Imperial College London
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Professor Thomas N Williams

Faculty of Medicine, Department of Surgery & Cancer

Chair in Haemoglobinopathy Research
 
 
 
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Contact

 

tom.williams Website

 
 
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Location

 

Norfolk PlaceSt Mary's Campus

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Summary

 

Publications

Citation

BibTex format

@article{Nardo-Marino:2022:10.1111/bjh.17975,
author = {Nardo-Marino, A and Petersen, J and Brewin, J and Birgens, H and Williams, T and Kurtzhals, J and Rees, D and Glenthøj, A},
doi = {10.1111/bjh.17975},
journal = {British Journal of Haematology},
pages = {609--617},
title = {Oxygen gradient ektacytometry does not predict pain in children with sickle cell anaemia},
url = {http://dx.doi.org/10.1111/bjh.17975},
volume = {197},
year = {2022}
}
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RIS format (EndNote, RefMan)

TY  - JOUR
AB  - The loss of red blood cell (RBC) deformability in sickle cell anaemia (SCA) is considered the primary factor responsible for episodes of acute pain and downstream progressive organ dysfunction. Oxygen gradient ektacytometry (Oxygenscan) is a recently commercialised functional assay that aims to describe the deformability of RBCs in SCA at differing oxygen tensions. So far, the Oxygenscan has been evaluated only by a small number of research groups and the validity and clinical value of Oxygenscan-derived biomarkers have not yet been fully established. In this study we examined RBC deformability measured with the Oxygenscan in 91 children with SCA at King’s College Hospital in London. We found a significant correlation between Oxygenscan-derived biomarkers and well-recognised modifiers of disease severity in SCA: haemoglobin F and co-inherited α-thalassaemia. We failed, however, to find any independent predictive value of the Oxygenscan in the clinical outcome measure of pain, as well as other important parameters such as hydroxycarbamide treatment. Although the Oxygenscan remains an intriguing tool for basic research, our results question whether it provides any additional information in predicting the clinical course in children with SCA, beyond measuring known markers of disease severity.
AU  - Nardo-Marino,A
AU  - Petersen,J
AU  - Brewin,J
AU  - Birgens,H
AU  - Williams,T
AU  - Kurtzhals,J
AU  - Rees,D
AU  - Glenthøj,A
DO  - 10.1111/bjh.17975
EP  - 617
PY  - 2022///
SN  - 0007-1048
SP  - 609
TI  - Oxygen gradient ektacytometry does not predict pain in children with sickle cell anaemia
T2  - British Journal of Haematology
UR  - http://dx.doi.org/10.1111/bjh.17975
UR  - http://hdl.handle.net/10044/1/93012
VL  - 197
ER  -
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