NECROSE CUTANEE REVELANT UN DEFICIT CONGENITAL EN PROTEINE C : A PROPOS DE 3 CAS
- Service de Neonatologie Et Reanimation Neo-Natale, CHU Hassan II, Universite Sidi Mohammed Ben Abdellah, Fes, Maroc.
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Neonatal skin necrosis in the context of a congenital homozygous protein C deficiency is a rare inherited autosomal recessive disorder, it is characterized by rapidly extensive necrotic patches occurring a few hours after birth in a newborn who doesnt present any hemodynamic disorder. The diagnosis is based on the assay of protein C activity which is collapsed or even undetectable. Early diagnosis and replacement therapy are the mainstays of management before the onset of disseminated intravascular coagulation. We report three cases of newborns presenting with DIC in the context of protein C deficiency and the course of which was fatal.
[S. Halouani, W. Kojmane, F. Hmami and S. Atmani (2021); NECROSE CUTANEE REVELANT UN DEFICIT CONGENITAL EN PROTEINE C : A PROPOS DE 3 CAS Int. J. of Adv. Res. 9 (Mar). 80-83] (ISSN 2320-5407). www.journalijar.com
chu hassan II Fès Maroc
Morocco