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3.
Autops Case Rep ; 5(2): 55-9, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26484336

RESUMO

Acquired hemophilia A (AHA) is a rare disorder that results from the presence of autoantibodies against the clotting factor VIII (FVIII) causing hemorrhagic disorders. This entity is mostly associated with autoimmune diseases, pregnancy, the postpartum period, drugs and malignancy. Among the solid cancers, prostate neoplasm is the most common cause of AHA. The management of AHA involves the control of active bleeding and the use of specific therapies to eliminate the inhibitor. The authors describe the case of an 87-year-old man with prostate cancer who developed a bleeding disorder 5 years after the cancer diagnosis. Treatment with prednisone did not reach a satisfactory clinical response, which was only achieved with the association of azathioprine. The patient became asymptomatic with no further bleeding episodes, but developed a fatal sepsis after 3 months of treatment with these immunosuppressive agents.

4.
Acta Ortop Bras ; 23(3): 162-6, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26207096

RESUMO

OBJECTIVE: To assess whether joint lavage, viscosupplementation and triamcinolone improve joint pain, function and quality of life in patients with severe hemophilic arthropathy. METHODS: Fourteen patients with knee and/or ankle hemophilic arthritis with and without involvement of other joints underwent joint lavage and subsequent injection of hylan G-F20 and triamcinolone in all affected joints. The patients answered algo-functional questionnaires (Lequesne and WOMAC), visual analog scale for pain (VAS) and SF-36 preoperatively, and at one, three, six and twelve months postoperatively. RESULTS: Sixteen knees, 15 ankles, 8 elbows and one shoulder were treated in 14 patients. Six patients had musculoskeletal bleeding [ankle (1), leg muscle (2) and knees (4)] at 3 months affecting the results. Pain did not improve significantly. Function improved (WOMAC p=0.02 and Lequesne p=0.01). The physical component of SF-36 improved at all time points except at 3 months, with best results at one-year follow-up (baseline = 33.4; 1 month = 39.6; 3 months= 37.6; 6 months 39.6 and 1 year = 44.6; p < 0.001). CONCLUSION: Joint lavage followed by injection of triamcinolone and hylan G-F20 improves function and quality of life progressively up to a year, even in severe hemophilic arthropathy. Level of Evidence IV, Case Series.

5.
Acta ortop. bras ; 23(3): 162-166, May-Jun/2015. tab, graf
Artigo em Inglês | LILACS | ID: lil-748142

RESUMO

Objetivo: Avaliar se lavagem articular, viscossupplementação e triancinolona melhoram a dor, função e qualidade de vida de pacientes com artropatia hemofílica grave. Métodos: Quatorze pacientes com poliartrite hemofílica do joelho e/ou tornozelo com ou sem envolvimento de outras articulações foram submetidos a lavagem articular e subsequente injeção de hilano G-F20 e triancinolona em todas as articulações afetadas. Os pacientes responderam aos questionários algo funcionais (Lequesne e WOMAC), escala visual analógica parador (EVA) e SF-36 no pré-operatório, um, três, seis e 12 meses de pós-operatório. Resultados: Dezesseis joelhos, 15 tornozelos,oito cotovelos e um ombro foram tratados em 14 pacientes. Seis pacientes tiveram sangramento musculoesquelético [tornozelo (1),músculo da perna (2) e joelhos (4)] próximo da avalição de três meses afetando os resultados. A dor não melhorou significativamente.A função melhorou (WOMAC p = 0,02, Lequesne p = 0,01).O componente físico do SF-36 melhorou em todos os momentos,exceto em três meses, com melhores resultados após um ano(pré-operatório = 33.4; um mês = 39,6; três meses = 37,6; seismeses = 39,6; um ano = 44,6; p <0,001). Conclusão: A lavagem articular seguida da injeção de triancinolona e hilano G-F20 melhora a função e a qualidade de vida na artropatia hemofílica grave. Nível de Evidência IV, Série de Casos.


