Analysis of the effects of low/intermediate dose of coagulation factor Ⅷ on 30 adult patients with severe hemophilia A in a single center / 中华血液学杂志

Objective: To evaluate the clinical effects of low- and intermediate-dose factor Ⅷ (F Ⅷ) prophylaxis in Chinese adult patients with severe hemophilia A. Methods: Thirty adult patients with severe hemophilia A who received low- (n=20) /intermediate-dose (n=10) F Ⅷ prophylaxis at Nanjing Drum Tower Hospital affiliated with Nanjing University Medical College were included in the study. The annual bleeding rate (ABR), annual joint bleeding rate (AJBR), number of target joints, functional independence score of hemophilia (FISH), quality of life score, and health status score (SF-36) before and after preventive treatment were retrospectively analyzed and compared. Results: The median follow-up was 48 months. Compared with on-demand treatment, low- and intermediate-dose prophylaxis significantly reduced ABR, AJBR, and the number of target joints (P<0.05) ; the improvement in the intermediate-dose prophylaxis group was better than that in the low-dose prophylaxis group (P<0.05). Compared with on-demand treatment, the FISH score, quality of life score, and SF-36 score significantly improved in both groups (P<0.05), but there was no significant difference between the two groups (P>0.05) . Conclusion: In Chinese adults with severe hemophilia A, low- and intermediate-dose prophylaxis can significantly reduce bleeding frequency, delay the progression of joint lesions, and improve the quality of life of patients as compared with on-demand treatment. The improvement in clinical bleeding was better with intermediate-dose prophylaxis than low-dose prophylaxis.

Therapeutic options for hemarthrosis in severe hemophilic patients / Opções terapêuticas para as hemartroses em pacientes hemofílicos graves

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)

Avaliação do perfil clínico-terapêutico e da resposta à profilaxia dos portadores de hemofilia A e B em um centro de referência no Ceará / Evaluation of the clinical-therapeutic profile and the response to prophylaxis of patients with hemophilia A and B in a reference center in Ceará

Objetivo: Avaliar o perfil clínico-terapêutico e a resposta à profilaxia em pacientes hemofílicos A e B em um centro de referência no Ceará. Métodos: Estudo de coorte retrospectivo, com dados de 133 hemofílicos A e B, em profilaxia entre 2016 e 2021, por meio de prontuários médicos e sistema Web Coagulopatias. Resultados: Os pacientes todos do sexo masculino em sua maioria foram hemofílicos A (93,2%), na forma grave, residentes em Fortaleza, com maior prevalência do município de Guaiúba. A maioria fazia uso de Fator VIII recombinante e em profilaxia secundária, em relação ao comprometimento articular a maioria não apresentou relato de hemartroses (66,9%), articulação-alvo (87,9%) ou artropatia (54,9%), porém os hemofílicos em profilaxia terciária apresentaram um maior comprometimento articular em relação a profilaxia primária e secundária. Verificou-se uma correlação negativa entre o tempo de profilaxia e a dose de fator utilizada, demonstrando que quanto maior o tempo de profilaxia menor a dose do fator utilizada. Um total de 13 hemofílicos A grave desenvolveram inibidor de fator VIII realizando imunotolerância (ITI) com sucesso total em 84,6%. Por meio da curva ROC, foi verificado uma associação entre a necessidade de ITI e a dose de fator de coagulação, com acurácia de 67,7% de que o uso de doses maiores de fator predispõe ao desenvolvimento de inibidores. Conclusão: Os dados do estudo permitem inferir que quanto mais precoce o tratamento de profilaxia menor é comprometimento articular, dose do fator utilizada e menor predisposição de desenvolver inibidores dos fatores da coagulação.

