Hemophilia the royal disease
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very good presentatiion for the students.it consist of details about definition causes incidence clinical features investigation management prophylaxis common students use this presentation and prepare for the exams,...it is more than enough for your exam preparation
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This document discusses surgical hemostasis. It begins by outlining the learning objectives, which are to understand what hemostasis is, the causes of excessive bleeding during or after surgery, and how to evaluate a patient's hemostasis before surgery. It then defines hemostasis as the arrest of blood escape through natural or artificial means. It describes the natural hemostasis process involving vasoconstriction, platelet plug formation, and blood clot formation. It lists the 12 clotting factors and the intrinsic and extrinsic clotting pathways. The document concludes by discussing causes of bleeding during or after surgery, including defects of hemostasis, and the steps of pre-operative evaluation of a patient's hemost
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This document provides an overview of hemostasis, the process by which bleeding is stopped. It discusses the key mechanisms of hemostasis, including:
1) Vascular constriction which reduces blood flow to the injury site.
2) Formation of a platelet plug via platelet adhesion, granule release and aggregation.
3) Formation of a blood clot through the coagulation cascade which involves the intrinsic, extrinsic, and common pathways leading to thrombin generation and fibrin clot formation.
Investigation of hemostatic function includes tests of vascular hemostasis, platelet function, and coagulation factor assays to identify abnormalities that may cause bleeding disorders.
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1) Hemostasis is the process by which blood maintains its fluid state within vessels but also forms clots at sites of injury. It involves vasoconstriction, platelet plug formation, and coagulation cascade.
2) For blood not to clot normally in circulation, the endothelium prevents platelet adhesion, negatively charged particles repel clotting factors, circulation velocity must decrease for clotting, and natural anticoagulants and fibrinolysis are present.
3) Coagulation occurs through the intrinsic and extrinsic pathways activating prothrombin to thrombin which converts fibrinogen to fibrin threads forming a clot.
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Hemostasis is the process by which bleeding is stopped. It involves platelets, clotting factors, and the endothelium. When a blood vessel is injured, the processes of vascular constriction, platelet plug formation, fibrin formation, and fibrinolysis work together to form a blood clot. The extrinsic and intrinsic pathways activate coagulation factors that generate thrombin, which converts fibrinogen to fibrin to form the clot. Counterregulatory mechanisms then limit clotting and allow for clot resorption and tissue repair.
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2) Important bleeding disorders discussed include hemophilia A (factor VIII deficiency), hemophilia B (factor IX deficiency), von Willebrand disease (a disorder of the von Willebrand clotting factor), and fibrinogen deficiency.
3) These disorders are diagnosed through tests of bleeding time and clotting factor levels and activity. Treatment involves replacing the missing clotting factor through products like cryoprecipitate, desmopressin, or clotting factor concentrates.
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The document discusses blood clotting and the factors involved. Platelets and clotting factors work together to form blood clots which act as a mesh to stop bleeding from injuries. When a blood vessel is damaged, platelets stick to the injury site and clump together to form a platelet plug. Platelets and damaged tissue then release clotting factors which activate the blood clotting mechanism and produce fibrin to seal the wound. Impaired clotting can lead to excessive bleeding or blockages in arteries from blood clots.
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This document summarizes hemostasis, the process by which bleeding is stopped. It discusses the three components of hemostasis - extravascular, vascular, and intravascular. The normal hemostasis process involves platelet plug formation and fibrin clot formation via the coagulation cascade. Coagulation factors, platelets, and fibrinogen are involved. Hemostasis is balanced by natural anticoagulants. Genetic or acquired bleeding disorders can result from deficiencies in specific coagulation factors or platelets. Common disorders discussed include hemophilia A/B/C and von Willebrand disease.