Objective: To assess whether joint lavage, viscosupplementation and triamcinolone improve joint pain, function and quality of life in patients with severe hemophilic arthropathy. METHODS: Fourteen patients with knee and/or ankle hemophilic arthritis with and without involvement of other joints underwent joint lavage and subsequent injection of hylan G-F20 and triamcinolone in all affected joints. The patients answered algo-functional questionnaires (Lequesne and WOMAC), visual analog scale for pain (VAS) and SF-36 preoperatively, and at one, three, six and twelve months postoperatively. RESULTS: Sixteen knees, 15 ankles, 8 elbows and one shoulder were treated in 14 patients. Six patients had musculoskeletal bleeding [ankle (1), leg muscle (2) and knees (4)] at 3 months affecting the results. Pain did not improve significantly. Function improved (WOMAC p=0.02 and Lequesne p=0.01). The physical component of SF-36 improved at all time points except at 3 months, with best results at one-year follow-up (baseline = 33.4; 1 month = 39.6; 3 months= 37.6; 6 months 39.6 and 1 year = 44.6; p < 0.001). CONCLUSION: Joint lavage followed by injection of triamcinolone and hylan G-F20 improves function and quality of life progressively up to a year, even in severe hemophilic arthropathy. Level of Evidence IV, Case Series.


Assuntos
Humanos , Tornozelo , Ácido Hialurônico/administração & dosagem , Ácido Hialurônico/uso terapêutico , Cotovelo , Hemofilia A , Artropatias , Joelho , Osteoartrite , Ombro , Viscossuplementação
6.
Knee Surg Sports Traumatol Arthrosc ; 23(3): 785-91, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25839071

RESUMO

PURPOSE: This study assessed the results of two-portal knee arthroscopic synovectomy in terms of bleeding recurrence, knee function, quality of life (QOL), and radiographic staging in a prospective case series of patients with haemophilia. METHODS: Nine knees from eight patients (median age 16.1 years; range 9.6-25 years) with haemophilia and recurrent knee haemarthrosis were prospectively evaluated. Yearly recurrence of bleeding was evaluated once a year for 5 years postoperatively. Range of motion (ROM) and radiographic staging, as well as results of the short form (SF)-36 and subjective knee form of the International Knee Documentation Committee (IKDC) and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) questionnaires, were evaluated before surgery and at the end of follow-up. RESULTS: Mean bleeding recurrence was significantly reduced during the 5-year follow-up period. Questionnaire results showed significant improvements (IKDC P = 0.015, WOMAC P = 0.011, and SF-36 P = 0.023), whereas ROM was not significantly affected. Arthropathy progressed from Arnold-Hilgartner radiographic stage III to stage IV (P = 0.0082). CONCLUSIONS: Two-portal knee arthroscopic synovectomy was effective at reducing bleeding recurrence and improving knee function and QOL in patients with haemophilia, but did not interrupt the progression of radiographic changes.


Assuntos
Artroscopia , Hemartrose/cirurgia , Articulação do Joelho/cirurgia , Sinovectomia , Adolescente , Adulto , Artroscopia/métodos , Criança , Indicadores Básicos de Saúde , Hemartrose/etiologia , Hemofilia A/complicações , Humanos , Masculino , Cuidados Pós-Operatórios , Estudos Prospectivos , Qualidade de Vida , Amplitude de Movimento Articular , Recidiva , Resultado do Tratamento , Adulto Jovem
7.
Arq Bras Cardiol ; 96(3 Suppl 1): 1-68, 2011.
Artigo em Inglês, Português | MEDLINE | ID: mdl-21655875
8.
Arq. bras. cardiol ; 96(3,supl.1): 1-68, 2011. ilus, tab
Artigo em Português | LILACS | ID: lil-588887
9.
Medicina (B Aires) ; 68(3): 227-42, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18689157