Objective: to evaluate the clinical-therapeutic profile and response to prophylaxis in hemophiliac A and B patients at a referral center in Ceará. Methods: Retrospective cohort study, with data from 133 hemophiliacs A and B, undergoing prophylaxis between 2016 and 2021, using medical records and the Web Coagulopathies system. Results: Most of the patients were male patients with severe hemophilia A (93.2%), residing in Fortaleza, with a higher prevalence in the city of Guaiúba. Most made use of recombinant Factor VIII and in secondary prophylaxis, in relation to joint involvement, the majority did not report hemarthroses (66.9%), target joint (87.9%) or arthropathy (54.9%). however, hemophiliacs on tertiary prophylaxis showed greater joint impairment in relation to primary and secondary prophylaxis. There was a negative correlation between the prophylaxis time and the factor dose used, demonstrating that the longer the prophylaxis time, the lower the factor dose used. A total of 13 severe A hemophiliacs developed factor VIII inhibitor performing immunotolerance (ITI) with total success in 84.6%. Using the ROC curve, an association was verified between the need for ITI and the dose of coagulation factor, with an accuracy of 67.7% that the use of higher doses of factor predisposes to the development of inhibitors. Conclusion: The study data allow us to infer that the earlier the prophylaxis treatment, the less joint impairment, the dose of the factor used and the less predisposition to develop coagulation factor inhibitors.

Perfil epidemiológico de pacientes com Hemofilia A e doenças associadas ao uso do fator VIII/recombinante / Epidemiological profile of patients with Hemophilia A and diseases associated with the use of factor VIII/recombinant

Introdução: o Brasil é o quarto país em casos de Hemofilia A. O tratamento é infundir o fator de coagulação ausente. Reações ao uso do fator podem incluir manifestações alérgicas, doenças virais transfusionais e aloanticorpos. Objetivo: analisar o perfil epidemiológico de pacientes com Hemofilia A, e as doenças associadas ao uso do fator VIII e fator VIII recombinante. Metodologia: estudo transversal descritivo e retrospectivo. A coleta de dados foi realizada nos prontuários de pacientes com diagnóstico de hemofilia A, preenchidos com mais de 70% das informações, na Fundação Hospitalar de Hematologia e Hemoterapia do Amazonas, Brasil. Resultados: o Ministério da Saúde identificou no Estado do Amazonas, o registro de 276 indivíduos com diagnóstico de Hemofilia A. Incluídos para análise neste estudo 164 prontuários. Características sociodemográficas: homens 99,4%, adolescentes (28%) e jovens (26,8%); de cor parda 67,1%, ensino fundamental incompleto 28,6% e, exercendo a ocupação de estudante 42,7%. Condição clínica: 36,6% classificados com hemofilia A grave. Todos os pacientes tiveram diagnóstico clínico e laboratorial. O parentesco mais comum é o de irmãos com 35,3%. Sintomas predominantes: hemartrose 45,4%; dor 31,9%; edema 24% e artropatia 8,5%. O fator VIII recombinante, administrado em 34,8% dos pacientes, enquanto o fator VIII plasmático em 28,0%. Administrados doses de 2000UI a 2999UI. As complicações: artralgia 77,4% e hemorragia 77,4%. Conclusão: cuidados qualificados dos profissionais de saúde auxiliam na prevenção de complicações sérias, resultando em qualidade de vida ao hemofílico.

Introduction: Brazil is the fourth country in cases of Hemophilia A. The treatment is to infuse the missing clotting factor. Reactions to the use of the factor can include, allergic manifestations, transfusion viral diseases and alloantibodies. Objective: analyze the epidemiological profile of patients with Hemophilia A, and the diseases associated with the use of factor VIII and recombinant factor VIII. Methodology: descriptive and retrospective cross-sectional study. Data collection was carried out in the medical records of patients diagnosed with hemophilia A, filled with more than 70% of the information, at the Fundação Hospitalar de Hematologia e Hemoterapia do Amazonas, Brazil. Results: in the Ministry of Health of Brazil, it was identified, for the State from Amazonas, the registry of 276 individuals diagnosed with Hemophilia A. Included in the analysis for this study, 164 medical records. Sociodemographic characteristics: male, 99.4%, adolescents (28%) and young people (26.8%), brown skin color, 67.1%, incomplete elementary school, 28.6%, and working as a student, 42.7%. Clinical condition: 36.6% classified with severe hemophilia A. All patients had a clinical and laboratory diagnosis. The most common kinship is that of brothers, 35.3%. Predominant symptoms: hemarthrosis 45.4%, pain 31.9%, edema 24% and arthropathy 8.5%. Recombinant factor VIII, administered in 34.8% of patients, while plasma factor VIII in 28.0%. Doses of 2000 IU to 2999 IU were administered. Complications: arthralgia 77.4% and hemorrhage 77.4%. Conclusion: qualified care by health professionals helps to prevent serious complications, resulting in quality of life for the hemophiliac.

