Acute chest syndrome
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
- Pulmonary infection
- C. pneumoniae and mycoplasma are most common organisms
- May also be caused by viruses, H. flu, klebsiella, staph
- Fat emboli
- Lodge in pulmonary vasculature → additional ischemia
- Rib infarction
- Overly aggressive IV hydration
- Vaso-occlusive pain crisis
- Asthma
Clinical Features
- Fever >38.5
- Cough
- Wheezing
- Tachypnea
- Chest pain
Differential Diagnosis
Sickle cell crisis
- Vaso-occlusive pain crisis
- Bony infarction
- Dactylitis
- Avascular necrosis of femoral head
- Acute chest syndrome
- Asthma
- Pulmonary hypertension
- Gallbladder disease
- Acute hepatic sequestration
- Infection
- Parvovirus B19
- Splenic sequestration
- CVA
- Cerebral aneurysm and ICH
- Priapism
- Papillary necrosis
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:
- Fever >38.5
- Cough
- Wheezing
- Tachypnea
- Chest pain
- Note: CXR findings may lag behind the clinical features
Management
- O2
- Titrate to pulse oximetry >92%
- Incentive Spirometer
- Hydration
- Oral hydration preferred
- IV hydration with hypotonic fluid if patient unable to tolerate PO
- Analgesia
- Pulmonary toilet is important but avoid excessive sedation
- Bronchodilators
- Antibiotics
- 3rd generation cephalosporin + macrolide
- 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
- Consider transfusion to goal of hemoglobin 11 / hematocrit 30 for:
- 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
- Consider for:
Complications
- Pulmonary Embolism (bone marrow, fat or thrombotic)
- Pneumonia
- CVA
- Sepsis
See Also
Video
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