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Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Course
Open accessAbstract
Acute chest syndrome (ACS) is an important cause of morbidity and mortality in sickle cell disease (SCD). Previous studies reported conflicting pictures of ACS making therapeutic interventions difficult. The Cooperative Study of Sickle Cell Disease prospectively followed 3,751 patients enrolled from birth to 66 years of age for ACS. Data on presenting signs and symptoms, laboratory findings, and hospital course were collected. There were 1,722 ACS episodes in 939 patients. Young children (age 2 to 4 years) presented with fever and cough, a negative physical exam, and rarely had pain. Adults were often afebrile and complained of shortness of breath, chills, and severe pain. Upper lobe disease was more common in children; multilobe and lower lobe disease affected adults more often. Severe hypoxia occurred in 18% of adults tested and could not be predicted by examination or laboratory findings. Bacteremia was documented in 3.5% of episodes, but was strongly influenced by age (14% of infants and 1.8% of patients <10 years). ACS was most common in winter with children having the most striking increase. Transfusion was used less frequently, but earlier in children. Young children were hospitalized for 5.4 days versus 9 days for adults. Fifty percent of adults had a pain event in the 2 weeks preceding ACS and children were more likely to have febrile events. The death rate was four times higher in adults than in children. Fatal cases generally developed rapid pulmonary failure and one third were associated with bacteremia. Age has a striking effect on the clinical picture of ACS. 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.
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“Infection is the most common ACS trigger in children; common isolates include atypical microorganisms and Streptococcus pneumoniae (208).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [208]
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“Elevated initial platelet count and dramatic drops during VOC have been found to portend a poor prognosis in ACS (29, 208, 219).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [208]
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Orphan drugs for sickle vaso-occlusion: dawn of a new era of targeted treatment
Dampier, C. (2015) · Orphan Drugs Research and Reviews · doi:10.2147/odrr.s46305 · Cited by 5
“55,56 Some of these pulmonary episodes represent an infectious process, particularly in young children.”
Association of KLOTHO polymorphisms with clinical complications of sickle cell anemia
Batista, J. V. G. F., Pereira‐Martins, D. A., Falcão, D. A., Domingos, I. F., Arcanjo, G. D. S., Hatzlhofer, B. L. D., Weinhäuser, I., Batista, T. H. C., Cardoso, P. R. G., dos Anjos, A. C., et al. (2021) · Annals of Hematology · doi:10.1007/s00277-021-04532-w · Cited by 5
Garnier, M., Hafiani, E. M., Arbelot, C., Blayau, C., Labbé, V., Stankovic, K., Lionnet, F., Bonnet, F. J., Fulgencio, J.-P., Fartoukh, M. S., et al. (2019) · Annals of Intensive Care · doi:10.1186/s13613-019-0583-y · Cited by 5
Acute Chest Syndrome in Sickle Cell Anemia
Ballas, S. K. (2000) · Journal of Intensive Care Medicine · doi:10.1046/j.1525-1489.2000.00123.x · Cited by 5
Saunthararajah, Y., & Vichinsky, E. P. (2017) · Elsevier eBooks · doi:10.1016/b978-0-323-35762-3.00042-1 · Cited by 6
Self-citationTransient Aortic Thrombus in a Sickle-Cell Patient with Chest Pain
Filipek, M. S., Primack, S. L., Hronas, T., & Koslin, D. B. (2000) · American Journal of Roentgenology · doi:10.2214/ajr.175.5.1751287 · Cited by 5
Antibiotics for treating community acquired pneumonia in people with sickle cell disease
Martí-Carvajal, A. J., & Conterno, L. D. O. (2012) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd005598.pub3 · Cited by 5
Delicou, S. (2016) · Hematology & Transfusion International Journal · doi:10.15406/htij.2016.02.00042 · Cited by 4
Khalaf, Z., & Mahmood, M. (2022) · Surgery in Practice and Science · doi:10.1016/j.sipas.2022.100143 · Cited by 4
Cheminet, G., Brunetti, A., Khimoud, D., Ranque, B., Michon, A., Flamarion, E., Pouchot, J., Jannot, A., & Arlet, J. (2023) · British Journal of Haematology · doi:10.1111/bjh.18777 · Cited by 4
“In contrast, the prevalence of chest pain (73%) and crackles (64%) is consistent with the literature data.(6,13,28) Interestingly, bronchial breathing was observed in 32% of episodes; to the best of our knowledge, this phenomenon has not previously been described in the literature on ACS.”
