RESUMO
INTRODUCTION: Rare coagulation factor deficiencies are less-known disorders with variable effects on the patient's life. Management of such patients is a challenge due to the paucity of evidence-based data, more so when patients with these rare disorders encounter a more rare, related condition, like inhibitor development or thrombosis. AREA COVERED: A comprehensive literature search related to RCFDs and management was performed in PubMed in order to discuss therapeutic options and challenges, prophylaxis, management of minor and major surgeries, obstetric and gynecological complications, inhibitor development, and thrombosis. EXPERT OPINION: Although significant changes have occurred in the management of RCFDs in recent years, more evidence-based studies besides expert opinion are needed for optimal management.
Assuntos
Transtornos de Proteínas de Coagulação/tratamento farmacológico , Transtornos de Proteínas de Coagulação/etiologia , Doenças Raras/tratamento farmacológico , Doenças Raras/etiologia , Biomarcadores , Coagulação Sanguínea/efeitos dos fármacos , Fatores de Coagulação Sanguínea/genética , Fatores de Coagulação Sanguínea/uso terapêutico , Transtornos de Proteínas de Coagulação/diagnóstico , Transtornos de Proteínas de Coagulação/prevenção & controle , Terapia Combinada , Gerenciamento Clínico , Suscetibilidade a Doenças , Feminino , Humanos , Gravidez , Complicações Hematológicas na Gravidez/diagnóstico , Complicações Hematológicas na Gravidez/tratamento farmacológico , Complicações Hematológicas na Gravidez/etiologia , Prognóstico , Doenças Raras/diagnóstico , Doenças Raras/prevenção & controle , Resultado do TratamentoAssuntos
Transtornos de Proteínas de Coagulação/fisiopatologia , Hemofilia A/fisiopatologia , Hemofilia B/fisiopatologia , Doenças de von Willebrand/fisiopatologia , Anestesia Geral , Transtornos de Proteínas de Coagulação/prevenção & controle , Hemofilia A/prevenção & controle , Hemofilia B/prevenção & controle , Humanos , Procedimentos Cirúrgicos Operatórios , Doenças de von Willebrand/prevenção & controleRESUMO
We compared the effects of two third-generation progestogens, desogestrel (DSG) and gestodene (GSD), on coagulation and fibrinolysis in Brazilian users of oral contraceptives (OCs). Forty-six women were evaluated before treatment and after six cycles of treatment. The coagulation, anticoagulant, and fibrinolytic systems were investigated. During the use of the DSG-containing OC, the activity of factors VII, VIII, IX, X, and XII increased significantly whereas the GSD-containing OC caused no changes in coagulation parameters. Concerning the anticoagulant pathways, the DSG-containing OC increased protein C levels and decreased total protein S levels, and the GSD-containing OC only decreased total protein S. Both OCs increased plasminogen activity, although the DSG-containing OC increased fibrin degradation products levels and decreased the tissue plasminogen activator antigen. In conclusion, we have found that in Brazilian women the effects of DSG and GSD on hemostatic parameters are different and, therefore, third-generation progestogens may not contribute equally to the thrombotic risk.