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This document discusses bleeding and clotting disorders. It provides details on:
1. The normal physiology of hemostasis including vascular, platelet and plasma factors that prevent bleeding.
2. Specific platelet and plasma factor deficiencies that can cause bleeding disorders.
3. The classification of bleeding disorders into defects of blood vessels, platelet disorders, and clotting factor deficiencies.
4. Diagnostic tests for evaluating bleeding patients including screening coagulation tests, specific factor assays, and platelet function tests.
surgical haemostasis olofin.pptx
The document discusses surgical haemostasis, which is the process of preventing or stopping blood loss from injured blood vessels during or after surgery. It defines haemostasis and outlines its importance in surgery. The physiology of haemostasis is described, involving vasoconstriction, platelet plug formation, and coagulation/fibrin formation. Causes of bleeding during or after surgery are discussed, including defects in haemostasis or platelet function. Methods of achieving haemostasis are covered, such as mechanical techniques like pressure, sutures, and cauterization, as well as chemical agents, blood products, and thermal techniques. Management of haemostasis in the pre-operative, intra-operative, and post-operative periods
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Hemostasis is the process of blood clotting and subsequent dissolution of the clot following tissue repair. It involves 3 main events: 1) vascular constriction, 2) platelet aggregation forming a temporary plug, and 3) fibrin formation creating a stable clot. The clot must later dissolve for normal blood flow to resume. Von Willebrand disease is a bleeding disorder caused by deficient or abnormal von Willebrand factor, which helps platelets bind to collagen. It ranges from mild to severe and is diagnosed based on bleeding history and lab tests of von Willebrand factor and coagulation factors. Treatment depends on severity but may include desmopressin or coagulation factor replacement.
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The document discusses coagulation and disseminated intravascular coagulation (DIC). It begins by explaining the three stages of hemostasis: vascular spasm, primary hemostasis involving platelet plug formation, and secondary hemostasis involving fibrin strand formation. It then details the coagulation cascade and its four phases: initiation, amplification, propagation, and clot stabilization. The document concludes by covering the causes, mechanisms, clinical manifestations, diagnosis, differential diagnosis, and treatment of DIC.
Lecture 1 hemostasis first secion
Hemostasis is the body's response to bleeding and involves plasma factors, platelets, and blood vessels. It occurs in stages - first the blood vessel constricts, then platelets form a plug by adhering and aggregating at the site of injury. Finally, the coagulation cascade strengthens the platelet plug by converting fibrinogen to fibrin to form a mesh. The coagulation process and platelet plug formation are tightly regulated to be localized to the injury site and later resolved by the fibrinolytic system which breaks down the clot to restore vessel patency.
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This document summarizes the role of platelets in hemostasis, which is the arrest of bleeding through a complex interaction between blood vessels, platelets, and plasma proteins. It occurs in two stages: primary hemostasis involves platelet adhesion, aggregation, and activation at the site of injury to form a temporary platelet plug. Secondary hemostasis builds upon this through the blood coagulation pathway, forming a stable fibrin clot around the platelet plug to finally stop bleeding and maintain blood volume homeostasis. The document outlines the specific steps and roles of platelets, platelet receptors, and secreted chemicals in achieving primary hemostasis through adhesion, aggregation, and activation at the site of vascular injury.
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This document summarizes hemostasis and evaluating bleeding patients. It describes the phases of hemostasis, clues from history and physical exam, laboratory evaluation, and treatment of common causes of coagulopathy. Key points include the phases of primary and secondary hemostasis, patterns of bleeding that suggest platelet or coagulation factor defects, tests of platelet function and coagulation factors, and treatment of liver disease, anticoagulation, DIC, von Willebrand disease, and hemophilia.
Anticoagulants & coagulation disorders
This document discusses anticoagulants and coagulation disorders. It defines anticoagulants as substances that prevent or delay blood clotting. It describes commonly used laboratory and clinical anticoagulants such as heparin, vitamin K antagonists, and aspirin. It also discusses coagulation disorders including defective clotting factors as seen in hemophilia, von Willebrand disease, and disseminated intravascular coagulation (DIC). The document provides details on the causes, symptoms, inheritance patterns, and treatments of various coagulation disorders.