BackgroundResultCohen, R. T., Burrowes, S. A. B., Williams, C. J., Neri, C. M., Klings, E. S., Jones, K. C., Walkey, A. J., & Drainoni, M. (2024) · Pediatric Blood & Cancer · doi:10.1002/pbc.31142 · Cited by 4
Age‐dependent pathophysiology of acute chest syndrome in children with sickle cell disease
Renella, R. (2015) · American Journal of Hematology · doi:10.1002/ajh.23975 · Cited by 4
“ACS5 acute chest syndrome, VOC5 vasoocclusive crisis, tmedian readmit5median time of re-admission post-initial ACS episode, n.s5 statistically non-significant difference.”
BackgroundInfluentialAcute Chest Syndrome in Children with Sickle Cell Anaemia: An Audit in Port Harcourt, Nigeria
George, I. O., & Aiyedun, C. N. (2015) · Open Journal of Pediatrics · doi:10.4236/ojped.2015.54048 · Cited by 4
Transfusion therapy for sickle cell disease
Howard, J., & Robinson, S. E. (2016) · ISBT Science Series · doi:10.1111/voxs.12223 · Cited by 4
Clinical Manifestations of Sickle Cell Anemia: Infants and Children
Nickel, R. S., & Hsu, L. L.-Y. (2016) · Springer eBooks · doi:10.1007/978-3-319-06713-1_9 · Cited by 4
A Differential Approach to an Uncommon Case of Acute Anemia in a Child With Sickle Cell Disease
Srinivasan, A., & Gourishankar, A. (2019) · Global Pediatric Health · doi:10.1177/2333794x19848674 · Cited by 4
Innocent Murmurs, Syncope, and Chest Pain
Newburger, J. W., Alexander, M. E., & Fulton, D. R. (2006) · Elsevier eBooks · doi:10.1016/b978-1-4160-2390-6.50027-1 · Cited by 4
SARS‐CoV‐2 infection in pediatric patient with hemoglobin SC disease
Dagalakis, U., Hammershaimb, E. A., McArthur, M. A., & Macatangay, R. A. (2020) · Pediatric Blood & Cancer · doi:10.1002/pbc.28430 · Cited by 4
Heilbronner, C., Grimaud, M., Oualha, M., Sommet, J., Rambaud, J., Brousse, V. A. M., Allali, S., & Renolleau, S. (2021) · Archives de Pédiatrie · doi:10.1016/j.arcped.2021.06.002 · Cited by 4
Fatal pulmonary thromboembolism associated with hemoglobin SC disease in a 15 year-old boy
Amancio, T. T., Costa, A. D. C. L., Borsato, M. R. L., Marins, L., Bacchi, L. M., & Zerbini, M. C. N. (2011) · Autopsy and Case Reports · doi:10.4322/acr.2011.004 · Cited by 4
Al-Eyadhy, A. A., & Al‐Jelaify, M. (2022) · Journal of Infection and Chemotherapy · doi:10.1016/j.jiac.2022.05.017 · Cited by 4
Friedrich, P. M., Steinfield, E., Kim, F., Hays, M. M., Lehmann, L., & Sprinz, P. G. (2015) · American Journal of Health Education · doi:10.1080/19325037.2015.1021059 · Cited by 5
Candida albicans induced acute chest syndrome in sickle cell disease
Agarwal, H. S., & Kuttesch, J. F. (2019) · Pediatric Blood & Cancer · doi:10.1002/pbc.27727 · Cited by 4
Acute venous thromboembolism after initiation of voxelotor for treatment of sickle cell disease
Lemon, N. M., Sterk, E. J., & Rech, M. A. (2021) · The American Journal of Emergency Medicine · doi:10.1016/j.ajem.2021.12.031 · Cited by 4
Metadata
- Authors
- Vichinsky, E. P., Styles, L. A., Colangelo, L. H., Wright, E. C., Castro, O., Nickerson, B., & Disease, T. C. S. O. S. C.
- Affiliations
- Children's Hospital of Orange County (US)
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 1997-03-01
- Volume
- 89
- Issue
- 5
- Pages
- 1787-1792
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W1272191569
- Cited by
- 573
- References
- 30
- Citations by year
- 2026: 13 · 2025: 18 · 2024: 13 · 2023: 17 · 2022: 22 · 2021: 23 · 2020: 20 · 2019: 27 · 2018: 22 · 2017: 20 · 2016: 32 · 2015: 22 · 2014: 26 · 2013: 28 · 2012: 25
- FWCI
- 10.28
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Blood (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Epidemiology › Pneumonia and Respiratory Infections
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Malaria Research and Control
- Keywords
- acute chest syndrome, sickle cell disease, clinical features, age differences, bacteremia, hypoxia, respiratory failure, pain, fever, hospital course, mortality, seasonal variation, blood transfusion
- MeSH terms
- Adolescent, Adult, Aged, Anemia, Sickle Cell, Chest Pain, Child, Child, Preschool, Humans, Infant, Middle Aged, Prospective Studies, Respiratory Insufficiency, Syndrome
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