Protein C deficiency

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[Protein C deficiency].

Severe protein C deficiency (i.e. protein C activity <1 IU dL(-1)) is a rare autosomal recessive disorder that usually presents in the neonatal period with purpura fulminans (PF) and severe disseminated intravascular coagulation (DIC), often with concomitant venous thromboembolism (VTE). Recurrent thrombotic episodes (PF, DIC, or VTE) are common. Homozygotes and compound heterozygotes often pos...

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Ophthalmic manifestations of neonatal protein C deficiency.

Protein C is a vitamin K dependent plasma zymogen which acts by regulating coagulation by its specific inactivation of factors V and VII, and facilitates fibrinolysis by elevating circulating plasminogen activator levels. Protein C deficiency is a rare condition which predisposes to episodes ofpotentially blinding and lethal thromboembolism. In the newborn period, congenital protein C deficienc...

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Ischemic stroke due to protein C deficiency.

Plasma protein C exerts anticoagulatory effects by inactivating factors V and VIII. Hereditary protein C deficiency is transmitted as an autosomal dominant disorder. Homozygous individuals usually develop purpura fulminans as newborns; heterozygous protein C-deficient individuals are at increased risk for venous thrombosis and pulmonary embolism. However, arterial thrombosis has been only rarel...

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Protein C deficiency in liver disease.

Protein C is a vitamin K-dependent zymogen of a serine protease that is found in blood plasma. The active form, activated protein C, can inhibit blood coagulation and stimulate fibrinolysis. Protein C is synthesized in the liver as a single chain protein. Its synthesis requires several post-translational modifications including carboxylation of glutamic acid residues, hydroxylation of aspartic ...

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Cerebral infarction associated with protein C deficiency.

BACKGROUND AND PURPOSE A deficiency of plasma protein C, both the hereditary and acquired types, is one cause of thromboembolic disease. Several antineoplastic agents have been reported to decrease the production of protein C in the liver by impairing either the absorption or metabolism of vitamin K, leading to acquired protein C deficiency. CASE DESCRIPTION We treated a young woman with prot...

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ژورنال

عنوان ژورنال: Indian Journal of Hematology and Blood Transfusion

سال: 2013

ISSN: 0971-4502,0974-0449

DOI: 10.1007/s12288-013-0298-4