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 | ||
==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
- 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
Complications
- Pulmonary Embolism (bone marrow, fat or thrombotic)
- Pneumonia
- CVA
- Sepsis
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
Evaluation
- New infiltrate on CXR with at least one of the following:[1]
- 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
- 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:
See Also
Video
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