RESUMO

The Committee of Latin America on the Therapeutics of Inhibitor Groups (CLOTTING) is composed of a number of hemophilia specialists from Latin America. The group aims to encourage the adoption of a good standard of care for Latin American patients with hemophilia. The occurrence of inhibitors in patients with hemophilia poses clinical challenges, and it is estimated that between 1000 and 3000 patients in Latin America are affected by hemophilia with inhibitors. There is an urgent need to establish a regional consensus and clinical guidelines for the diagnosis and treatment of these patients. We present an extensive review based on best current clinical practice and published literature, as seen from a Latin American perspective, taking into account the variable nature of hemophilia care available in the various countries in this Region.


Assuntos
Coagulantes/administração & dosagem , Fator IX/administração & dosagem , Fator VIII/administração & dosagem , Hemofilia A/tratamento farmacológico , Hemofilia B/tratamento farmacológico , Adulto , Fatores de Coagulação Sanguínea/administração & dosagem , Criança , Relação Dose-Resposta a Droga , Esquema de Medicação , Quimioterapia Combinada , Hemofilia A/classificação , Hemofilia A/diagnóstico , Hemofilia B/classificação , Hemofilia B/diagnóstico , Humanos , América Latina , Guias de Prática Clínica como Assunto , Índice de Gravidade de Doença
12.
Cerebrovasc Dis ; 19(3): 147-51, 2005.
Artigo em Inglês | MEDLINE | ID: mdl-15644626

RESUMO

BACKGROUND: Cerebral venous thromboses (CVT) with distinct clinical presentations have been shown worldwide. However, there is little information regarding race-ethnic differences in this disease. METHODS: We prospectively studied 50 CVT patients from Brazil, comparing clinical and laboratory data among white (W) and African-Brazilian (AB) patients. RESULTS: Seventy percent of the patients were female, 26 W and 23 AB, mean age 34.7 years. Multiple sinus CVT, deep CVT and worse outcome were significantly more frequent in AB than in W patients. There was a trend towards a higher frequency of factor V Leiden and prothrombin mutation in W than in AB, and of protein C deficiency in AB. CONCLUSIONS: CVT was more severe in AB patients than in W patients. Race-ethnic differences may account for the heterogeneous distribution of inherited thrombophilia in this series.


Assuntos
Trombose Intracraniana/etnologia , Trombose Venosa/etnologia , Adolescente , Adulto , Grupo com Ancestrais do Continente Africano , Idoso , Idoso de 80 Anos ou mais , Grupo com Ancestrais do Continente Asiático , Brasil/epidemiologia , Criança , Estudos de Coortes , Grupo com Ancestrais do Continente Europeu , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Fatores de Risco , População Urbana
13.
Sao Paulo; s.n; 1999. 119 p. ilus, tab, graf.
Tese em Português | Hanseníase | ID: han-13221

RESUMO

A hanseniase e uma doenca infecciosa cronica, cujo curso evolutivo, lento e insidioso, pode ser interrompido por episodios agudos ou subagudos, caracterizando os periodos reacionais. O comprometimento vascular e muito frequente, sendo a vasculite cronica um achado comum no doente multibacilar. Durante os surtos de reacao tipo II foram relatados episodios de tromboses venosa e/ ou arterial, muitas vezes constituindo a causa de obito desses doentes. Analisamos as dosagens plasmaticas de trombomodulina e do antigeno do fator de von Willebrand como marcadores de lesao e disfuncao endotelial, respectivamente, em doentes multibacilares, durante episodios de reacao tipo II e fora dos periodos reacionais. Apos consentimento previo informado, foram coletadas 163 amostras de um total de 34 doentes com hanseniase, formas multibacilares de sofrerem reacao tipo II AU)


Assuntos
Hanseníase Virchowiana/sangue , Endotélio Vascular/lesões , Trombomodulina/análise , Fator de von Willebrand/análise , Biomarcadores/análise
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