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The document discusses management of intraoral bleeding during surgery. It covers hemostasis mechanisms, factors influencing surgical bleeding, adverse effects of bleeding, characteristics of ideal hemostatic agents, and various methods of hemostasis including mechanical, thermal, and chemical approaches. Common hemostatic agents discussed include adrenaline, bone wax, thrombin, gelfoam, and fibrin glue. Proper agent selection and application technique are important to control bleeding during oral surgery procedures.
Bleeding disorders Causes, Types, and Diagnosis
https://userupload.net/v3l4i8jsk7wq
Factor II, V, VII, X, or XII deficiencies are bleeding disorders related to blood clotting problems or abnormal bleeding problems. Von Willebrand's disease isthe most common inherited bleeding disorder. It develops when the blood lacks von Willebrand factor, which helps the blood to clot.
Haemostasis in surgery
Surgical hemostasis is one of the pillars of modern surgery. Adequate hemostasis in a surgical patient involves a detailed perioperative clinical evaluation and investigation, and various intra operative techniques and options. Ensuring adequate surgical hemostasis reduces morbidity and mortality by modulating the metabolic response to trauma, decreasing the incidence of post operative anemia, reduces rates of surgical site infection and ultimately improving wound healing
Bleeding and coagulation disorders hemostasis
1) Hemostasis is the process by which blood maintains its fluid state within vessels but also forms clots at sites of injury. It involves vasoconstriction, platelet plug formation, and coagulation cascade.
2) For blood not to clot normally in circulation, the endothelium prevents platelet adhesion, negatively charged particles repel clotting factors, circulation velocity must decrease for clotting, and natural anticoagulants and fibrinolysis are present.
3) Coagulation occurs through the intrinsic and extrinsic pathways activating prothrombin to thrombin which converts fibrinogen to fibrin threads forming a clot.
Hemostasis of blood
Hemostasis is the process by which bleeding is stopped. It involves platelets, clotting factors, and the endothelium. When a blood vessel is injured, the processes of vascular constriction, platelet plug formation, fibrin formation, and fibrinolysis work together to form a blood clot. The extrinsic and intrinsic pathways activate coagulation factors that generate thrombin, which converts fibrinogen to fibrin to form the clot. Counterregulatory mechanisms then limit clotting and allow for clot resorption and tissue repair.
Introduction to Haemostasis
This document provides an introduction to haemostasis by Dr. Ibrahim Khider. It defines haemostasis as the process of stopping blood flow following injury to a blood vessel. There are three components involved - the extravascular tissues, blood vessels, and intravascular platelets and plasma proteins. Haemostasis occurs through primary, secondary, and tertiary processes. Primary haemostasis involves platelets forming a platelet plug at the site of injury within 3-5 minutes. Secondary haemostasis reinforces this plug with a fibrin clot formed from plasma coagulation factors, taking 5-10 minutes. Tertiary haemostasis involves fibrinolysis to remove the clot as healing occurs over 48-72 hours.
Haemostasis and thrombosis ppt
This document discusses haemostasis (arrest of blood loss), thrombosis (pathological formation of blood clots), and factors that influence clotting. It also outlines different types of clots and emboli. Three main ways drugs can affect clotting are by impacting blood coagulation, platelet function, and fibrin removal. Specific drugs are highlighted that impact the coagulation cascade, platelet activation, and fibrinolytic system. These include heparins, warfarin as blood thinners, antiplatelet drugs like aspirin, and fibrinolytic drugs like streptokinase and tissue plasminogen activator. Their uses, mechanisms of action, properties and monitoring are described.
