An observational study in Sickle Cell Disease, sponsored by National Heart, Lung, and Blood Institute (NHLBI). Completed at 1 site in United States. Open to participants aged 18 Years to 99 Years, including healthy volunteers. Per ClinicalTrials.gov, last updated 2018-10-09.
Sponsored by National Heart, Lung, and Blood Institute (NHLBI) · Observational
Background:
Objectives:
Eligibility:
Design:
sickle cell disease.
Severe recurrent pain is the most common cause of acute morbidity in sickle cell disease1. The underlying pathogenesis was initially thought to be ischemia from obstruction of capillary beds by stiffened red blood cells; however, there is evidence that other factors contribute to the pathogenesis of sickle cell pain crisis, such as inflammation and coagulation, ischemia-reperfusion injury, angiogenesis balance, and vasomotor tone processes that are regulated by endothelial nitric oxide.
Recent clinical data suggest that subjects with sickle cell disease suffer from chronically impaired nitric oxide bioavailability. This has been attributed to increased consumption of nitric oxide by hemoglobin and reactive oxygen species, or decreased production of nitric oxide by endothelial cells; however the roles of nitric oxide bioavailability and endothelial dysfunction during acute pain crisis are controversial and incompletely understood. Although there have been several studies of endothelial function in steady state sickle cell disease, there has been no comprehensive study of endothelial function during pain crisis.
In this study, our primary objective is to measure the reactive hyperemia index (a measure of the endothelial response to shear stress) in twenty sickle cell subjects during acute pain crisis and to compare it with the reactive hyperemia index measured after recovery from pain crisis. This will identify whether there are acute changes in endothelial cell function during sickle cell pain crisis. Our secondary objective is to compare the reactive hyperemia index of thirty-five sickle cell subjects in steady state versus the reactive hyperemia index of thirty-five healthy control subjects. This will identify whether there are chronic differences in endothelial function between sickle cell subjects and healthy control subjects.
This study will determine if there are defects in endothelium-dependent vasodilation in response to shear stress during sickle cell pain crisis. Moreover, this study provides an opportunity to evaluate new physiologic biomarkers of sickle cell pain crisis based on measurements of blood flow, temperature, and oxygenation in the skin. These measurements may serve as clinical endpoints in future studies of disease pathogenesis or therapeutic interventions for sickle cell disease.
1,103 studies on the registry are indexed under Anemia, Sickle Cell; 235 are open to participants now.
This study's enrollment of 94 is close to the median of 100 across 287 observational studies indexed under Anemia, Sickle Cell.
Browse Anemia, Sickle Cell studies →National Heart, Lung, and Blood Institute (NHLBI) is the lead sponsor of 1,117 studies on the registry; 71 are open to participants now.
Of its 57 completed or terminated interventional studies of FDA-regulated products, 49 (86%) have results posted.
Counted across the registry records on this site, refreshed daily.
INCLUSION CRITERIA FOR SUBJECTS WITH SICKLE CELL DISEASE IN PAIN CRISIS:
Diagnosis of sickle cell anemia:
EXCLUSION CRITERIA FOR SUBJECTS WITH SICKLE CELL DISEASE IN PAIN CRISIS:
Administration of any of the following drugs within the last 14 days:
Diagnosis with any of the following chronic diseases or conditions:
SICKLE CELL DISEASE SUBJECTS IN STEADY STATE
INCLUSION CRITERIA FOR SUBJECTS WITH SICKLE CELL DISEASE IN STEADY STATE:
Diagnosis of sickle cell anemia:
a.Diagnosis of sickle cell disease (electrophoresis or HPLC documentation of hemoglobin SS, SC, S-beta-thalassemia or other hemoglobinopathies causing sickle cell disease is required).
EXCLUSION CRITERIA FOR SUBJECTS WITH SICKLE CELL DISEASE IN STEADY STATE:
Administration of any of the following drugs within the last 14 days:
Diagnosis of any of the following chronic diseases or conditions: \<TAB>
HEALTHY CONTROL SUBJECTS
INCLUSION CRITERIA FOR HEALTHY CONTROL SUBJECTS
EXCLUSION CRITERIA FOR HEALTHY CONTROL SUBJECTS
Administration of any of the following drugs within the last 14 days:
Diagnosis with any of the following chronic diseases or conditions:
Skin Blood Flood
Time frame: 1 month
Skin Temperature
Time frame: 1 month
Tissue oxygenation
Time frame: 1 month
This study is completed, as verified in Oct 2018. You cannot join it, but the record below documents what was studied.
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National Heart, Lung, and Blood Institute (NHLBI)