A life of a thalassemia patient 1e

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Slide สอนแพทย์ประจำบ้านอายุรศาสตร์รามาธิบดี 23 ตค 56

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A life of a thalassemia patient 1e

  1. 1. A life of a thalassemia patient
  2. 2. • A one-year-old child presented to you with failure to thrive, poor performance. • PE revealed that she is pale and icteric. • Diagnosis of thalassemia • Epigenetics
  3. 3. beta A delta A2 alpha
  4. 4. beta thalassemia beta A gamma F delta A2 alpha
  5. 5. alpha thalassemia alpha beta A delta A2
  6. 6. Pathophysiology of thalassemia • Decrease globin synthesis • Excess globin chain • Ineffective erythropoiesis • Hemolysis
  7. 7. • She is diagnosed with thalassemia. • Hb typing : E 77% F 23% • Hemoglobinopathy and thalassemia
  8. 8. Hemoglobin E beta alpha beta E E A beta E E alpha
  9. 9. beta thalassemia/ Hemoglobin E beta beta E alpha F E
  10. 10. • Another boy in the thalassemia clinic has different profile. He is sick and pale only whe he has fever. • Difference in severity of thalassemia’s • Benefits of splenectomy
  11. 11. • The girl’s parents ask you if they can have another child. • Possibility of having another thalassemia baby.
  12. 12. • She is transfused 3-4 times a year due to her anemic symptoms. • Types of transfusion program • Risks during transfusion
  13. 13. • The parents decided that they will not have more babies, and asked for your opinion about stem cell transplantation. • Risk classification in stem cell transplantation • Stem cell donor sources • Cost effectiveness of stem cell transplantation in thalassemia
  14. 14. • Stem cell donor cannot be found. • She is put on hypertransfusion program. • Risk and sequale of iron overload
  15. 15. Iron overload • When to consider • How to diagnose • How to treat • How to give advice
  16. 16. Comparison of iron chelators Deferoxamine Deferiprone Deferasirox Oral route    Half life       Toxicities Intracellular chelation
  17. 17. • She did not received stem cell transplantation. At the age of 20, she began to experience recurrent pain in her RUQ of abdomen. • Others life events
  18. 18. Other complications from thalassemia • Gallstone • Venous thromboembolism and hypercoagulable state • Transfusion complication – Post transfusion hypertension – Transfusion reaction
  19. 19. • http://goo.gl/kGkf1B • http://www.slideshare.net/uartit/a-life-of-athalassemia-patient-1

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