Hemostasis
Hemostasis is the process of preventing blood loss from broken blood vessels. It is achieved through three main mechanisms: vascular constriction, platelet plug formation, and blood coagulation. Vascular constriction occurs when smooth muscle contracts after vessel wall trauma. Platelets aggregate at the injury site to form a plug and provide clotting factors. Blood coagulation activates clotting factors through the extrinsic and intrinsic pathways, ultimately converting prothrombin to thrombin which converts fibrinogen to fibrin forming a clot. Clots are dissolved by plasmin which degrades fibrin, and several factors prevent excessive clotting.
Bleeding Disorders: Causes, Types, and Diagnosis
1) Bleeding disorders can involve vascular, platelet, or coagulation disorders and cause symptoms like bruising, bleeding, and prolonged bleeding from minor cuts.
2) Important bleeding disorders discussed include hemophilia A (factor VIII deficiency), hemophilia B (factor IX deficiency), von Willebrand disease (a disorder of the von Willebrand clotting factor), and fibrinogen deficiency.
3) These disorders are diagnosed through tests of bleeding time and clotting factor levels and activity. Treatment involves replacing the missing clotting factor through products like cryoprecipitate, desmopressin, or clotting factor concentrates.
Hemostasis by Dr.Rafi
Hemostasis is the process by which the body stops bleeding from damaged blood vessels through primary and secondary hemostasis. Primary hemostasis involves platelet adhesion and aggregation to form a platelet plug. Secondary hemostasis activates the coagulation cascade of clotting factors to form a fibrin clot over the platelet plug. A balance between coagulation and fibrinolysis maintains normal blood fluidity. Disorders can cause bleeding from inadequate clotting or thrombosis from excessive clotting.
Blood clotting
The document discusses blood clotting and the factors involved. Platelets and clotting factors work together to form blood clots which act as a mesh to stop bleeding from injuries. When a blood vessel is damaged, platelets stick to the injury site and clump together to form a platelet plug. Platelets and damaged tissue then release clotting factors which activate the blood clotting mechanism and produce fibrin to seal the wound. Impaired clotting can lead to excessive bleeding or blockages in arteries from blood clots.
Pathology - Hemostasis
This document summarizes hemostasis, the process by which bleeding is stopped. It discusses the three components of hemostasis - extravascular, vascular, and intravascular. The normal hemostasis process involves platelet plug formation and fibrin clot formation via the coagulation cascade. Coagulation factors, platelets, and fibrinogen are involved. Hemostasis is balanced by natural anticoagulants. Genetic or acquired bleeding disorders can result from deficiencies in specific coagulation factors or platelets. Common disorders discussed include hemophilia A/B/C and von Willebrand disease.
[Int. med] bleeding disorders from SIMS Lahore
This document discusses bleeding and clotting disorders. It provides details on:
1. The normal physiology of hemostasis including vascular, platelet and plasma factors that prevent bleeding.
2. Specific platelet and plasma factor deficiencies that can cause bleeding disorders.
3. The classification of bleeding disorders into defects of blood vessels, platelet disorders, and clotting factor deficiencies.
4. Diagnostic tests for evaluating bleeding patients including screening coagulation tests, specific factor assays, and platelet function tests.
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Hemophilia
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Hemophilia
Hemophilia is a genetic bleeding disorder caused by deficiencies in clotting factors VIII or IX. It is inherited in an X-linked recessive pattern and primarily affects males. Symptoms range from easy bruising to spontaneous internal bleeding that can be life-threatening. Treatment involves replacement of the missing clotting factor through infusions to prevent or treat bleeding episodes. Repeated bleeding can lead to chronic joint damage over time without proper management.
Hemophilia
Haemophilia is a bleeding disorder caused by a deficiency of coagulation factor VIII or IX. It is inherited in an X-linked recessive pattern, affecting mostly males. The two main types are haemophilia A (factor VIII deficiency) and haemophilia B (factor IX deficiency). Symptoms include prolonged bleeding from minor injuries or surgery. Joint bleeding is also common and can lead to joint damage. Treatment involves replacement of the missing clotting factor, either on-demand for bleeds or through prophylaxis to prevent bleeds. Other treatments include desmopressin or antifibrinolytics. Complications include the development of inhibitors against the replacement factors.
