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Rev. esp. enferm. dig ; 110(8): 522-526, ago. 2018. tab
Artigo em Espanhol | IBECS | ID: ibc-177764

RESUMO

Introducción: la infección por virus hepatitis C (VHC) ha sido causa de importante morbi-mortalidad en hemofilia, planteándose el trasplante hepático (TH) por cirrosis y/o carcinoma hepatocelular (CHC). Caso clínico: presentamos los casos con coagulopatías congénitas e infección por VHC sometidos a TH en nuestro centro: tres pacientes con hemofilia A y uno con enfermedad de von Willebrand (EvW) tipo 3. Evaluamos el curso de la coagulopatía, el manejo perioperatorio, el consumo de factor y componentes sanguíneos y la supervivencia postrasplante. El factor deficitario se comenzó a administrar en bolo iv directo una hora antes del inicio de la cirugía para alcanzar un nivel deseado de dicho factor de 100 UI/dl, mantenido hasta conseguir el control estable de la hemostasia. Los tres pacientes con hemofilia A curaron su coagulopatía postrasplante. El factor VIII (FVIII) fue 93 UI/dl a los once años, 59 UI/dl a los 13 meses y 109 UI/dl a los nueve meses postrasplante en cada uno de los casos. El consumo medio perioperatorio de concentrados de FVIII fue 175 UI/kg, infundido hasta 36 h postrasplante de media. El paciente con EvW tipo 3 consiguió atenuar el curso natural de su sintomatología hemorrágica sin que se detectaran niveles hemostáticos del antígeno del factor von Willebrand (FVW:Ag) postrasplante. Discusión: tras el trasplante hepático, se produce la curación de la hemofilia A y la mejoría del fenotipo hemorrágico en la EvW tipo 3


Introduction: infection with the hepatitis C virus (HCV) causes significant morbidity and mortality in patients with hemophilia. Finally, patients are considered for a liver transplantation (LT) due to cirrhosis and/or hepatocellular carcinoma (HCC). Case report: we report the cases of congenital coagulopathy and HCV infection that underwent LT in our institution. There were three patients with hemophilia A and one patient with von Willebrand disease (vWD) type 3. The coagulopathy outcome, perioperative management, factor and blood product usage and post-transplant survival were assessed. The deficient factor was initially administered in a direct bolus one hour before surgery with a target level of 100 IU/dl, which was sustained until stable hemostasis was reached. All three patients with hemophilia A were cured of their coagulopathy following transplantation. Factor VIII (FVIII) was 93 IU/dl at eleven years, 59 IU/dl at 13 months and 109 IU/dl at nine months post-transplant, in each case. The mean perioperative usage of FVIII concentrates was 175 IU/kg; concentrates were infused for an average of 36 hours post-transplant. The natural course of the bleeding symptoms of the patient with type-3 vWD was attenuated, with no detectable hemostatic levels of von Willebrand factor antigen (vWF:Ag) after transplantation. Discussion: after transplantation, hemophilia A cure and improved bleeding phenotype of type-3 vWD reduced morbidity and mortality. However, potential graft reinfection with HCV and relapsing HCC cast a shadow over these optimum results


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Transplante de Fígado/métodos , Hemofilia A/complicações , Doença de von Willebrand Tipo 3/complicações , Hepatite C Crônica/cirurgia , Transtornos da Coagulação Sanguínea/complicações , Monitorização Intraoperatória/métodos , Testes de Função Hepática/estatística & dados numéricos
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