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Hemophilia ppt lakshmi

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LAKSHMIHANSHITA

Hemophilia is an inherited bleeding disorder caused by deficient clotting factors VIII or IX. It is primarily found in males but can be transmitted by female carriers. Hemophilia A is the most common type, accounting for 80-85% of cases and is caused by factor VIII deficiency. Hemophilia B or Christmas disease accounts for 15-20% of cases and results from factor IX deficiency. Diagnosis involves prolonged aPTT and bleeding times as well as low factor VIII or IX levels. Treatment focuses on replacing the missing clotting factor through infusions while also managing pain and preventing injuries.

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HEMOPHILIA (Royal Disease) PRESENTED BY: MRS. M.LASKHMI, M.SC (N) OBSTETRIC & GYNECOLOGICAL NURSING
Hemophilia ppt lakshmi
INTRODUCTION Hemophilia is an inherited bleeding disorder due to deficiency of plasma coagulation factors. People with hemophilia have low levels of either factor VIII (8) or factor IX (9).
INCIDENCE It is primarily found in males but transmitted by female carriers. Hereditary hemophilia account about 80% of cases as sex linked chromosomes About 20% of cases may account as acquired hemophilia due to autoimmune disorder/ spontaneous mutation
History of hemophilia Hemophilia is sometimes referred to as the royal disease, because it affected the royal families of England, Germany, Russia and Spain in the 19th and 20th centuries. Queen Victoria of England, is believed to have been the carrier of hemophilia B, or factor IX deficiency. She passed the trait on to three of her nine children. Her son Leopold died of a hemorrhage after a fall when he was 30. Her daughters Alice and Beatrice passed it on to several of their children. Hemophilia was carried through various royal family members for three generations after Victoria, then disappeared.
Hemophilia ppt lakshmi
CLASSIFICATION The following two are the common: Hemophilia A (Classic Hemophilia) This type is caused by a lack or decrease of clotting factor VIII, the antihemophilic factor (AHF). It accounts for 80-85% of all cases.1in 5000-10,000 male births. Hemophilia B (Christmas Disease) This type is caused by a lack or decrease of clotting factor IX, the plasma thromboplastin component (PTC). 15-20% cases.1 in 20,000- 34,000 male births.
CLASSIFICATION Less common Hemophilia C This type is caused by a lack or decrease of factor XI, plasma thromboplastin antecedent (PTA). Hagemans disease: deficiency of factor XII Von Willebrands disease: reduced level of Von Willebrands factor, a protein that binds with factor VIII & protects it from rapid breakdown within blood.
Hemophilia classification Classified based upon the factor VIII & IX level in plasma.
CAUSES Hemophilia is caused by a mutation or change, in one of the genes, that provides instructions for making the clotting factor proteins needed to form a blood clot. This change or mutation can prevent the clotting protein from working properly or to be missing altogether. These genes are located on the X chromosome.
Causes of acquired hemophilia
Pathophysiology
Hemophilia ppt lakshmi
CLINICAL MANIFESTATIONS
HEMORRHAGE JOINTS (HEMARTHROSIS)
Hemophilia ppt lakshmi
Diagnostic evaluation
Diagnostic evaluation A typical aPTT value is 30 to 40 seconds.- prolonged Bleeding time is between 2-7 minutes- normal clotting time in a person is between 8-15 minutes.-prolonged Normal ranges for factor VIII levels are 50% to 150%. If your factor VIII activity level is less than 50%, you may have hemohilia A. The reference range for factor IX is between 60% and 140% of normal values.
MANAGEMNET Replacement of missing coagulation factors Factor VIII made from cryoprecipitate- Hemophilia A Factor IX & XI made from Fresh frozen plasma. Fresh whole blood transfusion can be given if the commercially prepared factors are not available.
MANAGEMNET Mild to moderate hemophilia- Desmopressin (Release of factor VIII from the endothelial stores)
MANAGEMNET Antifibrinolytics Aminocaproic acid & tranexamic acid are given for mucosal bleeding
MANAGEMNET Supportive management for hemarthrosis
Cont Pain- Analgesic like paracetamol or NSAIDs. Avoid aspirin, indomethacin & betazolidone as they inhibit platelets function. Synovectomy can be recommended to remove damage synovium.
MANAGEMNET Orthotics can be used to prevent injury
MANAGEMNET Gene therapy is now under research Genetic counselling & antenatal diagnosis should be arranged
COMPLICATIONS Airway obstruction due to bleeding into the neck & pharynx Intracranial bleeding Intestinal obstruction Compartment syndrome Degenerative joint disease Chronic hepatitis Risk of HIV/AIDS
Prevent complications Prevention of injury Avoidance of IM injection Immunization against hepatitis B Providing emotional support Precautions should be taken before any surgery.
Once you choose hope, anything is possible -Christopher Reeve Thank you