Presantation on bleeding disorder in pediatric patients
This document provides an outline and summary of a seminar presentation on bleeding disorders in pediatrics. The presentation covers an overview of homeostasis and blood clotting, approaches to evaluating a child with bleeding symptoms, relevant lab investigations, and different types of bleeding disorders seen in children including hereditary coagulation disorders like hemophilia A and von Willebrand disease, acquired coagulation disorders, platelet disorders, and vascular disorders. Specific conditions are described in detail including typical clinical features, inheritance patterns, laboratory findings, and treatment approaches.
Clotting Disorders.pptx
Hereditary and acquired coagulation disorders are caused by deficiencies in clotting factors that lead to defects in normal clot formation. Hereditary disorders include hemophilia A/classical hemophilia due to factor VIII deficiency and hemophilia B/Christmas disease due to factor IX deficiency. Von Willebrand's disease is the most common hereditary disorder characterized by qualitative or quantitative defects in von Willebrand factor. Acquired disorders involve deficiencies of multiple clotting factors and can be caused by vitamin K deficiency, liver disease, or disseminated intravascular coagulation. Coagulation disorders are diagnosed through prolonged coagulation tests and assays showing reduced clotting factor levels. Treatment focuses on replacing the
COAGULATION FACTORS AND DENTAL PROCEDURES
Coagulation factors are proteins in the blood that work together to help form blood clots. Some coagulation factor deficiencies can cause bleeding disorders like hemophilia. For dental procedures in patients with coagulation issues, replacement of deficient factors or use of antifibrinolytic drugs is often needed before, during, and after treatment to reduce bleeding risks. Local anesthetic techniques that minimize trauma are preferred. Proper factor level monitoring and hemostatic support tailored to the specific condition can allow for many routine dental treatments to be completed safely.
112/12/29--高雄地區第494次小兒科聯合病例討論會：淺談血友病.pdf
This document summarizes hemophilia, including its history, types, symptoms, diagnosis, and treatment. It discusses how hemophilia is an X-linked bleeding disorder caused by deficiencies in coagulation factors VIII or IX. The three main types are hemophilia A, B, and von Willebrand disease. Symptoms include bleeding in joints, muscles, and other tissues. Diagnosis involves screening for prolonged clotting times and factor level deficiencies. Treatment has progressed from plasma transfusions to recombinant factor replacements and newer therapies aiming to reduce bleeding risks and improve quality of life such as gene therapy and extended half-life factors. Comprehensive care requires a multidisciplinary approach addressing medical and psychosocial needs.
hemorrhage and shock in maxillofacial surgery.pptx
This is a presentation on hemorrhage and shock in oral and maxillofacial surgery
Hemophilia
Hemophilia A and B are X-linked bleeding disorders caused by deficiencies of coagulation factors VIII and IX respectively. Hemophilia A is more common, affecting about 1 in 5,000-10,000 live male births. The disorders are inherited but only affect males, with female carriers able to pass the gene to their sons or daughters. Common clinical manifestations include hemarthrosis, hematomas, and intracranial bleeding. Treatment involves replacement of the missing coagulation factor, initially through plasma-derived or recombinant products, with the goal of preventing bleeds or treating acute bleeds.
Hemorrhage
This document discusses hemorrhage in oral surgery, including:
- Defining hemorrhage as prolonged or uncontrolled bleeding.
- Local causes of hemorrhage from soft tissues or bone during oral surgery procedures.
- Systemic causes like bleeding disorders or anticoagulant use.
- Types of hemorrhage including primary, reactionary, and secondary bleeding.
- Management techniques for primary hemorrhage in normal patients including ligation of vessels, cautery, hemostatic agents, and hypotensive anesthesia.
