Paroxysmal Nocturnal Hemoglobinuria, Rheologic Disease, Sickle Cell Disease
Conditions
Keywords
Rheology, Sickle cell, Paroxysmal nocturnal hemoglobinuria, contrast ultrasound
Brief summary
The purpose of this study is to examine how abnormal blood flow in the small vessels (microvessels) of the heart, muscle and kidney in paroxysmal nocturnal hemoglobinuria (PNH) or sickle cell disease leads to poor functioning of the heart and kidney. To test this question, the investigators will perform imaging tests (contrast ultrasound perfusion imaging) to look at the flow and function of these microvessels and compare this information to heart and kidney function. To further look at this question, patients who have PNH will be studied before and after starting a new drug (Soliris) that decreases damage to blood cells. In patients with sickle cell disease, patients will be studied at baseline (not during a pain crisis) and also during a pain crisis if one develops.
Interventions
OTHERImaging
Contrast ultrasound perfusion imaging and complete echocardiography
Sponsors
Oregon Health and Science University
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
19 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
* Established diagnosis of PNH or SCD Age \> 18 years old
Exclusion criteria
* Pregnant or lactating women Presence of significant right to left shunting Allergy to ultrasound contrast agent Reactive airways disease Significant peripheral or coronary artery disease
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Microvascular perfusion | 1 hour | 1. Myocardial microvascular blood flow (MBF) and capillary blood velocity (CBV) on study day. 2. Renal MBF on study day 3. Skeletal muscle MBF and CBV on study day 4. Myocardial MBF and CBV during hyperemia 5. Skeletal muscle CBV during hyperemia |
Countries
United States
Outcome results
None listed