Avaliação da articulação temporomandibular em indivíduos com hemofilia grave: caracterização clínica e aplicação da relaxometria T2 em imagens de ressonância magnética / Evaluation of the temporomandibular joint in individuals with severe hemophilia: clinical characterization and application of T2 relaxometry in magnetic resonance images

A hemofilia é uma doença hemorrágica hereditária ligada ao cromossomo X, decorrente da ausência ou da baixa quantidade no plasma dos fatores de coagulação VIII (hemofilia A) ou IX (hemofilia B). Clinicamente, a hemofilia se caracteriza por episódios recorrentes de sangramentos, que podem ocorrer espontaneamente ou em decorrência de traumas. O sistema músculo-esquelético é freqüentemente afetado pelos eventos hemorrágicos nos indivíduos portadores de hemofilia. Dentro desse sistema, as articulações são alvos de episódios recorrentes de sangramentos, resultando no fenômeno a que chamamos de hemartrose, responsável por estimular a hiperplasia da membrana sinovial articular e caracterizando o quadro das sinovites hemofílicas. O ciclo vicioso típico de hemartrose-sinovite-hemartrose quando estabelecido, tem como consequência a cronificação das alterações agudas articulares. Histologicamente, na sinovite, é observada a proliferação de fibroblastos sinoviais e a presença de um infiltrado de células inflamatórias. Apesar de a articulação temporomandibular (ATM) ser uma articulação do tipo sinovial, são poucos os relatos sobre o seu envolvimento na hemartrose e na sinovite hemofílica. Em razão disso, o objetivo deste estudo foi o de avaliar a ATM por meio de imagens de ressonância magnética (RM) utilizando-se a técnica denominada relaxometria. A relaxometria, também chamada de mapeamento T2, consiste em calcular as variações na intensidade do sinal de radiofrequência (RF) emitido pelos prótons das moléculas de água em função da variação do tempo quando excitados por um pulso externo de RF e submetidos à influência de um campo magnético estático. Esta mensuração é possível de ser realizada por meio da chamada sequência multieco de spin. A literatura científica descreve que a concentração de ferro nos tecidos resulta em encurtamento do tempo T2 e em consequente diminuição da intensidade de sinal das amostras biológicas. Desta forma, a relaxometria realizada com o objetivo de quantificar a concentração de ferro pode servir como uma forma de se estudar as propriedades intrínsecas dos tecidos e o ambiente molecular das estruturas analisadas. Foram selecionados pacientes maiores de 18 anos de idade, comprovadamente portadores de hemofilia A ou B classificadas como grave e que tiveram seguimento regular ambulatorial. O grupo controle foi pareado por idade e sexo com os pacientes, sendo representado por voluntários sem histórico de discrasia sanguínea ou disfunção temporomandibular (DTM). Foram avaliados 39 indivíduos (18 pacientes e 21 controles). Os critérios de exclusão foram: pacientes portadores de próteses metálicas, marca-passo cardíaco, clipes metálicos intracranianos (para aneurisma), claustrofobia severa e não consentimento à participação no estudo. Este estudo observou que a morfologia dos discos articulares se altera com o passar do tempo em indivíduos portadores de hemofilia grave (p-valor = 0,006), entretanto, nenhuma diferença estatística foi observada no que diz respeito à intensidade de sinal calculada em 4 momentos distintos (p-valor > 0,05), sugerindo não haver uma concentração maior de ferro no interior do disco articular na amostra estudada, nem entre os grupos e nem na transição entre os momentos avaliados. Também não foi observada diferença estatística (p-valor > 0,05) no que diz respeito aos sintomas relatados (sintomatologia dolorosa, cefaleia e alteração no movimento de abertura bucal e lateralidade da mandíbula) entre indivíduos portadores de hemofilia e indivíduos do grupo controle, sugerindo que a ATM possui uma forte capacidade de adaptação em comparação com as demais articulações do tipo sinovial diante dos efeitos provocados pela degradação da cartilagem articular.

