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2016, Número 1

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Rev Cubana Pediatr 2016; 88 (1)


Deficiencia congénita de proteína C en un recién nacido con trombosis y necrosis de tejidos extensa

Acosta BB, Díaz ÁM, Fernández NR, Rivera AL, Chagues LO

Idioma: Español
Referencias bibliográficas: 31
Paginas: 88-98
Archivo PDF: 184.19 Kb.


RESUMEN

Uno de los trastornos hematológicos más graves del período neonatal es la deficiencia congénita de proteína C, de presentación muy rara, y causa de enfermedad tromboembólica severa y púrpura fulminante en recién nacidos. Se puede sintetizar como una entidad clínico-patológica, de aparición aguda, con trombosis de la vasculatura de la dermis, lo cual conduce a necrosis hemorrágica y progresiva de la piel, asociada a coagulación intravascular diseminada y hemorragia perivascular, que ocurre en el período neonatal. El paciente presentado exhibe los elementos clínicopatológicos que caracterizan la púrpura fulminante, cuyo origen se debe a una deficiencia hereditaria de proteína C, lo cual condujo a la aparición de complicaciones trombóticas severas.


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