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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Control | Age matched healthy subjects |
| |
| Sickle Cell Patients | Patients with established SCD |
| |
| Paroxysmal Nocturnal Hemoglobinuria patients | Patients with PNH |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Imaging | Other | Contrast ultrasound perfusion imaging and complete echocardiography |
|
| Measure | Description | Time Frame |
|---|---|---|
| Microvascular perfusion |
| 1 hour |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with SCD or PNH who are seen in the OHSU Hematology clinic and are asked to participate
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Oregon Health & Science University | Portland | Oregon | 97239 | United States |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| D006457 | Hemoglobinuria, Paroxysmal |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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| ID | Term |
|---|---|
| D014965 | X-Rays |
| ID | Term |
|---|---|
| D060733 | Electromagnetic Radiation |
| D055590 | Electromagnetic Phenomena |
| D060328 | Magnetic Phenomena |
| D055585 | Physical Phenomena |
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| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D009190 | Myelodysplastic Syndromes |
| D001855 | Bone Marrow Diseases |
| D011827 | Radiation |
| D011839 | Radiation, Ionizing |