Acute chest syndrome

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Background

  • A leading cause of hospitalization and death in adults with sickle cell disease
  • Occurs most commonly in the 2-4yr old age group and then declines with age
  • Due to pulmonary ischemia and infarction; complication of pneumonia

Causes

Clinical Features

Differential Diagnosis

Sickle cell crisis

Evaluation

Work-Up

  • CBC
  • Retic count
  • VBG / ABG
  • Blood culture /sputum cultures
  • CXR

Evaluation

  • New infiltrate on CXR with at least one of the following:[1]
    • Fever >38.5
    • Cough
    • Wheezing
    • Tachypnea
    • New hypoxemia
    • Chest pain
  • Note: CXR findings may lag behind the clinical features
  • Lung ultrasound to CXR or CT finding correlations[2]
    • Consolidation seen as hyperechoic punctiform air bronchograms
    • Ground-glass opacities seen as coalescent B lines
    • Pleural effusion, defined as large if interpleural distance > 25 mm

Management

  1. O2
    • Titrate to pulse oximetry >92%
  2. Incentive Spirometer
  3. Hydration
    • Oral hydration preferred
    • IV hydration with hypotonic fluid if patient unable to tolerate PO
  4. Analgesia
    • Pulmonary toilet is important but avoid excessive sedation
  5. Bronchodilators
  6. Antibiotics
  7. Transfusion (leucocyte depleted)
    • Consider transfusion to goal of hemoglobin 11 / hematocrit 30 for:
      • O2 Sat <92% on room air
      • hematocrit 10-20% below patient's usual hematocrit or dropping hematocrit
  8. Exchange transfusion
    • Consider for:
      • Progression of acute chest syndrome despite simple transfusion
      • Severe hypoxemia
      • Multi-lobar disease
      • Previous history of severe acute chest syndrome or cardiopulmonary disease

Complications

See Also

Video

References

  1. Novelli EM, Gladwin MT. Crises in Sickle Cell Disease. Chest. 2016; 149 (4): 1082-1093.
  2. Razazi et al. Bedside Lung Ultrasound During Acute Chest Syndrome in Sickle Cell Disease. Medicine (Baltimore). 2016 Feb; 95(7): e2553.