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Hemophilia ppt lakshmi

  • 1. HEMOPHILIA (Royal Disease) PRESENTED BY: MRS. M.LASKHMI, M.SC (N) OBSTETRIC & GYNECOLOGICAL NURSING
  • 3. INTRODUCTION Hemophilia is an inherited bleeding disorder due to deficiency of plasma coagulation factors. People with hemophilia have low levels of either factor VIII (8) or factor IX (9).
  • 4. INCIDENCE It is primarily found in males but transmitted by female carriers. Hereditary hemophilia account about 80% of cases as sex linked chromosomes About 20% of cases may account as acquired hemophilia due to autoimmune disorder/ spontaneous mutation
  • 5. History of hemophilia Hemophilia is sometimes referred to as the royal disease, because it affected the royal families of England, Germany, Russia and Spain in the 19th and 20th centuries. Queen Victoria of England, is believed to have been the carrier of hemophilia B, or factor IX deficiency. She passed the trait on to three of her nine children. Her son Leopold died of a hemorrhage after a fall when he was 30. Her daughters Alice and Beatrice passed it on to several of their children. Hemophilia was carried through various royal family members for three generations after Victoria, then disappeared.
  • 7. CLASSIFICATION The following two are the common: Hemophilia A (Classic Hemophilia) This type is caused by a lack or decrease of clotting factor VIII, the antihemophilic factor (AHF). It accounts for 80-85% of all cases.1in 5000-10,000 male births. Hemophilia B (Christmas Disease) This type is caused by a lack or decrease of clotting factor IX, the plasma thromboplastin component (PTC). 15-20% cases.1 in 20,000- 34,000 male births.
  • 8. CLASSIFICATION Less common Hemophilia C This type is caused by a lack or decrease of factor XI, plasma thromboplastin antecedent (PTA). Hagemans disease: deficiency of factor XII Von Willebrands disease: reduced level of Von Willebrands factor, a protein that binds with factor VIII & protects it from rapid breakdown within blood.
  • 9. Hemophilia classification Classified based upon the factor VIII & IX level in plasma.
  • 10. CAUSES Hemophilia is caused by a mutation or change, in one of the genes, that provides instructions for making the clotting factor proteins needed to form a blood clot. This change or mutation can prevent the clotting protein from working properly or to be missing altogether. These genes are located on the X chromosome.
  • 18. Diagnostic evaluation A typical aPTT value is 30 to 40 seconds.- prolonged Bleeding time is between 2-7 minutes- normal clotting time in a person is between 8-15 minutes.-prolonged Normal ranges for factor VIII levels are 50% to 150%. If your factor VIII activity level is less than 50%, you may have hemohilia A. The reference range for factor IX is between 60% and 140% of normal values.
  • 19. MANAGEMNET Replacement of missing coagulation factors Factor VIII made from cryoprecipitate- Hemophilia A Factor IX & XI made from Fresh frozen plasma. Fresh whole blood transfusion can be given if the commercially prepared factors are not available.
  • 20. MANAGEMNET Mild to moderate hemophilia- Desmopressin (Release of factor VIII from the endothelial stores)
  • 21. MANAGEMNET Antifibrinolytics Aminocaproic acid & tranexamic acid are given for mucosal bleeding
  • 23. Cont Pain- Analgesic like paracetamol or NSAIDs. Avoid aspirin, indomethacin & betazolidone as they inhibit platelets function. Synovectomy can be recommended to remove damage synovium.
  • 24. MANAGEMNET Orthotics can be used to prevent injury
  • 25. MANAGEMNET Gene therapy is now under research Genetic counselling & antenatal diagnosis should be arranged
  • 26. COMPLICATIONS Airway obstruction due to bleeding into the neck & pharynx Intracranial bleeding Intestinal obstruction Compartment syndrome Degenerative joint disease Chronic hepatitis Risk of HIV/AIDS
  • 27. Prevent complications Prevention of injury Avoidance of IM injection Immunization against hepatitis B Providing emotional support Precautions should be taken before any surgery.
  • 28. Once you choose hope, anything is possible -Christopher Reeve Thank you