Assuntos
Ácidos Linoleicos/efeitos adversos , Materia Medica/efeitos adversos , Parto , Óleos de Plantas/efeitos adversos , Plantas Medicinais , Púrpura/induzido quimicamente , Ácido gama-Linolênico/efeitos adversos , Adulto , Bebidas , Feminino , Humanos , Recém-Nascido , Ácidos Linoleicos/administração & dosagem , Materia Medica/administração & dosagem , Oenothera biennis , Extratos Vegetais/administração & dosagem , Extratos Vegetais/efeitos adversos , Folhas de Planta , Óleos de Plantas/administração & dosagem , Gravidez , Púrpura/sangue , Ácido gama-Linolênico/administração & dosagemRESUMO
We report a case of neonatal purpura fulminans associated with activated protein C resistance. Analysis of DNA demonstrated heterozygosity for the factor V R506Q mutation. The neonate, at 8 hours of age, had progressive purpuric skin lesions and later had evidence of microvascular, hemorrhagic thrombosis in the brain. The baby was treated with fresh frozen plasma infusions and had complete resolution of the skin lesions and no apparent long-term complications. We suggest that activated protein C resistance testing be included in the initial evaluation of neonatal purpura fulminans.
Assuntos
Fator V/genética , Púrpura/genética , Sequência de Bases , DNA/genética , Feminino , Humanos , Recém-Nascido , Embolia e Trombose Intracraniana/sangue , Dados de Sequência Molecular , Mutação , Reação em Cadeia da Polimerase , Proteína C/metabolismo , Tempo de Protrombina , Púrpura/sangueRESUMO
Ten patients with chronic purpuric and pigmented angiodermatitis in the extremities and two patients with acute and disseminated eczematoid-like purpura angiodermatitis were studied with current hemostasis parameters: (1) platelet aggregation, (2) platelet circulating aggregates, (3) clotting factor measurements and high molecular weight kininogen (HMWK) in their coagulation fraction. The results showed: (1) reactional thrombocytosis with morphologic changes; (2) increased levels of platelet circulating aggregates; (3) increased response of platelet aggregation to different agonists, especially at very low doses, and also when we used washed platelets resuspended in normal plasma; (4) delayed activation of the contact system; (5) decrease of the activity of the fibrinolysis activators; and (6) diminished function activity of the HMWK coagulation fraction. As a physiopathogenic hypothesis in these patients, there is a cutaneous pathology that could be entailed to these hematologic alterations of the platelet-HMWK-endothelial cells-kinins-coagulation-fibrinolysis system.