobre el patrón de la marcha. MATERIAL Y MÉTODOS: Se evaluaron 11 pacientes pediátricos con hemofilia A o B y antecedente de artropatía hemofílica en grado I o II. Se sometieron a un programa de rehabilitación de ocho semanas con Xbox KinectTM y mecanoterapia. Se utilizó el laboratorio de análisis de movimiento para evaluar el patrón de marcha y la escala de salud articular al inicio y término del programa. RESULTADOS: Se redujo 50% (p = 0.000), 68% (p = 0.016) y 48% (p = 0.000) el puntaje de la escala de salud total articular (STA), puntaje marcha global (PMG) y puntaje total de la escala de salud articular (P-HJHS-T) respectivamente. Se observó disminución en la fase de apoyo y zancada derecha y aumento en cadencia, longitud de paso, zancada izquierda y fase de balanceo. CONCLUSIONES: La mejoría global de los pacientes nos hace concluir que este estudio piloto abre una ventana de oportunidades para continuar el estudio de un programa integral de rehabilitación con base en el uso de la realidad virtual en pacientes pediátricos con artropatía hemofílica.

Assuntos

Hemofilia A , Realidade Virtual , Criança , Hemartrose , Hemofilia A/complicações , Humanos , Projetos Piloto , Estudos Retrospectivos

RESUMO

BACKGROUND: The pathophysiology of hemophilic arthropathy is complex and not completely understood. In this study, we aimed to identify biomarkers that can affect the hemophilic arthropathy severity. METHODS: Fifty patients were analyzed for biomarker frequencies; in 37 patients, articular symptoms were evaluated based on the physical joint examination score, and in 18, it was based on magnetic resonance imaging. Eight polymorphisms, namely FV 1691G>A, FII 20210G>A, MTHFR 677C>T and 1298A>C, TNFα-308G>A and -238G>A, ACAN VNTR, and IL1RN*2-VNTR were identified. RESULTS: Patients with the MTHFR 677TT genotype showed a higher number of affected joints (1.83 ± 0.9 vs. 0.55 ± 0.7 for CC; p = .023), whereas those with the MTHFR 1298AC genotype exhibited higher effusion according to two radiologists (0.90 ± 0.31/1.20 ± 0.63 vs. 0.38 ± 0.52/0.50 ± 0.53 for AA genotype; p = .043/0.036, respectively). In addition, patients with the TNFα-308GA genotype had more subchondral cysts (0.75 ± 0.95 vs. 0.07 ± 0.26 for GG genotype; p = .041). CONCLUSIONS: The distribution of risk genotypes for MTHFR and TNFα-308GA suggests their association with clinical parameters of hemophilic arthropathy. Cohort studies are essential to verify these associations.

Assuntos

Cartilagem/patologia , Marcadores Genéticos , Hemartrose/diagnóstico , Hemofilia A/fisiopatologia , Inflamação/diagnóstico , Adolescente , Cartilagem/metabolismo , Criança , Pré-Escolar , Feminino , Hemartrose/epidemiologia , Hemartrose/genética , Humanos , Incidência , Lactente , Recém-Nascido , Inflamação/epidemiologia , Inflamação/genética , Masculino , México/epidemiologia , Prognóstico

RESUMO

Background: In patients with hemophilia, radionuclide synoviorthesis, or the intra-articular injection of a radionuclide to decrease the synovial hypertrophy tissue, aims to decrease or avoid hemarthrosis. Aim: To evaluate the effectiveness of radionuclide synoviorthesis in hemophilia. Material and Methods: Observational retrospective study of the evolution of 107 male patients aged 3 to 54 years who were subjected to radionuclide synoviorthesis between 2007 and 2015. Results: Of 164 treated joints, in 65% treatment was successful, (defined as zero to two hemarthroses and absence of synovitis during the follow up period), in 17% it was partially successful (defined as two or less hemarthroses, but persistence of the synovitis) and failed in 18% of the procedures. No important complications were recorded. Conclusions: Radionuclide synoviorthesis has an overall 82% success rate, is minimally invasive, can be used at any age and is inexpensive We recommend its implementation in Chilean hemophilia treatment centers.

