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Published on: October 15, 2010
Peripheral arterial tonometry in assessing endothelial dysfunction in pediatric sickle cell disease
Krupa M Sivamurthy1, Carlton Dampier, MaryLou MacDermott
1Hematology/Oncology, St. Christopher's Hospital for Children, Philadelphia, Pennsylvania 19141, USA.
Pediatric Hematology and Oncology
|December 4, 2009
Summary
Peripheral arterial tonometry (PAT) measures endothelial dysfunction (EDF) in children with sickle cell disease (SCD). Many children with SCD showed blunted reactive hyperemia index (RHI) values, indicating impaired vascular function.
Area of Science:
- Pediatric Hematology
- Vascular Biology
- Noninvasive Diagnostics
Background:
- Sickle cell disease (SCD) involves hemolysis and oxidative stress, leading to endothelial dysfunction (EDF).
- Peripheral arterial tonometry (PAT) is a noninvasive method to assess EDF via reactive hyperemia index (RHI).
Purpose of the Study:
- To investigate the influence of hemoglobin genotype and treatment on EDF in children with SCD using PAT.
- To evaluate the correlation between RHI and clinical parameters in pediatric SCD patients.
Main Methods:
- PAT was employed to measure RHI in 36 children diagnosed with SCD.
- Statistical analysis, including Spearman correlation, was used to assess relationships between RHI, hemoglobin genotype, treatment, and clinical factors.
Main Results:
- A blunted RHI was observed in most children with SCD, particularly those with increased symptomatology (p = .032).
- Abnormal RHI values were noted in children receiving chronic transfusions or hydroxyurea therapy.
- RHI showed a significant correlation with the reticulocyte fraction (r = -.47, p = .037).
Conclusions:
- PAT and RHI represent promising tools for assessing endothelial dysfunction in pediatric sickle cell disease.
- Further research is warranted to explore the utility of PAT in managing SCD and its complications.
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