Acute chest syndrome: sickle cell disease

@article{Paul2011AcuteCS,
  title={Acute chest syndrome: sickle cell disease},
  author={Rabindra N. Paul and Oswaldo L. Castro and Anita Aggarwal and Patricia Ann Oneal},
  journal={European Journal of Haematology},
  year={2011},
  volume={87}
}
Acute chest syndrome (ACS) is a common complication and reason for hospital admission in patients with sickle cell disease (SCD). It is also the most common cause of death in this patient population. 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. This comprehensive review will focus on the pathogenesis, clinical… 
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52 Citations
Highly Influential Citations
5
Background Citations
14
Results Citations
2

52 Citations

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.
Severe Acute Chest Syndrome in a Sickle Cell Patient That Required Repeated Exchange Transfusion
TLDR
This case report presents an interesting severe form of ACS that, quite unusually, required repeated exchanged blood transfusion to achieve clinical improvement.
Sickle Cell Disease
TLDR
Children and adolescents with sickle cell disease must be periodically evaluated through oximetry, lung function tests, sleep study, and echocardiograms to detect obstructive and restrictive diseases that frequently worsens the lung condition.
Acute Chest Syndrome in Sickle Cell Disease Patients Post Caesarean Delivery
TLDR
It is suggested that prompt recognition of ACS in a pregnant woman with SCD and collaborative medical and obstetric management are essential to optimize maternal and fetal outcomes.
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.
Pulmonary complications in adult patients with sickle cell disease
TLDR
The present review comprises the following pulmonary complications of SCD: i) acute chest syndrome, ii) venous thromboembolus, iii) asthma, iv) respiratory dysfunction, v) respiratory disorders during sleeping, vi) pulmonary hypertension.
Sickle Cell Disease and Acute Chest Syndrome: Mechanisms and Pathogenenesis
TLDR
Experimental data indicate heme-induced ACS may involve vaso-occlusion and the release of neutrophil extracellular traps in the lung, and the heme hypothesis posits that the lung injury in ACS is caused by acute inflammation directly involvingextracellular heme, a prototypical erythroid DAMP molecule released by hemolysis.
Clinical management of the acute complications of sickle cell anemia: 11 years of experience in a tertiary hospital.
Rapid Fire: Sickle Cell Disease.
Acute Chest Syndrome, Avascular Necrosis of Femur, and Pulmonary Embolism All at Once: An Unexpected Encounter in the First-Ever Admission of a Sickle Cell Patient
TLDR
This case report is found to be the first case report in the world where all these three complications of sickle cell disease developed simultaneously in a patient.
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References

SHOWING 1-10 OF 195 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.
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.
Chest infections and syndromes in sickle cell disease of childhood.
  • Z. Dreyer
  • Medicine
    Seminars in respiratory infections
  • 1996
TLDR
Caution is advised when administering drugs such as morphine for pain control because of risk of exacerbation of pulmonary symptomatology, and maintenance of adequate oxygenation treatment of possible underlying infection, and adequate hemoglobin delivery are essential.
Acute chest syndrome of sickle cell disease: radiographic and clinical analysis of 70 cases
TLDR
The chest radiographs of children with ACS without an identifiable etiology have an extremely typical appearance and evolution, and only in cases which do not have this typical pattern should infection be suspected as the underlying cause.
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.
The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease.
TLDR
The acute chest syndrome (ACS), a pneumonia-like illness in sickle cell patients, is one of the most frequent causes of their morbidity and hospitalizations, and a positive association between ACS rate and steady-state leukocyte count is found.
Acute chest syndrome in adults with sickle cell disease.
TLDR
Results confirm that fat embolism is probably a frequent mechanism of ACS in adult patients, however, fat emblism was not associated with a more severe clinical course, suggesting that bronchoscopy and BAL have little impact on the management of these patients.
Phospholipase A2 levels in acute chest syndrome of sickle cell disease.
TLDR
A role for SPLA2 in the diagnosis and, perhaps, in the pathophysiology of patients with ACS is suggested and three different measures of clinical severity are correlated with the onset of ACS.
Acute chest syndrome in sickle cell disease: etiology and clinical correlates.
Acute kidney injury in sickle patients with painful crisis or acute chest syndrome and its relation to pulmonary hypertension.
  • V. Audard, S. Homs, A. Mekontso Dessap
  • Medicine
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • 2010
TLDR
AKI incidence during vaso-occlusive complications of SCD is relatively low and appears to be confined to patients with ACS and pulmonary hypertension, which suggests a pathophysiological process involving right ventricular dysfunction and venous congestion.
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