Pathophysiology

Pathophysiology Study Guide

©2018 of 131 Also known as factor VII deficiency, hemophilia A is a result of a lack of the clotting protein, factor VII, in the blood. This disorder is genetic in nature and is either passed from parents to children or occurs due to a mutated gene. The symptoms of this disorder include extended duration of bleeding, which can be both external from cuts and trauma, or internal bleeding from joints and muscles. Hemophilia A can be mild when the levels of factor VII range between 6% and 49%, as compared to the normal range of 50% to 150%; or mild ranging between 1% and 5%; or severe when the factor VII falls below 1%. People suffering from mild hemophilia A will experience bleeding issues only after serious injuries, surgery, or trauma. Hemophilia A, in mild forms, can cause menorrhagia, heavy menstruation, and even hemorrhage soon after childbirth among women. In moderate hemophilia A, people experience unusual bleeding after injuries, while in extreme cases, severe bleeding occurs after injuries, which repeats frequently, often causing bleeding from joints and muscles. Hemophilia B Hemophilia B is a bleeding disorder that is caused due to the deficiency of blood clotting factor IX in the blood. Just like hemophilia A, it is either inherited or caused due to a mutated gene. The major symptom of this disorder is bleeding, while others include bleeding in joints coupled with pain and swelling, bleeding while passing urine or stool, bruising, hemorrhage in the gastrointestinal and urinary tract, nose bleeds, spontaneous bleeding, and prolonged bleeding after injuries, tooth procedures, or surgeries. Tests that help diagnose hemophilia B disorder include partial thromboplastin time, prothrombin time, bleeding time, fibrinogen level, and serum factor IX activity. Other Coagulation Deficiencies Hemophilia C is caused by a deficiency of the clotting factor XI in the blood. Symptoms include blood in the urine, bruises, prolonged bleeding, and nosebleeds. It causes episodes of prolonged bleeding among women soon after childbirth. Another clotting disorder, known as Hypoprothrombinemia or factor II deficiency, is caused due to lack of Vitamin K. Symptoms include severe hemorrhage, bruising, nosebleeds, postpartum hemorrhage, prolonged bleeding, and hemorrhage after surgery. It is not a genetic disorder but is acquired and results from diseases of the liver or newborn hemorrhagic diseases. Factors Predisposing to Thrombosis Deep vein thrombosis is developed when blood clotting happens inside the deep veins of the body. These clots prevent the blood from circulating normally inside the body. Complications associated with thrombosis are development of pulmonary embolism and postphlebitic syndrome. Risk factors for thrombosis include genetic or inheritance of blood clotting disorders; prolonged resting on bed, like after paralysis or a lengthy stay in hospital; injury; surgery; pregnancy; hormone replacement therapy; birth control pills; obesity; smoking; cancer; failure of the heart; inflammatory bowel disease; family history of pulmonary embolism or thrombosis; age over 60 years; and sitting in one place for long duration. Achieve Page 43