Sickle cell disease and related hemoglobinopathies.

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چکیده

Objectives 1. Describe different combinations of normal and abnormal hemoglobins and their phenotypic expression 2. Describe the pathophysiology of vasooclusion secondary to hemoglobinopathies 3. Define and describe hand-foot syndrome 4. Describe features of osteomyelitis secondary to sickle cell disease and its differentiation from bone infarct 5. Describe features of avascular necrosis of bone associated with sickle cell disease 6. Discuss the complication rate of orthopaedic surgery for problems secondary to sickle cell disease 7. Describe the major non-orthopaedic problems associated with sickle cell disease

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Sickle cell disease and other hemoglobinopathies.

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Sickle cell disease is the most common type of hemoglobinopathies in the world that is caused by abnormal beta globin chain in hemoglobin. The disease is usually diagnosed in the first decade of life. Bone involvement is one of the most common clinical manifestations both in the acute setting (painful vaso-occlusive crises), and/or as a source of chronic disability (such as avascular necrosis)....

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عنوان ژورنال:
  • JAMA

دوره 224 5 Suppl  شماره 

صفحات  -

تاریخ انتشار 1973