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1.
Int J Lab Hematol ; 46(1): 128-134, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37704365

RESUMO

INTRODUCTION: Haemophilia B (HB) is an X-linked hereditary bleeding disorder characterized by coagulation factor IX (FIX) deficiency. To improve the quality of life of patients and adherence to treatment, recombinant factor concentrates modified to extend their half-life have been developed, called extended half-life factors (EHL: extended half-life). Nonacog beta pegol (N9-GP) is a glycopegylated recombinant human FIX molecule that has a half-life of 93 h with a single dose and has shown a higher recovery percentage than other molecules. To diagnose and monitor the treatment of haemophiliac patients, FIX activity is determined with the one-stage clotting assay (OSA) and/or the chromogenic assay. The objective of this work, carried out in three centres, was to measure the recovery of N9-PG with 10 different activated partial thromboplastin time (APTT) reagents on three platforms, in samples spiked in vitro with N9-GP, at four different concentration levels. METHODS: It was measured the recovery of N9-GP with 10 different APTT reagents (polyphenol, ellagic acid, silice dioxide, colloidal silica as APTT activator on three platforms, in sample spiked in vitro with N9-GP. RESULTS: The results show heterogeneity in the activity of N9-GP measured by OSA with the different APTT reagents when the calibrations were performed with the specific calibrator of each coagulometer. A recovery percentage between 87% and 108% was obtained only with polyphenol and ellagic acid as activator in the three platforms evaluated. The other reagents studied overestimate or underestimate, with no clear profile. When a calibration curve was performed with a calibrator prepared from the N9-GP vial, all APTT reagents met the established recovery requirement. CONCLUSION: APTT reagents with polyphenol or ellagic acid as activator would be the only ones appropriate when using the commercially available OSA with specific calibrator to monitor patients treated with N9-GP.


Assuntos
Fator IX , Hemofilia B , Polietilenoglicóis , Humanos , Fator IX/uso terapêutico , Indicadores e Reagentes , Qualidade de Vida , Ácido Elágico/uso terapêutico , Hemofilia B/diagnóstico , Hemofilia B/tratamento farmacológico , Polifenóis/uso terapêutico , Proteínas Recombinantes
2.
Cardiovasc Intervent Radiol ; 36(4): 964-9, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23150120

RESUMO

PURPOSE: Congenital hemophilia is a hereditary bleeding disorder that affects 1 in 5,000 males and is characterized by repetitive musculoskeletal bleeding episodes. Selective embolization of the knee and elbow arteries can prevent bleeding episodes. To evaluate the long-term efficacy of these procedures, we assessed the outcomes of 30 procedures performed in our center. METHODS: We performed 30 procedures in 27 hemophilic patients, including 23 knee, and 7 elbow procedures. To evaluate the efficacy of selective embolization of knee and elbow arteries in people with hemophilia, we analyzed the number of bleeding episodes during 12 months before the procedure compared with the amount of episodes that occurred 3, 6, and 12 months after embolization. RESULTS: Twenty-nine of 30 procedures were classified as successful. The median of 1.25 episodes per month (range 0-3) observed before the procedure was reduced to 0 (range 0-1.67; p < 0.001) at 3 months, 0.17 (range 0-1.67; p < 0.001) at 6 months, and 0.33 (range 0-1.67; p = 0.024) at 12 months. Three patients remained free of bleeding events for more than 6 months. Additionally, after the procedure there was a significant reduction in factor FVIII usage that sustained up to 12 months after the procedures. No serious adverse events were observed. CONCLUSIONS: Selective angiographic embolization of knee and elbow arteries is a feasible procedure that can prevent repetitive bleedings, which would translate in better joint outcomes for these patients.


Assuntos
Embolização Terapêutica/métodos , Hemartrose/terapia , Hemofilia A/complicações , Sinovite/terapia , Adolescente , Adulto , Angiografia/métodos , Angiografia Digital/métodos , Criança , Doença Crônica , Estudos de Coortes , Articulação do Cotovelo/diagnóstico por imagem , Articulação do Cotovelo/fisiopatologia , Feminino , Seguimentos , Hemartrose/etiologia , Hemartrose/fisiopatologia , Hemofilia A/diagnóstico por imagem , Hemofilia A/terapia , Humanos , Articulação do Joelho/diagnóstico por imagem , Articulação do Joelho/fisiopatologia , Masculino , Pessoa de Meia-Idade , Radiografia Intervencionista/métodos , Estudos Retrospectivos , Medição de Risco , Sinovite/diagnóstico por imagem , Sinovite/etiologia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
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