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homozygous protein c deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein c concentrate
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نویسنده
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kizilocak hande ,ozdemir nihal ,dikme gürcan ,koc begum ,celkan tiraje
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منبع
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journal of thrombosis and thrombolysis - 2018 - دوره : 45 - شماره : 2 - صفحه:315 -318
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چکیده
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Purpura fulminans in neonates is a rapidly progressive thrombotic disorder manifesting as hemorrhagic skin infarction and disseminated intravascular coagulation. being inherited in an autosomal dominant manner, it is a medical emergency. clinical presentations of patients vary depending on the genetic mutations. retinal and intracranial hemorrhages are the worst clinical scenarios with persistent morbidity. during acute phase, fresh frozen plasma, protein c concentrates and anticoagulant therapy should be administered rapidly. here we report a patient with homozygous protein c deficiency.
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کلیدواژه
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neonatal purpura fulminans ,protein c deficiency ,low molecular weight heparin ,fresh frozen plasma ,protein c concentrate
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آدرس
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istanbul university, cerrahpasa faculty of medicine, division of pediatric hematology-oncology, turkey, kanuni sultan süleyman training and research hospital, division of pediatric hematology-oncology, turkey, istanbul university, cerrahpasa faculty of medicine, division of pediatric hematology-oncology, turkey, istanbul university, cerrahpasa faculty of medicine, division of pediatric hematology-oncology, turkey, istanbul university, cerrahpasa faculty of medicine, division of pediatric hematology-oncology, turkey
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Authors
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