Yttrium-90 radiosynovectomy in knees and ankles (25 joints in 22 hemophilic patients). Short-term results

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.

Avaliação da Força Muscular em Adultos com Hemofilia de uma Associação de Pacientes em Brasília / Assessment of Muscle Strength in Adults with Hemophilia from an Association of Patients in Brasília / Evaluación de la fuerza muscular en adultos con hemofilia de una asociación de pacientes en Brasilia

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

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.

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.

The intra-articular use of an hemostatic and non-adherent bicomponent for pain control, hemarthrosis, and knee range of motion after anterior cruciate ligament reconstruction

Introduction: Complications in the recent postoperative period of anterior cruciate ligament reconstruction are common. Among them, pain, hemarthrosis, and difficulty of complete range of motion. The purpose of this study is to evaluate the use of the intra-articular carboxymethylcellulose ­ polysaccharide B bicomponent shortly after anterior cruciate ligament reconstruction, and to compare the results obtained for pain control, hemarthrosis, and knee range of motion with a control group. Materials and methods: randomized, and prospective clinical trial of thirty-two patients divided into two groups: reconstruction of the anterior cruciate ligament with an intra-articular injection of a bicomponent carboxymethylcellulose-polysaccharide B (n = 16) and without the bicomponent (n = 16). Pain, hemarthrosis and knee range of motion were evaluated in the first postoperative week. Results: the group with bicomponent presented less pain on the third (p = 0.017) and fifth (p = 0.029) postoperative day when compared to the control group. Hemarthrosis was significantly lower on the first postoperative day (p = 0.001), and there was a significant improvement in the range of motion on the seventh day of surgery (p = 0.008) in this same group. Conclusions: the use of intra-articular carboxymethylcellulose-polysaccharide B showed superior results for pain control, hemarthrosis, and gain in the knee range of motion in the recent postoperative period (up to seven days) after anterior cruciate ligament reconstruction, when compared to patients from the control group

Clinical study on factor Ⅷ inhibitor in children with hemophilia A / 中华血液学杂志

Objective: To reveal the related factors of inhibitors and differences ofhemorrhage and joint disease before and after the production of inhibitors in children with hemophilia A (HA) . Methods: Retrospective analyses of the clinical data of 381 children with HA under the age of 16 registered in the Registration Management Center of Hemophilia in Henan Provincial from January 2015 to August 2018. Results: A total of the 381 children were enrolled with 116 (30.4%) mild, 196 (51.4%) moderate, and 69 (18.1%) severe cases; 54 patients (14.2%) had inhibitors, including 22 high and 32 low titer inhibitors. Positive family history was positively associated with inhibitors[P<0.001, OR=3.299 (95%CI 1.743-5.983) ], and high-intensity exposure was associated with inhibitors[P=0.002, OR=2.587 (95%CI 1.414-4.731) ]. High-intensity exposure was associated with high titer inhibitor production[P=0.001, OR=8.689 (95%CI 2.464-30.638) ], and high-intensity exposure increased the risk of high titer inhibitors in HA patients. After inhibitors occurred in 54 patients with HA, the rates of overall joint annual bleeding (z=-3.440, P=0.001) and traumatic annual bleeding (z=-2.232, P=0.026) increased, but the rates of the annual joint bleeding (z=-1.342, P=0.180) and spontaneous annual bleeding (z=-1.414, P=0.157) remained to be not statistically significant. The joint ultrasound score did not change significantly after the inhibitor information (z=-0.632, P=0.527) . Conclusions: Positive family history and high-intensity exposure could increase the risk of F Ⅷ inhibitors in HA patients, and high-intensity exposure increased the risk of high titer inhibitors. The rates of the overall joint annual bleeding and traumatic annual bleeding increased after the inhibitor information.

Sinoviortesis con radioisótopos en hemofilia: Experiencia de un centro en Chile / Radionuclide synoviorthesis in hemophilia: experience in 107 patients

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.

