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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.

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OBJECTIVES: To evaluate the applicability and compliance with guidelines for early initiation of long-term prophylaxis in infants with severe hemophilia A and to identify factors associated with guideline compliance. STUDY DESIGN: This real-world, prospective, multicenter, population-based FranceCoag study included almost all French boys with severe hemophilia A, born between 2000 and 2009 (ie, after guideline implementation). RESULTS: We included 333 boys in the study cohort. The cumulative incidence of long-term prophylaxis use was 61.2% at 3 years of age vs 9.5% in a historical cohort of 39 boys born in 1996 (ie, before guideline implementation). The guidelines were not applicable in 23.1% of patients due to an early intracranial bleeding or inhibitor development. Long-term prophylaxis was delayed in 10.8% of patients. In the multivariate analysis, 2 variables were significantly associated with "timely long-term prophylaxis" as compared with "delayed long-term prophylaxis": hemophilia treating center location in the southern regions of France (OR 23.6, 95% CI 1.9-286.7, P = .013 vs Paris area) and older age at long-term prophylaxis indication (OR 7.2 for each additional year, 95% CI 1.2-43.2, P = .031). Long-term prophylaxis anticipation was observed in 39.0% of patients. Earlier birth year (OR 0.5, 95% CI 0.3-0.8, P = .010 for birth years 2005-2009 vs 2000-2004) and age at first factor replacement (OR 1.9 for each additional year, 95% CI 1.2-3.0, P = .005) were significantly associated with "long-term prophylaxis guideline compliance" vs "long-term prophylaxis anticipation." CONCLUSIONS: This study suggests that long-term prophylaxis guidelines are associated with increased long-term prophylaxis use. However, early initiation of long-term prophylaxis remains a challenge.

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No presente trabalho é descrito o uso do plasma rico em plaquetas (PRP) no tratamento de um caso de osteoartrose naturalmente adquirida em um cão. O PRP foi obtido a partir de sangue venoso autólogo coletado em citrato de sódio. A separação do precipitado celular do sobrenadante foi realizada de forma estéril. Após adição de cloreto de cálcio e liberação dos fatores de crescimento, o PRP foi infiltrado no joelho do animal em estudo. Após o tratamento, os parâmetros de apoio, crepitação e dor articular apresentaram melhora, não havendo intolerância do animal à infiltração. Preliminarmente, pode-se dizer que é esta é uma técnica minimamente invasiva e de alta aplicabilidade. O emprego do PRP para infiltração mostrou ser simples, rápido, barato e seguro para o tratamento da osteoartrose no animal em questão. Estudos com maior número de casos em cães são necessários, a fim de validar este procedimento como prática terapêutica na rotina clínica.(AU)

The use of platelet-rich plasma (PRP) in the treatment of a natural case of osteoarthrosis in a dog is reported in the present work. The PRP was obtained through autologous venous blood collected in sodium citrate. The cellular precipitate was separated from the supernatant in a sterile condition. After the addition of calcium chloride and the release of growth factors, the PRP was infiltrated in the knee of the dog under study. After the treatment, weight-bearing, crackle and joint pain parameters presented improvement without intolerance to the infiltration by the animal. Preliminarily, it can be concluded that this is a minimally invasive method with high applicability, as well as being simple, quick, cheap and safe for the treatment of degenerative joint disease to the described animal. The study of a larger number of cases in dogs is necessary in order to validate the procedure for clinical routine.(AU)

En este estudio se ha descrito el uso del plasma rico en plaquetas (PRP) en el tratamiento de un caso de osteoartritis, adquirido naturalmente por un perro. El PRP ha sido obtenido a partir de la sangre venosa autóloga recogido en citrato de sodio. La separación del precipitado celular del sobrenadante se ha realizado de forma estéril. Después de la adición de cloruro de calcio y liberación de los factores de crecimiento, el PRP ha sido infiltrado en la rodilla del animal en estudio. Tras el tratamiento, los parámetros de apoyo, crepitación y dolor articular presentaron mejora, no ocurriendo intolerancia del animal a la infiltración. Preliminarmente, se puede decir que esta es una técnica poco invasiva y de alta aplicabilidad. El empleo del PRP para infiltración ha mostrado ser sencillo, rápido, barato y seguro para el tratamiento de osteoartritis en el animal estudiado. Estudios con mayor número de casos en perros son necesarios, para que se pueda validar este procedimiento como práctica terapéutica en la rutina clínica.(AU)

