Browsing by Author "Diniz, MJ"
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- Causas de Morte em Doentes com Hemofilia: Estudo Retrospectivo de 1979 a 2007, no Serviço de Imunohemoterapia do HSJPublication . Costa, C; Antunes, M; Diniz, MJ
- Cirurgia e Hemofilia com Inibidor: 4 Casos ClínicosPublication . Santos, A; Diniz, MJ; Antunes, M
- Cost of Immune Tolerance Induction in Hemophilia A Patients: Results from the ITER StudyPublication . Fusco, F; Gringeri, A; Scalone, L; Mantovani, L; Rocino, A; Altisent, C; Astermark, J; Diniz, MJ; Fijnvandraat, K; Klamroth, R; Lambert, T; Lavigne-Lissalde, G; Lopez Fernandez, MF; Morfini, M
- Elective Laparoscopic Cholecistectomy in a Severe Hemophilia A Patient with Inhibitors to Factor VIIPublication . Santos, A; Antunes, M; Diniz, MJ; Pimentel, T
- Glanzmann's Thrombastenia Treatment: a Center's ExperiencePublication . Santos, A; Antunes, M; Diniz, MJ
- HCV Infection in Patients with Hereditary Bleeding DisordersPublication . Caiado, A; Antunes, M; Santos, AL; Esteves, J; Diniz, MJIntroduction: Hepatitis C virus (HCV) infection in patients with hereditary bleeding disorders (HBDs), as a consequence of treatment with transfusion of human bloodderived components between the late 1970s and 1980s, represents a major health concern. Objectives: Assessment and evaluation of the burden of HCV infection, its complications, and treatment in a population of patients with HBDs. Methods: Analysis of a series of 161 patients with HBDs treated in the Immunohemotherapy Service of the Centro Hospitalar de Lisboa Central (Lisboa, Portugal), consultation and systematic review of the patients clinical processes, elaboration of a database comprising the information gathered; and statistical study of its variables: age, gender, degree of severity of the bleeding disorder, treatment modality, and major and minor complications of HCV infection. Results: Sixty-five (40%) of the 161 patients have HCV infection. Among the patients with hemophilia A, 36% are severe and 62% of those have HCV infection; 9% moderate with 57%; 25% mild with 20%. In the hemophilia B group, 8% are severe with 23% infected and 6% moderate or mild with 10%. Concerning the patients with von Willebrand disease, 12% have type 2 with 16% infected and 4% have type 3 with 86%. Conclusions: HCV infection represents a very significant complication of the treatment employed in the past in the studied population. Considering that most of these patients were infected in the late 1970s and early 1980s, and the natural evolution of HCV infection in patients without bleeding disorders, it is expected that the prevalence of major complications will rise significantly in the coming years. Prophylactic measures should be implemented to enhance the follow-up protocols and prevent further development of liver damage in these patients.
- Hemorragia do Psoas-Ilíaco em Doentes com Hemofilia. Experiência do Serviço de Imuno-Hemoterapia do Centro Hospitalar de Lisboa - Hospital de S. JoséPublication . Santos, N; Caldas, J; Antunes, M; Diniz, MJ
- Hemorragias do Sistema Nervoso Central em HemofiliaPublication . Leão, C; Dominguez, C; Vieira, A; Diniz, MJ; Carvalho, F
- Non-Genetic Risk Factors and the Development of Inhibitors in Haemophilia: a Comprehensive Review and Consensus ReportPublication . Astermark, J; Altisent, C; Batorova, A; Diniz, MJ; Gringeri, A; Holme, PA; Karafoulidou, A; Lopez-Fernández, MF; Reipert, BM; Rocino, A; Schiavoni, M; von Depka, M; Windyga, J; Fijnvandraat, KThe development of inhibitors to the infused factor in patients with haemophilia is a serious clinical problem. Recent evidence suggests that alongside the strong genetic contribution to inhibitor formation, there are a number of non-genetic factors--perceived by the immune system as danger signals--which promote formation of inhibitors. This study provides a comprehensive review of clinical studies relating to these factors and also presents a survey of opinion concerning their importance and clinical influence, conducted among the members of the European Haemophilia Treatment Standardisation Board (EHTSB). Taken together, this information highlights the lack of robust data concerning the influence of several non-genetic risk factors on inhibitor development, and an urgent need for prospective, well-conducted studies that adhere to recommendations made by the European Medicines Agency (EMEA) for studying inhibitors. Based on current literature, the EHTSB formulated consensus recommendations. It is desirable to minimize intensive treatment wherever possible, given the clinical situation. Prophylaxis should be offered to all children, although we still need to determine optimal dosing with respect to inhibitor development, and age for starting treatment. Vaccinations should be given subcutaneously and concomitant factor concentrate infusions avoided. According to the board, there is no evidence in the literature supporting suggestions that the type of concentrate influences inhibitor risk; but all patients should be monitored during their first exposures. Furthermore, there is no evidence to support an association between pregnancy-related issues, breast feeding and treatment-related factors (e.g. route of administration, or use of blood components) and inhibitor development.
- Prevalência de Inibidores nos Doentes da Consulta de Hemofilia do Serviço de Imuno-HemoterapiaPublication . Caldeira, C; Monteiro, A; Cerezo, E; Diniz, MJ
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