Hemarthrosis Occurred after Arthroscopic Rotator Cuff Repair in a Chronic Renal Failure Patient with a Stenosis in an Ipsilateral Arteriovenous Fistula / 대한정형외과학회잡지

Hemarthrosis occurring after arthroscopic surgery for lesions of the shoulder joint is a very rare complication that can develop due to an injury to the blood vessels when an anterior portal is formed. This is a complication that rarely develops in patients who are taking antithrombotic drugs or who do not have associated diseases, such as thrombocytopenia. We report a case of hemarthrosis that occurred after performing arthroscopic surgery to repair a rotator cuff tear in a patient with a stenosis in an arteriovenous fistula for hemodialysis in the ipsilateral upper arm.

Frequência de alterações posturais em hemofílicos / Frequency of postural alterations in haemophiliacs

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)

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)

Treatment of Recurrent Hemarthrosis after Total Knee Arthroplasty

PURPOSE: The purpose of this study is to evaluate the incidence and treatment of recurrent hemarthrosis after total knee replacement (TKR). MATERIALS AND METHODS: Among a total of 5,510 patients who underwent TKR from March 2000 to October 2016, patients who had two or more bleeding 2 weeks after surgery were studied. Conservative treatments were performed for all cases with symptoms. In patients who did not respond to conservative treatment several times, embolization was performed. We retrospectively evaluated the postoperative bleeding time, bleeding frequency, treatment method, and outcome. RESULTS: Seventeen (0.3%) of the 5,510 patients developed recurrent hemarthrosis. Bleeding occurred at an average of 2 years 3 months after the operation. Joint aspiration was performed 3.5 times (range, 2 to 10 times) on average, and 14 cases (82.3%) were treated with conservative treatment. In 3 patients with severe bleeding and hemorrhage, embolization was performed. CONCLUSIONS: Recurrent hemarthrosis after TKR is a rare disease with a low incidence of 0.3% and usually could be treated by conservative treatment. If recurrences occur repeatedly, embolization through angiography or surgical treatment may be considered, but the results are not satisfactory and careful selection of treatment modalities is warranted.

Clinical application and optimization of HEAD-US quantitative ultrasound assessment scale for hemophilic arthropathy / 中华血液学杂志

Objective: To assess the feasibility of HEAD-US scale in the clinical application of hemophilic arthropathy (HA) and propose an optimized ultrasound scoring system. Methods: From July 2015 to August 2017, 1 035 joints ultrasonographic examinations were performed in 91 patients. Melchiorre, HEAD-US (Hemophilic Early Arthropathy Detection with UltraSound) and HEAD-US-C (HEAD-US in China) scale scores were used respectively to analyze the results. The correlations between three ultrasound scales and Hemophilia Joint Health Scores (HJHS) were evaluated. The sensitivity differences of the above Ultrasonic scoring systems in evaluation of HA were compared. Results: All the 91 patients were male, with median age of 16 (4-55) years old, including 86 cases of hemophilia A and 5 cases hemophilia B. The median (P25, P75) of Melchiorre, HEAD-US and HEAD-US-C scores of 1 035 joints were 2(0,6), 1(0,5) and 2(0,6), respectively, and the correlation coefficients compared with HJHS was 0.747, 0.762 and 0.765 respectively, with statistical significance (P<0.001). The positive rates of Melchiorre, HEAD-US-C and HEAD-US scale score were 63.0% (95%CI 59.7%-65.9%), 59.5% (95%CI 56.5%-62.4%) and 56.6% (95%CI 53.6%-59.6%) respectively, and the difference was statistically significant (P<0.001). Even for 336 cases of asymptomatic joints, the positive rates of Melchiorre, HEAD-US-C and HEAD-US scale score were 25.0% (95%CI 20.6%-29.6%), 17.0% (95%CI 12.6%-21.1%) and 11.9% (95%CI 8.4%-15.7%) respectively, and the difference was statistically significant (P<0.001). There were significant changes (P<0.05) in the ultrasonographic score of HA before and after onset of hemorrhage in 107 joints of 40 patients. The difference in variation amplitude of HEAD-US-C scores and HEAD-US scores before and after joint bleeding was statistically significant (P<0.001). Conclusion: Compared with Melchiorre, there were similar good correlations between HEAD-US, HEAD-US-C and HJHS. HEAD-US ultrasound scoring system is quick, convenient and simple to use. The optimized HEAD-US-C scale score is more sensitive than HEAD-US, especially for patients with HA who have subclinical state, which make up for insufficiency of sensitivity in HEAD-US scoring system.

