RESUMO
There is little experience in the prevention of the severe hemorrhagic diathesis of factor X coagulation factor deficiency, before surgical procedures. A female with congenital deficiency of factor X that received prothrombin complex concentrates that contained 1000 IU of factor X (16.7 U/I Kg BW) inmediately prior to a cesarean section in two occasions, is reported. Factor X concentration rose from 1 to 25 percent in the first ocassion and from 10 to 63 percent in the second. In both episodes, factor X decreased in the first 24 h of the postoperative period and required new infusions of prothrombin complex concentrates. No episodes of abnormal bleeding were observed. It is concluded that the infusion of prothrombin complex concentrates prevents the hemorrhagic diathesis of factor X deficiency, despite its modest increase in plasma. A initial infusion of 1.000 UI of factor X (16-20 IU/Kg BW), followed by 500 IU (8-10 IU/Kg) every 24 hours is suggested for an adequate management of this condition
Assuntos
Humanos , Feminino , Gravidez , Adulto , Transtornos da Coagulação Sanguínea/congênito , Deficiência do Fator X/congênito , Transtornos Hemorrágicos/tratamento farmacológico , Tempo de Protrombina , Fator X/administração & dosagem , Cesárea/métodos , Complicações Hematológicas na Gravidez/tratamento farmacológicoRESUMO
Se analizan las principales características clínicas y de laboratorio de las deficiencias aisladas y combinadas de los factores II, VII y X. Se señala la importancia del uso de diferentes reactivos y metodologías para poderlos clasificar, sobre todo las variantes, debido a que en estos casos se presentan niveles inmunológicos normales con niveles disminuidos de actividad funcional o coagulante. Se discute brevemente el diagnóstico diferencial de estos trastornos, asi como se esbozan algunas pautas para el manejo del paciente con estas complicaciones hemorragicas hereditarias