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2016, Number 1

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Rev Cubana Pediatr 2016; 88 (1)

Congenital C protein deficiency in a newborn with extensive thrombosis and necrosis of tissues

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

Language: Spanish
References: 31
Page: 88-98
PDF size: 184.19 Kb.


ABSTRACT

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.


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