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  • Occurs due to single genetic mutation with single amino acid substitution in the Hb molecule

  • Common in African, Indian, Middle Eastern children

 

CLINICAL FEATURES

 

Sickle cell trait

  • Causes disability in severe hypoxia

  • Children have chronic anemia (Hb- 8- 10 g/dL)

  • There is predisposition to infection- Staphylococcus, Pneumococcus and Haemophilus

 

Sickle cell crisis

  • Generally occurs following infection, cold, dehydration or hypoxia

    This causes

  • Thrombosis, hemolysis, marrow aplasia or acute splenic/ Liver sequestration

  • Any acute medical or surgical emergency may be mimicked

  • Can cause severe aching bony pain and low-grade fever, Cerebral sickling can occur with psychosis, fits, strokes or focal deficits.

 

Acute chest syndrome

  • Leading cause of death in sickle cell anaemia

    Presents as

  • Chest pain, hypoxia, pulmonary infiltrates, cough, tachypnea, wheezing

 

Acute splenic sequestration

  • Occurs due to sudden trapping of large numbers of RBS in the spleen causing severe anaemia

  • Presents as spleen enlargement, hypovolemia and thrombocytopenia

  • There is 30% chance if sequestration happening in children by 5 years of age

 

Osteomyelitis and septic arthritis

  • Presents with fever, soft tissue swelling.

  • Salmonella is a common organism

 

INVESTIGATIONS

  • Sickling test 

  • Hb Electrophoresis

  • Full blood count/ Peripheral smear – shows anaemia

  • Infections markers- CRP/ Cultures

  • Arrange CT head if neurological symptoms

 

MANAGEMENT

  • Supportive treatment- Oxygen, rest

  • Opioids( intravenous) are required to control pain

  • Rehydration with Iv fluids

  • Transfusion may be required if severe anaemia or CNS or Lung complications

  • Empirical antibiotic therapy 

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