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… 

Figures from this paper

Acute Chest Syndrome in Children with Sickle Cell Disease: Current Perspectives on Pathogenesis and Treatment
TLDR
Although the efficacy of several modalities in attenuating or preventing ACS are well established, the outcomes from instituting others are not convincing and more research is, therefore, needed to strengthen the evidence for their therapeutic efficacy.
Acute Chest Syndrome in Children with Sickle Cell Disease
TLDR
A review of the etiology, pathophysiology, risk factors, clinical presentation of ACS, and preventive and treatment strategies for effective management of ACS finds the presence of severe hypoxemia is a useful predictor of severity and outcome.
How I Treat Acute Chest Syndrome in Asthmatic Children with Sickle Cell Disease. A Practical Review
TLDR
This literature review failed to conclude on therapeutic modalities of ACS in asthmatic children with sickle cell disease, and only a well designed, multicenter adequately-powered randomized controlled study of each of them will allow assessing their real benefits and risks.
Therapeutic Approaches of Acute Thoracic Syndrome in Patients with Falciform Disease
TLDR
Early diagnosis and introduction of an effective approach to complication are needed to improve outcomes and minimize associated morbidity and mortality in patients with sickle cell disease.
Role of Steroids in Sickle Cell Patients With Acute Chest Syndrome
TLDR
Treatment with systemic steroids for ACS is associated with more severe disease and Corticosteroids were not associated with a higher readmission rate in the study population, even in ACS patients with comorbid asthma.
Cerebral fat embolism syndrome mimicking thrombotic thrombocytopenic purpura in a patient with hemoglobin SC disease
TLDR
A 54-year-old man with hemoglobin SC disease and a history of substance abuse presented to the Emergency Department from a nursing home with progressive weakness, shortness of breath, and lower back pain, and the presence of iris atrophy as a complication of his sickle cell disease.
Recent advances in the management of acute bronchiolitis
TLDR
Apnea is a very important aspect of the management of young infants with bronchiolitis and clinical, radiographic, and pathologic characteristics, as well as the recent advances in management of acute bronchiolaitis are focused on.
Original Research: Acute chest syndrome in sickle cell disease: Effect of genotype and asthma
TLDR
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.
Acute chest syndrome in sickle cell disease patients: Experience from a resource constrained setting
TLDR
The use of the algorithm as well as the preemptive EBT improved the outcome of the children accessing care at a University Teaching Hospital in a resource-constrained setting.
Acute bronchiolitis in infants, a review
TLDR
The cornerstones of the management of viral bronchiolitis are the administration of oxygen and appropriate fluid therapy, and overall a “minimal handling approach” is recommended.
...
...

References

SHOWING 1-10 OF 137 REFERENCES
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.
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.
"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.
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.
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.
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.
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.
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.
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.
...
...