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No presente trabalho é descrito o uso do plasma rico em plaquetas (PRP) no tratamento de um caso de osteoartrose naturalmente adquirida em um cão. O PRP foi obtido a partir de sangue venoso autólogo coletado em citrato de sódio. A separação do precipitado celular do sobrenadante foi realizada de forma estéril. Após adição de cloreto de cálcio e liberação dos fatores de crescimento, o PRP foi infiltrado no joelho do animal em estudo. Após o tratamento, os parâmetros de apoio, crepitação e dor articular apresentaram melhora, não havendo intolerância do animal à infiltração. Preliminarmente, pode-se dizer que é esta é uma técnica minimamente invasiva e de alta aplicabilidade. O emprego do PRP para infiltração mostrou ser simples, rápido, barato e seguro para o tratamento da osteoartrose no animal em questão. Estudos com maior número de casos em cães são necessários, a fim de validar este procedimento como prática terapêutica na rotina clínica...

The use of platelet-rich plasma (PRP) in the treatment of a natural case of osteoarthrosis in a dog is reported in the present work. The PRP was obtained through autologous venous blood collected in sodium citrate. The cellular precipitate was separated from the supernatant in a sterile condition. After the addition of calcium chloride and the release of growth factors, the PRP was infiltrated in the knee of the dog under study. After the treatment, weight-bearing, crackle and joint pain parameters presented improvement without intolerance to the infiltration by the animal. Preliminarily, it can be concluded that this is a minimally invasive method with high applicability, as well as being simple, quick, cheap and safe for the treatment of degenerative joint disease to the described animal. The study of a larger number of cases in dogs is necessary in order to validate the procedure for clinical routine...

En este estudio se ha descrito el uso del plasma rico en plaquetas (PRP) en el tratamiento de un caso de osteoartritis, adquirido naturalmente por un perro. El PRP ha sido obtenido a partir de la sangre venosa autóloga recogido en citrato de sodio. La separación del precipitado celular del sobrenadante se ha realizado de forma estéril. Después de la adición de cloruro de calcio y liberación de los factores de crecimiento, el PRP ha sido infiltrado en la rodilla del animal en estudio. Tras el tratamiento, los parámetros de apoyo, crepitación y dolor articular presentaron mejora, no ocurriendo intolerancia del animal a la infiltración. Preliminarmente, se puede decir que esta es una técnica poco invasiva y de alta aplicabilidad. El empleo del PRP para infiltración ha mostrado ser sencillo, rápido, barato y seguro para el tratamiento de osteoartritis en el animal estudiado. Estudios con mayor número de casos en perros son necesarios, para que se pueda validar este procedimiento como práctica terapéutica en la rutina clínica...

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Studies have determined the effects of joint immobilization on the articular cartilage of sedentary animals, but we are not aware of any studies reporting the effects of joint immobilization in previously trained animals. The objective of the present study was to determine whether exercise could prevent degeneration of the articular cartilage that accompanies joint immobilization. We used light microscopy to study the thickness, cell density, nuclear size, and collagen density of articular cartilage of the femoral condyle of Wistar rats subjected to aerobic physical activity on an adapted treadmill five times per week. Four groups of Wistar rats were used: a control group (C), an immobilized group (I), an exercised group (E), and an exercised and then immobilized group (EI). The right knee joints from rats in groups I and EI were immobilized at 90 °C of flexion using a plastic cast for 8 weeks. Cartilage thickness decreased significantly in group I (mean, 120.14 ± 15.6 µm, P < 0.05), but not in group EI (mean, 174 ± 2.25), and increased significantly in group E (mean, 289.49 ± 9.15) compared with group C (mean, 239.20 ± 6.25). The same results were obtained for cell density, nuclear size, and collagen density (in all cases, P < 0.05). We concluded that exercise can prevent degenerative changes in femoral articular cartilage caused by immobilization of the knee joint.

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OBJECTIVES: To evaluate the impact of the 2002 guidelines on the current status of prophylaxis in French children with severe hemophilia A or B. STUDY DESIGN: Clinical information was captured, in a prospective way, using FranceCoag Network. We retrospectively studied 291 patients with severe (<1 IU/dL) hemophilia A and B, with no history of inhibitors. RESULTS: Our results demonstrate that the availability of national medical guidelines has improved clinical practice in France. In the past decade, the proportion of children with severe hemophilia undergoing prophylaxis has shown a significant 2- to 3-fold increase: ∼80% of these children>3 years of age are now receiving prophylaxis. In severe hemophilia A and B, the age at which prophylaxis commences has significantly decreased: 4.0 and 6.1 years for the period 1996-1999 as opposed to 1.8 and 1.4 years for the period 2004-2007 (P=.0001). CONCLUSIONS: Long-term clinical and physical evaluations of patients will be necessary to establish the benefits of this increase in prophylactic treatment on the prevention of hemophilic arthropathy.

