Acute chest syndrome: Difference between revisions

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


===Causes===
===Causes===
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**[[C. pneumoniae]] and [[mycoplasma]] are most common organisms
**[[C. pneumoniae]] and [[mycoplasma]] are most common organisms
**May also be caused by viruses, [[H. flu]], [[klebsiella]], [[staph]]
**May also be caused by viruses, [[H. flu]], [[klebsiella]], [[staph]]
*[[Fat emboli]]
*[[Fat embolism|Fat emboli]]
**Lodge in pulmonary vasculature → additional ischemia
**Lodge in pulmonary vasculature → additional ischemia
*Rib infarction
*Rib infarction
*Overly aggressive IV hydration
*Overly aggressive [[IVF|IV hydration]]
*[[Vaso-occlusive pain crisis]]
*[[Vaso-occlusive pain crisis]]
*[[Asthma]]
*[[Asthma]]
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*[[Tachypnea]]
*[[Tachypnea]]
*[[Chest pain]]
*[[Chest pain]]
==Complications==
*[[Pulmonary Embolism]] (bone marrow, fat or thrombotic)
*[[Pneumonia]]
*[[CVA]]
*[[Sepsis]]


==Differential Diagnosis==
==Differential Diagnosis==
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*CBC
*CBC
*Retic count
*Retic count
*VBG / ABG
*[[VBG]] / [[ABG]]
*Blood culture /sputum cultures
*Blood culture /sputum cultures
*[[CXR]]
*[[CXR]]


===Evaluation===
===Evaluation===
*New infiltrate on CXR with at least one of the following:<ref>Novelli EM, Gladwin MT.  Crises in Sickle Cell Disease.  Chest.  2016; 149 (4): 1082-1093.</ref>
*New infiltrate on [[CXR]] with at least one of the following:<ref>Novelli EM, Gladwin MT.  Crises in Sickle Cell Disease.  Chest.  2016; 149 (4): 1082-1093.</ref>
**Fever >38.5
**[[Fever]] >38.5
**Cough
**[[Cough]]
**Wheezing
**[[Wheezing]]
**Tachypnea
**[[Tachypnea]]
**New hypoxemia  
**New [[hypoxemia]]
**[[Chest pain]]
**[[Chest pain]]
*Note: CXR findings may lag behind the clinical features
*Note: CXR findings may lag behind the clinical features
*Lung ultrasound to CXR or CT finding correlations<ref>Razazi et al. Bedside Lung Ultrasound During Acute Chest Syndrome in Sickle Cell Disease. Medicine (Baltimore). 2016 Feb; 95(7): e2553.</ref>
*[[Lung ultrasound]] to CXR or CT finding correlations<ref>Razazi et al. Bedside Lung Ultrasound During Acute Chest Syndrome in Sickle Cell Disease. Medicine (Baltimore). 2016 Feb; 95(7): e2553.</ref>
**Consolidation seen as hyperechoic punctiform air bronchograms
**Consolidation seen as hyperechoic punctiform air bronchograms
**Ground-glass opacities seen as coalescent B lines
**Ground-glass opacities seen as coalescent B lines
**Pleural effusion, defined as large if interpleural distance > 25 mm
**[[Pleural effusion]], defined as large if interpleural distance > 25 mm


==Management==
==Management==
#O2
#[[O2]]
#*Titrate to pulse oximetry >92%
#*Titrate to pulse oximetry >92%
#Incentive Spirometer
#Incentive Spirometer
#Hydration
#Hydration
#*Oral hydration preferred
#*[[oral rehydration therapy|Oral hydration]] preferred
#*[[IV hydration]] with hypotonic fluid if patient unable to tolerate PO
#*[[IV hydration]] with hypotonic fluid if patient unable to tolerate PO
#[[Analgesia]]
#[[Analgesia]]
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#*3rd generation [[cephalosporin]] + [[macrolide]]
#*3rd generation [[cephalosporin]] + [[macrolide]]
#[[Transfusion]] (leucocyte depleted)
#[[Transfusion]] (leucocyte depleted)
#*Consider transfusion to goal of hemoglobin 11 / hematocrit 30 for:
#*Consider [[pRBCs|transfusion]] to goal of hemoglobin 11 / hematocrit 30 for:
#**O2 Sat <92% on room air
#**O2 Sat <92% on room air
#**hematocrit 10-20% below patient's usual hematocrit or dropping hematocrit
#**hematocrit 10-20% below patient's usual hematocrit or dropping hematocrit
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#**Multi-lobar disease
#**Multi-lobar disease
#**Previous history of severe acute chest syndrome or cardiopulmonary disease
#**Previous history of severe acute chest syndrome or cardiopulmonary disease
==Complications==
*[[Pulmonary Embolism]] (bone marrow, fat or thrombotic)
*[[Pneumonia]]
*[[CVA]]
*[[Sepsis]]


==See Also==
==See Also==

Revision as of 23:13, 30 September 2019

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

Complications

Differential Diagnosis

Sickle cell crisis

Evaluation

Work-Up

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

Evaluation

Management

  1. O2
    • Titrate to pulse oximetry >92%
  2. Incentive Spirometer
  3. Hydration
  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

See Also

Video

{{#widget:YouTube|id=pKxAsqj4GOE}}

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.