How I treat acute chest syndrome in children with sickle cell disease.

@article{Miller2011HowIT,
  title={How I treat acute chest syndrome in children with sickle cell disease.},
  author={Scott T. Miller},
  journal={Blood},
  year={2011},
  volume={117 20},
  pages={
          5297-305
        }
}
Acute chest syndrome describes new respiratory symptoms and findings, often severe and progressive, in a child with sickle cell disease and a new pulmonary infiltrate. It may be community-acquired or arise in children hospitalized for pain or other complications. Recognized etiologies include infection, most commonly with atypical bacteria, and pulmonary fat embolism (PFE); the cause is often obscure and may be multifactorial. Initiation of therapy should be based on clinical findings… Expand
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It is found that Acute chest syndrome (ACS) was more common in the sickle cell disease genotypes SS and S/beta-thalassemia-null, and that ACS was more frequent in patients treated for asthma. Expand
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References

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Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Course
TLDR
In children, ACS was milder and more likely due to infection, whereas in adults ACS was severe, associated with pain and had a higher mortality rate, which was four times higher in adults than in children. Expand
Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease
TLDR
A treatment protocol that included matched transfusions, bronchodilators, and bronchoscopy was evaluated and it was found that infection and fat embolism are underdiagnosed in patients with the syndrome. Expand
"Acute chest syndrome" in adults with sickle cell anemia. Microbiology, treatment, and prevention.
TLDR
Pneumococcal polysaccharide vaccine has great potential for preventing life-threatening infection in children with sickle cell anemia, but may not change the incidence or severity of the acute chest syndrome in adults. Expand
Inhaled nitric oxide for acute chest syndrome in people with sickle cell disease.
TLDR
To assess the effectiveness of inhaled nitric oxide for treating acute chest syndrome by comparing improvement in symptoms and clinical outcomes against standard care, and to assess the benefits and risks of this form of treatment as an adjunct to established therapies. Expand
Acute chest syndrome.
TLDR
Current information for acute chest syndrome, its definition, frequency, pathogenesis, clinical features, complications, investigations, management and prevention, and recent advances in management are reviewed. Expand
Antibiotics for treating acute chest syndrome in people with sickle cell disease.
TLDR
No randomised controlled trials on efficacy and safety of the antibiotic treatment approaches for people with sickle cell disease suffering from acute chest syndrome were identified. Expand
Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease.
TLDR
Intravenous dexamethasone has a beneficial effect in children with SCD hospitalized with mild to moderately severe acute chest syndrome and gender and previous episodes of ACS were the only variables that appeared to predict response to dexamETHasone, as measured by lengh of hospital stay. Expand
Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia.
TLDR
Results indicate that when PFE is associated with ACS, it is characterized by a distinct clinical course, and that bronchial lavage is a safe and useful test in diagnosing PFE in patients with ACS. Expand
Beneficial effect of blood transfusion in children with sickle cell chest syndrome.
TLDR
The low hemoglobin value at presentation in patients makes dilution of sickle cells possible by packed red blood cell transfusion rather than exchange transfusion, and it is concluded that early blood transfusion may be valuable in shortening the course and decreasing mortality. Expand
Acute chest syndrome in sickle cell disease: etiology and clinical correlates.
TLDR
Patients with bacterial pneumonia were sicker, as shown by fever and hospitalization of longer duration, the percent of those requiring red blood cell transfusion, and the presence of pleural effusions, while the lower incidence of bacterial pneumonias among the authors' patients compared with that previously reported may reflect the use of penicillin prophylaxis and pneumococcal immunization to prevent S. pneumoniae infections. Expand
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