Acute chest syndrome (ACS) is not just precipitated by infection
Acute chest syndrome (ACS) is not just precipitated by infection: Excerpt from Avoiding Common Pediatric Errors
Author:
Sarika Joshi, MD
What to Do - Gather Appropriate Data
There is a need to treat the multiple causes of ACS.
ACS is a common complication and reason for hospital admission in
children with sickle cell disease (SCD). It is also the most common cause
of death in this patient population. ACS is defined as a new pulmonary
infiltrate, involving at least one complete segment, on chest radiograph and
at least one of the following signs or symptoms: (a) chest pain; (b) fever; (c) increased work of breathing (i.e., use of accessory muscles, nasal flaring),
tachypnea, cough, or wheeze; or (d) hypoxia.
ACS is the end result of a variety of processes that result in deoxygenationofhemoglobinSandsicklingofredbloodcells,leadingtovaso-occlusion,
local ischemia, and vascular damage. Most of the time, the trigger for ACS
in an individual patient cannot be identified. However, although infection
is the most common identifiable cause for ACS, other important triggers
are vaso-occlusive crisis (VOC) and asthma. In fact, many patients who
develop ACS have been hospitalized for a different reason, often VOC. Appropriate treatment for ACS targets these multiple etiologies, with the goal
of improving oxygenation and, therefore, a reduction in sickling and lung
damage.
Initial management of ACS includes antibiotics, fluids and analgesia,
and respiratory support, often including bronchodilators. Transfusions are
also a mainstay of therapy. Viruses (i.e., respiratory syncytial virus), bacteria (i.e., encapsulated organisms), Mycoplasma and Chlamydia are common
infectious agents in acute chest syndrome. Typically, patients are treated
empirically with a combinationof broad-spectrumantibiotics, such as ceftriaxone (a third-generation cephalosporin) and azithromycin (a macrolide). In
more severe casesof ACS, vancomycin is addedto covermethicillin-resistant
Staphylococcus aureus and penicillin-resistant Streptococcal pneumoniae.
Generally, fluids are administered, as dehydration increases the likelihood of sickling. Adequate analgesia for VOC, especially of the back, chest,
and abdomen, prevents splinting and hypoventilation due to pain. However,
oversedation from opioids can also result in hypoventilation. Hypoventilation leads to atelectasis and a mismatch of ventilation and perfusion, further
exacerbating sickling and lung damage. Incentive spirometry should be encouraged to help prevent atelectasis.
Basic respiratory support involves oxygen supplementation to maintain
an arterial oxygen saturation >92%. For patients with increasing oxygen
requirements, or those who are unable to maintain an adequate respiratory
effort, the use of positive pressure ventilation should be considered. Asthma
is more common is children with SCD, and children with SCD and asthma
may be at increased risk for ACS. Patients with ACS and a history of asthma
should receive scheduled bronchodilator therapy, irrespective of exam findings. Bronchodilator therapy should also be considered in patients with ACS
and no history of asthma.
The goal of simple transfusion in ACS is to increase hemoglobin to
11 g/dL or hematocrit to 30%, thereby improving oxygenation. Some indications include anemia (i.e., hematocrit 10%–20% below the patient's baseline), PaO2<60 mm Hg on arterial blood gas, and disease progression. In
severe cases of ACS, partial exchange transfusion may be used. In summary,
common precipitants of ACS include not only infection, but also VOC and
asthma. Treatment for ACS needs to address these multiple etiologies, and
acutetherapyincludesantibiotics,fluids,analgesia, oxygen,bronchodilators,
and transfusion.
Suggested Readings
Boyd JH, Moinuddin A, Strunk RC, et al. Asthma and acute chest in sickle-cell disease. Pediatr
Pulmonol. 2004;38:229–232.
Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome
in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;
342:1855–1865.
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Book Source Details
- Book Title: Avoiding Common Pediatric Errors
- Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
- Year of Publication: 2008
- Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Avoiding Common Pediatric Errors
Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7489-6
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