Assuntos

Humanos , Masculino , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Radioisótopos/administração & dosagem , Rênio/uso terapêutico , Sinovite/terapia , Radioisótopos de Ítrio/uso terapêutico , Hemartrose/terapia , Hemofilia A/terapia , Sinovite/fisiopatologia , Sinovite/diagnóstico por imagem , Fatores de Tempo , Reprodutibilidade dos Testes , Estudos Retrospectivos , Resultado do Tratamento , Hemartrose/fisiopatologia , Hemartrose/diagnóstico por imagem , Hemofilia A/fisiopatologia , Injeções Intra-Articulares

RESUMO

Introduction: A hemophilia is a chronic disease of genetic origin caused by a mutation of the genes which encode blood clotting factors. One of the consequences of this alteration is the involvement of the musculoskeletal system, which may influence the posture of these individuals. Objective:To estimate the frequency of postural changes in hemophilia patients, and identify possible factors associated. Methods:A cross-sectional descriptive study, conducted with individuals aged ≥ 18 years,with diagnosis of hemophilia,in follow-up at the Hematology and Hemotherapy Foundation of Bahia (HEMOBA).The participants were submitted to postural evaluation,through visual analysis, Adams test and the application of a semi-structured form.Epi Info® (v.3.5.2) was used for data analysis and to verify the existence of associations between the variables of the study were used the Exact Fisher, Qui-Square and Qui-Square Tests with the Yates correction and considered as statistically significant associations with p <0.05.Results:Twenty-nine hemophiliacs participated in this study, with mean age of 34.98 ± 12,6 years, 25 (86.2%) reported having hemophilic arthropathy. Of the 13 research participants (68.4%) presented scoliotic posture detected by the Adams test.The change was more frequent in individuals with more than one joint affected by hemophilic arthropathy, with a statistically significant value for this variable (p = 0.039).Conclusion:The frequencies of postural changes in individuals with hemophilia are elevated with a significant association for individuals over 40 years of age and with with more than one joint affected by haemophilic arthropathy. (AU)

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Introdução: A hemofilia é uma doença crônica de origem genética causada por uma mutação dos genes que codificam os fatores de coagulação sanguíneos. Uma das consequências dessa alteração é o acometimento do sistema musculoesquelético, o que pode influenciar na postura desses indivíduos. Objetivo: Estimar a frequência de alterações posturais em portadores de hemofilia, e identificar possíveis fatores associados. Métodos: Estudo descritivo de corte transversal, conduzido com indivíduos com idade ≥ 18 anos, com diagnóstico de hemofilia, em acompanhamento na Fundação de Hematologia e Hemoterapia da Bahia (HEMOBA). Os participantes foram submetidos à avaliação postural, por meio da análise visual, teste de Adams e aplicação de um formulário semi-estruturado. Empregou-se o Epi Info® (v.3.5.2) para análise dos dados e, para verificar a existência de associações entre as variáveis do estudo foram utilizados os Testes exato de Fisher, Qui-quadrado (bicaudal) e Qui-Quadrado com a correção de Yates. Consideradas como estatisticamente significantes associações com p <0,05. Resultados: Participaram deste estudo 29 hemofílicos, com média de idade de 34,9 ± 12,6 anos, 25 (86,2%) informaram ter artropatia hemofílica. Dos participantes da pesquisa 13 (68,4%) apresentaram postura escoliótica detectada pelo teste de Adams. A alteração foi mais frequente nos indivíduos com mais de uma articulação afetada pela artropatia hemofílica, com valor estatisticamente significativo para esta variável (p= 0,039). Conclusão: As frequências de alterações posturais em indivíduos com hemofilia são elevadas com associação significativa para os indivíduos com idade superior a 40 anos e com mais de uma articulação afetada pela artropatia hemofílica. (AU)

Assuntos

Hemartrose , Hemofilia A , Postura

RESUMO

The pigmented vellonodular sinovitis (PVNS) is benign neoplasm with synovial proliferation and hemosiderin deposit, characterized by large compromising joints, especially the knee. At present, two variants of clinics, the diffuse form (PVNSD) and the localized (PVNSL) are described. Arthroscopic synovectomy and radiosynoviorthesis (RSO) is the treatment that has shown the best functional results. Nuclear magnetic resonance is an appropriate method for conducting the diagnosis of PVNSL.

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La sinovitis vellonodular pigmentada (SVP) es una neoplasia benigna con proliferación sinovial y depósito de hemosiderina, se caracteriza por comprometer grandes articulaciones, en especial la rodilla. En la actualidad se describen dos variantes clínicas, la forma difusa (SVPD) y la localizada (SVPL). La sinovectomía artroscópica y sinovectomía por radiación conforman el tratamiento que ha demostrado mejores resultados funcionales. La resonancia magnética nuclear es un método adecuado para establecer el diagnóstico de la SVPL.