Clinical and Radiological Evaluation After Chemical Synovectomy With Rifampicin in Hemophilic Arthropathy: Korean Experience With a 2-Week Interval Protocol

OBJECTIVE: To assess the clinical outcome of chemical synovectomy with rifampicin in hemophilic arthropathy by using the World Federation of Hemophilia (WFH) scoring system and plain radiograph. METHODS: We performed rifampicin synovectomy (RS) on 30 joints of 28 hemophilic patients diagnosed as hemophilic arthropathy stage I–III (based on Fernandez-Palazzi clinical classification). Clinical status (bleeding frequency, pain, joint physical status) and radiological staging were evaluated as parts of the WFH scoring system before and 1 year after RS. The patients were divided into two groups by the Arnold-Hilgartner scale of the initial X-ray as stage 3 or less for the low-stage group (n=17) and over 3 for the high-stage group (n=13). RESULTS: Total WFH joint physical scores were reduced after injection, and the number of bleeding episodes and pain showed especially significant improvement. For other subscores of the WFH joint physical score, only swelling, range of motion, and crepitus showed statistically significant improvement. According to the severity of the radiologic finding, the WFH joint physical score of both the low-stage and high-stage groups showed significant improvement. In the radiological aspect, the low-stage group, without joint space narrowing at the initial plain radiograph, showed no further aggravation after injection. However, in the high-stage group, radiology found aggravation regardless of the procedure. CONCLUSION: It is suggested that chemical synovectomy with rifampicin may prevent hemarthrosis and improve clinical symptoms. Especially in the early stage of arthropathy without joint-space narrowing, it seems to have an additional benefit that delays radiological aggravation and preserves joint status.

Hemarthrosis subtalar, a rare diagnosis / Hemartrose subtalar, um diagnóstico raro

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.

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.

Sinovitis vellonodular pigmentada localizada, causa de hemartrosis en rodilla / Localized pigmented vellonodular sunovitis, cause of knee hemarthrosis

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.

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.

Perfil clínico y epidemiológico de pacientes con hemofilia registrados en la Liga de Hemofílicos de Antioquia (Colombia) / Clinical and epidemiological profile of patients with hemophilia in Liga de Hemofilicos de Antioquia (Colombia)

Introducción: La hemofilia es una enfermedad hemorrágica hereditaria caracterizada por la ausencia o deficiencia de factores VIII y IX de la coagulación. Objetivo. Determinar el perfil clínico y epidemiológico de los pacientes con hemofilia registrados en la liga de hemofílicos de Antioquia (LIHEA) durante 2005-2011. Metodología. Estudio descriptivo transversal, la población fueron pacientes con hemofilia de la LIHEA que cumplieron con criterios de selección. Se diseñó un instrumento de recolección de información y se aplicó el instrumento Modelo de actitud en pacientes con hemofilia (MAPACHE). Las variables cuantitativas se analizaron con medidas de tendencia central y dispersión, a las variables cualitativas se les estimó frecuencias absolutas, proporciones y razones. Resultados. Se incluyeron 36 hombres con hemofilia, con una mediana de 22 años de edad. En cuanto a los antecedentes personales los más frecuentes fueron HTA (8.3%) y tromboflebitis (5.6%). El sedentarismo fue el factor de riesgo de estilo de vida más común (8.3%). Las manifestaciones clínicas más frecuentes fueron hemartrosis (94.4%), hematomas musculares (80.6%) y equimosis (77.8%). En la mitad de los pacientes la enfermedad generó algún tipo de discapacidad, siendo la más frecuente la física (44.4%). El tratamiento farmacológico más frecuente fue profilaxis con factor VIII con un 88.9%, acompañado de la natación como principal tratamiento no farmacológico con un 77.8%. En cuanto al MAPACHE el 30.6% manifestó una actitud negativa hacia la enfermedad, un 5.6% fue neutra y para el 63.9% restante fue positiva. Conclusión. La hemofilia tuvo grandes repercusiones físicas y emocionales para los pacientes estudiados que la padecen

