Sickle Cell Disease
Conditions
Brief summary
Endothelial dysfunction contributes to vaso-occlusion and acute pain in sickle cell disease. Near infrared spectroscopy (NIRS) technology can measure tissue oxygenation and endothelial function. The main objective of this study is to study the natural history of tissue muscle oxygenation using NIRS in pediatric sickle cell subjects experiencing acute pain and pediatric sickle cell patients in steady-state.
Interventions
DIAGNOSTIC_TESTNear infrared spectroscopy
NIRS is an emerging technology for measuring tissue oxygenation and endothelial function. NIRS light can penetrate through biological tissues, including skin, bone, and muscle. Similar to other optical methods, light is applied to the region of interest and undergoes scattering and absorption before being detected by a photosensor. This technique is commonly used to assess oxygen availability and consumption in living tissues. Using different wavelengths, NIRS can differentiate between oxygenated and deoxygenated hemoglobin in blood and can measure changes in total hemoglobin concentration, using the sum of oxygenated and deoxygenated hemoglobin.
Sponsors
National Institute of Allergy and Infectious Diseases (NIAID)
Children's National Research Institute
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
6 Years to 21 Years
Healthy volunteers
No
Inclusion criteria
for Subjects with Sickle Cell Disease in Pain Crisis: 1. Age 6 to 21 years old. 2. 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). 3. Acute onset pain crisis in a distribution typical for that subject, onset within the last 7 days and for which hospitalization and parenteral narcotic pain treatment are required. 4. Ability to provide informed written consent.
Exclusion criteria
for Subjects with Sickle Cell Disease in Pain Crisis: 1. Pregnancy. 2. History of non-trivial injury, burns, surgery or skin ulcers on the arms. 3. Fever or suspected sepsis at time of pain crisis 4. Administration of any of the following drugs within the last 14 days: * Phosphodiesterase-5 inhibitors (sildenafil, vardenafil, tadalafil) * Endothelin-1 receptor blockers (bosentan, sitaxentan, ambrisentan, tezosentan) * Nitric oxide donors (nitroglycerin, nitroprusside, nitrates) 5. Ingestion of caffeine within the 12 hours before the start of the study appointment, or tobacco use within the 30 days before the study appointment. 6. Diagnosis with any of the following chronic diseases or conditions: * History of high blood pressure * History of high cholesterol * History of diabetes * History of chronic kidney disease (serum creatinine must not be greater than 2 mg/dL) * History of coronary artery disease or peripheral vascular disease 7. Received a blood transfusion within 7 days of the study procedure. Inclusion Criteria for Subjects with Sickle Cell Disease in Steady State: 1. Age 6-21 years 2. 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). 3. Ability to provide informed written consent.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Tissue oxygenation | 6 months |
Secondary
| Measure | Time frame |
|---|---|
| Nitric oxide | 6 months |
Countries
United States
Outcome results
None listed