Abstract

ACOSTA BATISTA, Bárbara et al. Congenital C protein deficiency in a newborn with extensive thrombosis and necrosis of tissues. Rev Cubana Pediatr [online]. 2016, vol.88, n.1. ISSN 0034-7531.

One of the most serious hematological disorders of the neonatal period is congenital C protein deficiency of very rare occurrence and the main cause of severe thromboembolic disease and purpura fulminans in newborns. It may be summarized as a clinical and pathological entity of acute occurrence, with dermis vasculature thrombosis that leads to progressive hemorrhagic necrosis of the skin, associated to disseminate intravascular coagulation and perivascular hemorrhage in the neonatal period. The patient of this report showed the clinical and pathological elements characterizing purpura fulminans the origin of which is due to hereditary C protein deficiency that led to onset of severe thrombotic complications in this patient.

Keywords : purpura fulminans; thromboembolism; hereditary C protein deficiency; disseminate intravascular coagulation; newborn.

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