Introduction: Hemophilia is a hereditary hemorrhagic disorder characterized by the absence or deficiency of factors VIII and IX of the coagulation. Objective. To determine the clinical and epidemiological profile of patients with hemophilia recorded in the Liga de Hemofilicos de Antioquia (LIHEA) during 2005-2011. Methodology. Descriptive transversal study, population were patients with hemophilia from LIHEA which met selection criteria. We designed a data collection instrument and applied the attitude instrument in patients with hemophilia (MAPACHE). The quantitative variables were analyzed using measures of central tendency and dispersion, the qualitative variables were estimated absolute frequencies, proportions and reasons. This research had the respective ethical guarantee. Results. We included 36 men with hemophilia, with a median of 22 years of age. In terms of the personal history, the most frequent were hypertension (8.3%) and thrombophlebitis (5.6%). Sedentariness was the most common lifestyle risk factor (8.3%). The most frequent clinical manifestations were hemarthrosis (94.4%), muscle hematomas (80.6%) and ecchymosis (77.8%). In half of the patients, disease generated some kind of disability, being the most common the physics (44.4%). The most common drug treatment was prophylactic factor VIII with 88.9 %, accompanied by swimming as the main non-pharmacological treatment with 77.8 %. Respect MAPACHE, the 30.6% expressed a negative attitude towards the disease, a 5.6% was neutral and the 63.9% was positive. Conclusion. Hemophilia is still a disease of great physical and emotional implications for studied patients who suffer it

Spontaneous elbow hemarthrosis identified by point-of-care ultrasound

Traumatic or spontaneous hemarthroses are an important cause of joint effusions, and can complicate innate or acquired coagulopathies. The elbow is an unusual location for a spontaneous hemarthrosis; we describe a previously unreported case of warfarin-induced spontaneous elbow hemarthrosis, diagnosed by point-of-care ultrasound. On the basis of clinical and ultrasound findings arthrocentesis was deferred, and the patient was successfully treated with warfarin reversal and conservative care. Physical examination is unreliable for the detection of a joint effusion, and misdiagnosis and can lead to unnecessary investigation or resource use. Point-of-care ultrasound allows accurate, prompt, direct visualization of a joint effusion, and non-invasive confirmation of a hemarthrosis. Ultrasound can facilitate accurate diagnosis and characterization of joint effusions to improve the care of patients with coagulopathy.

Causes of Aseptic Persistent Pain after Total Knee Arthroplasty

BACKGROUND: Persistent pain after total knee arthroplasty (TKA) is dissatisfying to the patient and frustrating to the surgeon. The purpose of this study is to evaluate the aseptic causes and clinical course of intractable pain following TKA. METHODS: Of the total 2,534 cases of primary TKA reviewed, 178 cases were classified as having aseptic persistent pain that was not resolved within 1 year after surgery. Except for the cases with periprosthetic fracture (56 knees), 122 cases of aseptic painful TKA were divided into two groups: intra-articular group (83 knees) and extra-articular group (39 knees). RESULTS: In the intra-articular group, the main reasons for pain were aseptic loosening (n = 40), polyethylene wear (n = 16), instability (n = 10), recurrent hemarthrosis (n = 5), patellar maltracking (n = 4), tendon ruptures (n = 4), and stiffness (n = 2). In the extraarticular group, 10 knees (25.6%) were found to have nerve entrapment in the spine, 6 knees (15.4%) were found to have hip osteoarthritis or femoral head avascular necrosis. The reasons for persistent knee pain in the remaining 23 knees (59.0%) still remain elusive. CONCLUSIONS: Persistent pain after TKA originated from pathology of extra-articular origin in a considerable number of cases in this study. Therefore, it is important to perform thorough preoperative evaluations to reduce pain resulting from extra-articular causes. Furthermore, meticulous surgical procedures and optimal alignment are required to reduce pain of intra-articular origin related to implant wear, instability, and patellar maltracking.