Association between plasma free haem and incidence of vaso‐occlusive episodes and acute chest syndrome in children with sickle cell disease
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Summary: We tested the hypothesis that extracellular haem is linked to the incidence of acute complications of sickle cell disease (SCD). Using multivariable regression analysis, higher plasma free haem, but not total plasma haem, was associated with increased odds of vaso‐occlusive crisis (VOC) [P = 0·028, odds ratio (OR); 2·05, 95% Confidence Interval (CI) = 1·08–3·89] and acute chest syndrome (ACS) [P = 0·016, OR; 2·56, CI = 1·19, 5·47], after adjusting for age and gender in children with SCD. These findings suggest that haem and factors that influence its concentration in plasma may be informative of the risk of VOC and ACS in SCD patients.
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The role of hemolysis in acute chest syndrome is underscored by clinical observations linking severe hemolysis to increased risk.
“The role of hemolysis in ACS is underscored by clinical observations linking severe hemolysis to increased risk (216, 217)”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [216]
The Abstract reports that higher plasma free haem was associated with increased odds of acute chest syndrome in children with sickle cell disease. Results and Discussion also links higher bilirubin, a marker of haemolysis, to ACS, but cautions that haemolysis itself may not be a major discriminator. The evidence supports an association with specific markers, not the broader claim that severe haemolysis increases risk. (LLM, conf=0.85)
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Pathophysiology of Sickle Cell Disease
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- Authors
- Adisa, O. A., Hu, Y., Ghosh, S., Aryee, D., Osunkwo, I., & Ofori‐Acquah, S. F.
- Affiliations
- Emory University (US)
- Department of Biostatistics and Bioinformatics Rollins School of Public Health Emory University Atlanta GA USA
- Division of Hematology, Oncology and Bone Marrow Transplantation Department of Pediatrics Emory University School of Medicine Emory University Atlanta GA USA
- Venue
- British Journal of Haematology
- Publisher
- Wiley
- Published
- 2013-06-27
- Volume
- 162
- Issue
- 5
- Pages
- 702-705
- ISSN
- 0007-1048 (ISSN-L)
- 1365-2141
- PMC
- PMC3745813
- OpenAlex
- W2031998621
- Cited by
- 88
- References
- 16
- Citations by year
- 2026: 4 · 2025: 7 · 2024: 1 · 2023: 5 · 2022: 4 · 2021: 11 · 2020: 8 · 2019: 9 · 2018: 6 · 2017: 9 · 2016: 12 · 2015: 7 · 2014: 3 · 2013: 1 · 2012: 1
- FWCI
- 3.77
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- British Journal of Haematology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Aflac
- National Heart, Lung, and Blood Institute (R01HL106192, K02HL088026, U01HL117721)
- Topics
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Hemoglobin structure and function
- Health Sciences › Medicine › Hematology › Blood groups and transfusion
- Keywords
- sickle cell disease, vaso-occlusive crisis, acute chest syndrome, children, disease complications, risk factors
- MeSH terms
- Adolescent, Anemia, Sickle Cell, Arterial Occlusive Diseases, Child, Child, Preschool, Female, Heme, Humans, Male, Biomarkers, Acute Chest Syndrome
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