Hemo
This document provides an overview of hematology, including the functions and components of blood, blood forming organs, red blood cells, white blood cells, platelets, blood disorders, and common blood tests. Key points include that blood transports oxygen, nutrients, hormones, and waste; is made up of plasma, red blood cells, white blood cells and platelets; and common disorders include anemia, leukemia, hemophilia, and disseminated intravascular coagulation. Laboratory tests discussed include complete blood count, coagulation studies, and differential white blood cell count.
Bleeding and clotting disorders in children
This document provides information on bleeding and coagulation disorders in children. It begins with definitions of bleeding disorders and then describes the phases of hemostasis. It discusses the etiology of bleeding disorders including vessel wall abnormalities like Ehlers-Danlos syndrome, platelet disorders like idiopathic thrombocytopenic purpura, and coagulation disorders such as hemophilia A. It provides details on specific disorders, their causes, clinical features, lab findings, and treatment options. The document concludes with approaches to evaluating a child presenting with a bleeding disorder.
HEMORRHAGIC DISEASES OF THE NEWBORN.pptx
This document discusses hemorrhagic diseases of the newborn, which are caused by vitamin K deficiency or other coagulation factor deficiencies. It defines hemorrhagic disease as an accentuation of the normal vitamin K deficiency in newborns. The key causes are lack of vitamin K in the mother, thrombocytopenia, lack of intestinal bacteria, and deficiencies in coagulation factors II, VII, IX and X. Symptoms include bleeding from the nose, umbilicus, brain or skin. Diagnosis involves tests of bleeding time, prothrombin time, partial thromboplastin time and INR. Treatment is intravenous vitamin K, and prevention is through vitamin K administration after birth.
Chapter 5-5 HEMORRHAGIC DISEASES OF THE NEWBORN.pptx
This document discusses hemorrhagic diseases of the newborn, which are caused by vitamin K deficiency or other coagulation factor deficiencies. It defines hemorrhagic disease as an accentuation of the normal vitamin K deficiency in newborns. The key causes are lack of vitamin K in the mother, thrombocytopenia, lack of intestinal bacteria, and deficiencies in coagulation factors II, VII, IX and X. Symptoms include bleeding from the nose, umbilicus, brain or skin. Diagnosis involves tests of bleeding time, prothrombin time, partial thromboplastin time and INR. Treatment is intravenous vitamin K, and prevention is through vitamin K administration after birth.
Hemophilia by Suhasis Mondal
This document discusses hemophilia, including:
1. Hemophilia is an inherited bleeding disorder caused by deficiencies in coagulation factor VIII or IX.
2. There are two main types - Hemophilia A caused by factor VIII deficiency and Hemophilia B caused by factor IX deficiency.
3. Treatment involves replacing the missing clotting factor through products derived from plasma or recombinant sources to control bleeding and prevent joint damage. Regular prophylactic treatment starting in early childhood has been shown to significantly reduce long-term joint complications.