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There is a lack of publications concerning the use of primary prophylaxis in developing countries. The aim of this study was to evaluate the effectiveness of primary prophylaxis therapy in preventing the development of arthropathy in children with severe haemophilia A or B. From January 1999 to April 2009, a prospective study was carried out involving 39 patients with severe haemophilia A or B. These haemophilia A and haemophilia B patients received 20-40 UI kg(-1) of factors VIII and IX, three and two times per week, respectively. The patients were followed up by a multidisciplinary team. The analysis was carried out in 23 patients who had been on prophylaxis therapy for at least 12 months. The orthopaedic evaluation was performed according to the recommendations of the Orthopedic Advisory Committee of the World Federation of Hemophilia, by evaluating pain and bleeding, and by conducting physical examination and radiological assessment (Pettersson's Joint Score and magnetic resonance): 82.6% of patients who had used the factor regularly did not present any clinical or radiographic changes in the studied joints; 17.4% used the factor irregularly at the beginning of the treatment and of those, most patients presented mild changes in the joints; and 4.3% presented transient knee and ankle pain in spite of regular factor use. The preliminary results of primary prophylaxis confirm its effectiveness in preventing haemophilic arthropathy. Socioeconomic factors did not play a significant role.

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BACKGROUND: Limited joint mobility (LJM) in childhood insulin-dependent (type 1) diabetes is associated with a substantially increased risk of microvascular complications. Cross-sectional studies have not demonstrated a relationship between LJM and metabolic control. This study was designed to determine whether glycemic control, as measured by glycohemoglobin (hgbA1C) levels from the onset of diabetes, is associated with the occurrence of LJM. METHOD: Probands (n = 18) had hgbA1C values and recorded observation of joint function from soon after onset of their diabetes. Controls (n = 40) were matched to probands for gender and age at diagnosis and had follow-up beyond the age at which the proband was found to have LJM. RESULTS: The odds ratio for occurrence of LJM for the mean hgbA1C from diabetes onset was 1.46, 95% confidence limits 1.07 to 2.00. Thus, for every unit increase in average hgbA1C, there was approximately a 46% increase in the risk of LJM. When hgbA1C was dichotomized, the OR for hgbA1C of more than 8% was 2.55, and the OR was 4.54 if the hgbA1C was greater than 12%. Age at diagnosis and duration of diabetes were not independent prognostic factors for LJM. CONCLUSION: Glycemic control from onset of diabetes is strongly associated with occurrence of LJM.

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Musculoskeletal care is provided in many different settings by many different providers. Physicians are better prepared to treat acute diseases, acute manifestations and have problems in following chronic patients and to preventing losses of functional independence. Haematoma affecting muscles and joints is the most common musculoskeletal finding in haemophilic patients. Proper treatment is fundamental to prevent disability. Treatment aims at restoring the original muscle trophism and strength because joint stability is dependent on the uniform distribution of muscle power over different joint surfaces. Physical rehabilitation continues until complete anatomical and functional recovery is achieved. Coordination and proprioceptive stimulation complement the exercises to recover original muscle strength. Recurrent haemorrhages usually progress to chronic synovitis, the leading cause of motor disability and joint degeneration in haemophilic patients. Moreover, chronic synovitis is a predisposing factor for repetitive episodes of haemarthrosis, a leading cause of joint deformities and severe muscle atrophy. At this point, an efficient treatment alternative should be put into effect to restrain bleeding and prevent synovitis-related joint degeneration, which is synovectomy. Synovectomy is an effective alternative, less costly and easier to perform. Rifampicin synovectomy is advantageous because it is safe, not aggressive to the patient, can be repeated if necessary, and it does not prevent the use of either radiotherapy or surgery after, if necessary. Once the sequelae have set in, treatment tends to be conservative, and it aims at giving the patient functional independence for gait and other activities. The most common limb deformities seen in these patients are knee and elbow flexion, but the patient should also be assessed for compensatory scoliosis, hyperlordosis and lower limb length discrepancies. Chronic joint pain is a rather common complaint in this patient population with early arthrosis, and special drugs and physical treatment must be put into action. Rehabilitation is often inexpensive and very efficient.

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