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hemophilia B |
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hemophilia /he·mo·phil·ia/ (he″mo-fil´e-ah) a hereditary hemorrhagic diathesis due to deficiency of a blood coagulation factor. hemophilia A classical h.; an X-linked recessive form due to deficiency of coagulation factor VIII. hemophilia B Christmas disease; an X-linked recessive form due to deficiency of coagulation factor IX. hemophilia C an autosomal disorder due to lack of coagulation factor XI; seen predominantly in persons of Jewish ancestry and characterized by minor bleeding, mild bruising, severe prolonged postsurgical bleeding, and abnormal clotting test times. classical hemophilia h. A. vascular hemophilia von Willebrand's disease.
hemophilia B, a hereditary blood disorder, transmitted as an X-linked recessive trait and caused by a deficiency of factor IX, the plasma thromboplastin component. The condition is clinically similar to but less severe than hemophilia A. Also called Christmas disease. See coagulation factor, hemophilia. hemophilia (bleeder's disease) (hē´mōfil´ē n a sex-linked genetic disease manifested in males and characterized by severe hemorrhage. hemophilia A (classic hemophilia), n a hemorrhagic diathesis resulting from a deficiency of antihemophilic globulin (AHG); inherited as a recessive sex-linked characteristic and characterized by recurrent bouts of bleeding from even trivial injury. The coagulation time is prolonged, but the bleeding time is normal. hemophilia B (Christmas disease, hemophilia II, hemophilioid state C), n a hemorrhagic diathesis resulting from a deficiency of plasma thromboplastin component (PTC); transmitted as a sex-linked recessive characteristic and characterized clinically by the same manifestations as classic hemophilia. There is a delay in the generation of thromboplastin. The platelet count, bleeding time, tourniquet test, and thrombin and prothrombin times are normal. hemophilia C (plasma thromboplastin antecedent [PTA] deficiency, Rosenthal's syndrome), n a hemophilia-like condition believed to result from a deficiency of plasma thromboplastin antecedent (PTA), transmitted as a simple autosomal dominant trait and characterized by a moderate bleeding tendency after extraction of teeth or after tonsillectomy. Prothrombin consumption and thromboplastin generation are abnormal. See also factor XI. hemophilia, classic, n See hemophilia A. hemophilia, vascular, n a hereditary hemorrhagic disorder affecting both sexes and associated with a deficiency of antihemophilic globulin and vascular abnormalities characteristic of pseudohemophilia (von Willebrand disease). The bleeding time is prolonged, and severity of bleeding varies considerably among persons with this condition. hemophilia a condition characterized by impaired coagulability of the blood, and a strong tendency to bleed. See also deficiency of the following clotting factors, afibrinogenemia and hypoprothrombinemia, and proconvertin, stuart factor, plasma thromboplastin antecedent, hageman factor and fibrin stabilizing factor. hemophilia A classical hemophilia, due to deficiency of clotting factor VIII-C; occurs in dogs, horses and cats and is transmitted by the female to the male as a sex-linked recessive abnormality. hemophilia B a form similar to classical hemophilia but due to a deficiency of clotting factor IX; called also christmas disease and factor IX deficiency. Occurs in dogs and cats. hemophilia C in dogs an autosomal dominant form due to deficiency of clotting factor XI (plasma thromboplastin antecedent). Called also factor XI deficiency. The disease also occurs in cattle but the clinical disease is minor and it is inherited as a recessive character. double hemophilia dogs with both hemophilia A and hemophilia B have been produced experimentally. vascular hemophilia deficiency of clotting factor VIII and factor VIII-related antigen occurs in many breeds of dogs, particularly Doberman Pinschers, and in rabbits and pigs. See also von willebrand's disease. hemophilia B Christmas disease, factor IX hemophilia Hematology An X-R form of hemophilia caused by a defect in factor IX; it is clinically similar to hemophilia A, and often first manifest by hemarthrosis with joint destruction; these Pts
are often infected with blood-borne viruses–eg, HBV, HCV, HIV, parvoviruses; with time, IgG antibodies may develop against factor IX. See Hemophilia. Cf Thalassemia. Patient discussion about hemophilia B. Q. A christmas wish for each of you! To have all the presents in the world is not my desire for you! May your Christmas gift from me today be a sincere prayer that you will know: Peace that makes you sleep like a baby Joy that makes you laugh like a fool Happiness that lets you forget the size of your problems Hope that makes impossible dreams come true Love that makes you give from your heart Forgiveness when ‘you know not what you do”! Kindness that brings favor wherever you go And health that makes you act the age you choose. A. Each and everyone of you is in my thoughts and prayers. I wish you each a happy holidays and hope for the best the world has to offer for each and every one of you in the new year. Read more or ask a question about hemophilia BHow to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit webmaster's page for free fun content. |
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Normal blood quickly gels when it spills from blood vessels, but that of people and dogs with hemophilia B lacks a clotting protein called factor IX. Hemophilia B is characterized by deficient or defective factor IX. FIX:Fc is being developed for the treatment of hemophilia B in a strategic alliance with Biovitrum AB of Sweden with the companies sharing equally the costs and profits of development and commercialization. |
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