RESUMEN
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.
Asunto(s)
Trastornos de las Proteínas de Coagulación/tratamiento farmacológico , Trastornos de las Proteínas de Coagulación/etiología , Enfermedades Raras/tratamiento farmacológico , Enfermedades Raras/etiología , Biomarcadores , Coagulación Sanguínea/efectos de los fármacos , Factores de Coagulación Sanguínea/genética , Factores de Coagulación Sanguínea/uso terapéutico , Trastornos de las Proteínas de Coagulación/diagnóstico , Trastornos de las Proteínas de Coagulación/prevención & control , Terapia Combinada , Manejo de la Enfermedad , Susceptibilidad a Enfermedades , Femenino , Humanos , Embarazo , Complicaciones Hematológicas del Embarazo/diagnóstico , Complicaciones Hematológicas del Embarazo/tratamiento farmacológico , Complicaciones Hematológicas del Embarazo/etiología , Pronóstico , Enfermedades Raras/diagnóstico , Enfermedades Raras/prevención & control , Resultado del TratamientoAsunto(s)
Trastornos de las Proteínas de Coagulación/fisiopatología , Hemofilia A/fisiopatología , Hemofilia B/fisiopatología , Enfermedades de von Willebrand/fisiopatología , Anestesia General , Trastornos de las Proteínas de Coagulación/prevención & control , Hemofilia A/prevención & control , Hemofilia B/prevención & control , Humanos , Procedimientos Quirúrgicos Operativos , Enfermedades de von Willebrand/prevención & controlRESUMEN
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.
