Purpura fulminans in a case of protein C deficiency. 1988

S Ozsoylu, and B Cengiz, and A Karabent
Hacettepe University Faculty of Paediatrics, Haematology Unit, Ankara, Turkey.

We report a case of protein C deficiency which presented with purpura fulminans. The inheritance of protein C deficiency is discussed and the importance of warfarin (Coumadin) treatment in this conditions is emphasized.

UI MeSH Term Description Entries
D011693 Purpura Purplish or brownish red discoloration, easily visible through the epidermis, caused by hemorrhage into the tissues. When the size of the discolorization is >2-3 cm it is generally called Ecchymoses (ECCHYMOSIS). Petechiae,Purpuras
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D004211 Disseminated Intravascular Coagulation A disorder characterized by procoagulant substances entering the general circulation causing a systemic thrombotic process. The activation of the clotting mechanism may arise from any of a number of disorders. A majority of the patients manifest skin lesions, sometimes leading to PURPURA FULMINANS. Consumption Coagulopathy,Coagulation, Disseminated Intravascular,Disseminated Coagulation, Intravascular,Intravascular Coagulation, Disseminated,Intravascular Disseminated Coagulation,Coagulation, Intravascular Disseminated,Coagulations, Disseminated Intravascular,Coagulations, Intravascular Disseminated,Coagulopathies, Consumption,Coagulopathy, Consumption,Consumption Coagulopathies,Disseminated Coagulations, Intravascular,Disseminated Intravascular Coagulations,Intravascular Coagulations, Disseminated,Intravascular Disseminated Coagulations
D005260 Female Females
D005268 Femoral Vein The vein accompanying the femoral artery in the same sheath; it is a continuation of the popliteal vein and becomes the external iliac vein. Femoral Veins,Vein, Femoral,Veins, Femoral
D006493 Heparin A highly acidic mucopolysaccharide formed of equal parts of sulfated D-glucosamine and D-glucuronic acid with sulfaminic bridges. The molecular weight ranges from six to twenty thousand. Heparin occurs in and is obtained from liver, lung, mast cells, etc., of vertebrates. Its function is unknown, but it is used to prevent blood clotting in vivo and vitro, in the form of many different salts. Heparinic Acid,alpha-Heparin,Heparin Sodium,Liquaemin,Sodium Heparin,Unfractionated Heparin,Heparin, Sodium,Heparin, Unfractionated,alpha Heparin
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013927 Thrombosis Formation and development of a thrombus or blood clot in BLOOD VESSELS. Atherothrombosis,Thrombus,Blood Clot,Blood Clots,Thromboses
D014859 Warfarin An anticoagulant that acts by inhibiting the synthesis of vitamin K-dependent coagulation factors. Warfarin is indicated for the prophylaxis and/or treatment of venous thrombosis and its extension, pulmonary embolism, and atrial fibrillation with embolization. It is also used as an adjunct in the prophylaxis of systemic embolism after myocardial infarction. Warfarin is also used as a rodenticide. 4-Hydroxy-3-(3-oxo-1-phenylbutyl)-2H-1-benzopyran-2-one,Aldocumar,Apo-Warfarin,Coumadin,Coumadine,Gen-Warfarin,Marevan,Tedicumar,Warfant,Warfarin Potassium,Warfarin Sodium,Potassium, Warfarin,Sodium, Warfarin
D020151 Protein C Deficiency An absence or deficiency in PROTEIN C which leads to impaired regulation of blood coagulation. It is associated with an increased risk of severe or premature thrombosis. (Stedman's Med. Dict., 26th ed.) Deficiency, Protein C,Hereditary Thrombophilia Due To Protein C Deficiency,Deficiencies, Protein C,Protein C Deficiencies

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