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Presantation on bleeding disorder in pediatric patients
Presantation on bleeding disorder in pediatric patients
hemorrhage and shock in maxillofacial surgery.pptx
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Hemophilia the royal disease
- 5. Items to be discussed in this talk • What is hemophilia? • Types • Incidence • Genetics • Clinical presentation • Investigations • Management • Historical facts
- 6. What is Hemophilia? • A Hereditary bleeding disorder caused by deficiency of coagulation factor VIII, IX or XI. • X linked recessive Disorder
- 9. In Factor 8 or Factor 9 gene of chromosome 10
- 11. Signs and Symptoms of Hemophilia • Soft tissue hematomas • Hemarthroses • Muscle hemorrhage • CNS bleeding • Uncommon: epistaxis, other mucosal bleeding, prolonged bleeding from cuts
- 13. Pseudo Tumour Bleeding inside the soft tissues produces tumour like masses
- 14. • Internal Bleeding in joint spaces • Swelling • Pain during movements • Tenderness
- 16. TARGET JOINTS A particular Joint that has more than 3 bleeds within 6 months
- 17. Forearm Compartment Bleed •Volkman’s ischemic contracture•Compartment syndrome
- 19. Bleeding inside the cranial cavity
- 20. • PURPURAE • PETECHIAE • ECHYMOSES
- 21. Iatrogenic Complications in hemophilia •Circumcision •i.m. injection •i.j. stick
- 22. Clinical presentation Severity Factor VIII level Presentation Mild 5-30% of normal (0.05-0.25 IU/ml) -Severe bleeding after major trauma or surgery -Spontaneous bleeding is rare -May be asymptomatic Moderate 1-5% of normal (0.01-0.05 IU/ml) -Prolonged bleeding after minor trauma or surgery -Occasional spontaneous bleeding Severe <1% of normal (<0.01 IU/ml) -Spontaneous bleeding into joints or muscles, without identifiable hemostatic challenge -May be serious & life threatening
- 23. Investigations • Platelet count: Normal • Bleeding time: Normal • PT: Normal • Clotting time & PTT: Prolonged • Factor VIII or Factor IX assay: Decreased
- 26. Management • Prevention of bleeding episodes. • Replacement therapy. • Prophylactic therapy. • Other lines of therapy. • Management of complications.
- 27. PREVENTION Control Bleeding Episodes • Local measures: apply direct pressure; elevate or ice compress • Epistaxsis sit up lean forward
- 28. PREVENTION Prevent joint degeneration • Immobilize joint during acute bleeding • Progressive exercise • Avoid prolong immobility
- 29. Avoid IM injections Avoid contact sports
- 31. 2. Replacement therapy • Fresh whole blood • Whole plasma • Fresh Frozen Plasma • Cryoprecipitate • Factor VIII or IX Concentrate • Recombinant Factor VII (Novo-Seven): to bypass factor VIII in the coagulation pathway
- 32. After the time of World war II Plasma infusion
- 33. After 1960 - cryoprecipitate of plasma
- 34. Cryoprecipitate • Contains only – Fibrinogen – Factor VIII or IX and vWD factor – Factor XIII • Cryoprecipitate packs pooled from 5 donors • Short shelf life after thawing – 4 hours
- 35. After 1970- Home infusion
- 36. After 1990 - Recombinant Factor 8 or 9 infusion
- 37. Factor VIII or IX Concentrate • Factor VIII or IX Concentrate: 20 U/kg increases the level by ± 50%. Clinical Situation Raise factor VIII up to Mild bleeding 20-40% Severe or life threatening bleeding 100% Dose required = Target dose – Baseline level x Body weight x 0.5/1
- 38. • Factor 8 infusion twice daily x 3days a week • Factor 9 infusion thrice daily x 3days a week
- 42. 3. Prophylactic therapy • Indication: Before surgery After exposure to trauma • Maintain 100% of factor concentration in blood prior to surgery
- 43. 4. Other lines of therapy • Desmopressin: - Action: stimulates the release of stored factor VIII and von Willebrand factor. von Willebrand factor carries and binds factor VIII, which then can stay in the blood stream longer. -Administration: Injection or Nasal spray.
- 45. 4. Other lines of therapy • EACA (e –amino caproic acid): - Action: Antifibrinolytic delays clot lysis • -Use: Adjuvant therapy for dental procedures • Fibrin Glue: - Action: Contains fibrinogen, thrombin and factor XIII. Placed in the site of injury to stabilize clot. -Use: Dental procedures and after circumcision
- 49. Physiotherapy Start exercise once pain subsides Early restoration of Full range of motion Strength Proprioception, balance and coordination
- 51. At last we all know……. • What is hemophilia? • Types • Incidence • Genetics • Clinical presentation • Investigations • Management • Historical facts
Editor's Notes
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