Assuntos

Hemartrose , Sinovite Pigmentada Vilonodular , Artroscopia , Hemartrose/diagnóstico por imagem , Humanos , Articulação do Joelho , Imageamento por Ressonância Magnética , Estudos Retrospectivos , Sinovectomia , Sinovite Pigmentada Vilonodular/diagnóstico por imagem

RESUMO

Type B hemophilia usually affects patients with a family history of this disease and has a typical clinical picture. However, in the present case it appeared in a patient outside the typical age with no family history of hematologic malignancies and with an unusual clinical picture.

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A hemofilia do tipo B afeta normalmente pacientes com história familiar positiva para a doença e se apresenta com quadro clínico típico. No presente caso, no entanto, o diagnóstico se deu em um paciente fora da idade típica, sem histórico familiar de doenças hematológicas e quadro clínico diferente do habitual.

RESUMO

ABSTRACT Type B hemophilia usually affects patients with a family history of this disease and has a typical clinical picture. However, in the present case it appeared in a patient outside the typical age with no family history of hematologic malignancies and with an unusual clinical picture.

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RESUMO A hemofilia do tipo B afeta normalmente pacientes com história familiar positiva para a doença e se apresenta com quadro clínico típico. No presente caso, no entanto, o diagnóstico se deu em um paciente fora da idade típica, sem histórico familiar de doenças hematológicas e quadro clínico diferente do habitual.

Assuntos

Humanos , Masculino , Pré-Escolar , Articulação do Tornozelo , Hemartrose , Hemofilia B

RESUMO

Resumen: La sinovitis vellonodular pigmentada (SVP) es una neoplasia benigna con proliferación sinovial y depósito de hemosiderina, se caracteriza por comprometer grandes articulaciones, en especial la rodilla. En la actualidad se describen dos variantes clínicas, la forma difusa (SVPD) y la localizada (SVPL). La sinovectomía artroscópica y sinovectomía por radiación conforman el tratamiento que ha demostrado mejores resultados funcionales. La resonancia magnética nuclear es un método adecuado para establecer el diagnóstico de la SVPL.

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Abstract: The pigmented vellonodular sinovitis (PVNS) is benign neoplasm with synovial proliferation and hemosiderin deposit, characterized by large compromising joints, especially the knee. At present, two variants of clinics, the diffuse form (PVNSD) and the localized (PVNSL) are described. Arthroscopic synovectomy and radiosynoviorthesis (RSO) is the treatment that has shown the best functional results. Nuclear magnetic resonance is an appropriate method for conducting the diagnosis of PVNSL.

Assuntos

Humanos , Sinovite Pigmentada Vilonodular/diagnóstico por imagem , Hemartrose/diagnóstico por imagem , Artroscopia , Imageamento por Ressonância Magnética , Estudos Retrospectivos , Sinovectomia , Articulação do Joelho

RESUMO

BACKGROUND: Hemophilia is a potentially disabling condition as hemophilic arthropathy develops early in life and is progressive, especially in patients treated in an on-demand regime. OBJECTIVE: This study aimed to describe the structural joint status and the functional independence score of hemophiliac adults and correlate structural damage with the functional deficits found in these patients. METHODS: Hemophiliacs at the Juiz de Fora Regional Blood Center - HEMOMINAS Foundation, aged 18 years and over and treated in an on-demand regime, were clinically evaluated in respect to structural joint damage using the World Federation of Hemophilia Physical Examination Scale (WFH-PE) and functional deficits using the Functional Independence Score in Hemophilia (FISH). The Spearman rank test was used to evaluate the correlation between the two scores. RESULTS: Thirty-nine patients were evaluated. The mean age was 36.8 years. Target joints were detected in 69.2% of patients studied. The mean Physical Examination Scale and Functional Independence Score were 16.87 and 25.64, respectively. Patients with mild hemophilia showed no significant joint involvement. Patients with severe or moderate hemophilia had similar results regarding structural damage (p-value < 0.001) and functional deficits (p-value = 0.001). There was statistical significance in the correlation between the two scores (r = -0.850; p-value = 0.01). CONCLUSIONS: The World Federation of Hemophilia Physical Examination Scale and Functional Independence Score in Hemophilia may be useful to clinically assess structural joint damage and functional deficits in hemophiliacs as the tools are inexpensive and easy to administer and may be able to detect hemophilic arthropathy, which results from recurrent hemarthrosis and is common in the population studied.