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Sickle-Cell Disease Anemia

Sickle-Cell Disease Anemia. This is a severe hemophilic anemia resulting from homogenous inheritance of a gene which causes an amino-acid substitution in the hemoglobin molecule (6 Glu Val) So creating Hbs. It is common in black race. Classification.

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Sickle-Cell Disease Anemia

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  1. Sickle-Cell Disease Anemia

  2. This is a severe hemophilic anemia resulting from homogenous inheritance of a gene which causes an amino-acid substitution in the hemoglobin molecule (6 Glu Val) • So creating Hbs. It is common in black race.

  3. Classification • Homozygote (SS), has sickle cell anemia • Heterozygote (AS), has sickle cell trait • Heterozygote SC • Heterozygote SD • Homozygote CC • Homozygote DD

  4. Diagnosis • Sickling Test • Electrophoresis • Blood film

  5. Clinical Features *Anemia *Jaundice *Painful swelling of hands and feet: hand and foot syndrome *Enlarged spleen *Priapism

  6. Complications • Renal Failure • Bone necrosis • Infections e.g. Salmonella Osteomyelitis. • Leg Ulcers

  7. Sickle-cell Crisis Painful Crisis Thrombosis Marrow aplasia or sequestration Hemolysis

  8. Management • Analgesic • Cross match blood, FBC, blood culture, MSU, CXR • Dehydration • Antibiotics • Anti-malaria • +- Blood transfusion

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