neonatal purpura fulminans

نویسندگان

reza saeidi department of pediatrics, associate professor of neonatology, mashhad university of medical sciences,mashhad, iran

reza gharaee department of pediatrics, fellow of neonatology, mashhad university of medical sciences , mashhad, iran

zohreh nobakht rheumatologist, mashhad university of medical sciences , mashhad, iran

چکیده

neonatal purpura fulminans is a rare and life threatening disease that can be inherited or acquired in etiology. it manifests as dic and extensive subcutaneous thrombosis. the condition is often fatal unless there is prompt diagnosis, and judicious therapy. the most important causes of this condition are infections and congenital deficiency of anticoagulant proteins c and s.in the case of pc (protein c) deficiency,the management includes an acute phase of replacement therapy with fresh frozen plasma (ffp) or protein c concentrate and a maintenance therapy that includes anticoagulation with warfarin or low molecular weight heparin. here we report a case of neonatal purpura fulminans due to suspected protein c deficiency.

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Neonatal Purpura Fulminans

Neonatal purpura fulminans is a rare and life threatening disease that can be inherited or acquired in etiology. It manifests as DIC and extensive subcutaneous thrombosis. The condition is often fatal unless there is prompt diagnosis, and judicious therapy. The most important causes of this condition are infections and congenital deficiency of anticoagulant proteins C and S.In the case of PC (p...

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Neonatal purpura fulminans is a rare, life-threatening condition, caused by congenital or acquired deficiencies of protein C or S. The condition is often fatal unless there is early recognition of the clinical symptoms, prompt diagnosis, and judicious replacement therapy is initiated. The clinical presentation is that of acute disseminated intravascular coagulation and hemorrhagic skin necrosis...

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Neonatal purpura fulminans is a life threatening clinical entity characterized by extensive subcutaneous thrombosis and disseminated intravascular coagulation usually manifesting shortly after birth. We report an autosomal recessive form of the disease in a neonate who was diagnosed with compound heterozygosity for mutations in his protein C gene as the molecular basis of his disorder.

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عنوان ژورنال:
iranian journal of neonatology

جلد ۵، شماره ۱، صفحات ۳۴-۳۷

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