Iron-mediated Vascular Disease in Sickle Cell Disease.
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 150
- 试验地点
- 1
- 主要终点
- Liver iron concentration (LIC), pancreas R2*, and kidney R2*, measured by MRI
研究概览
简要总结
The purpose of this research study is to determine the frequency and severity of iron overload in patients with Sickle Cell Anemia and its relationship to blood vessel function. The investigators hypothesize that intermittent transfusions that these patients receive during hospitalizations produces significant iron overload and impairs blood vessel relaxation.
详细描述
Patients with sickle cell anemia often require blood transfusion as part of the treatment for their disease. Each teaspoon of transfused blood contains about 5 mg of iron and the levels of iron in sickle cell patients increase rapidly with each transfusion. While iron is necessary for many bodily functions, too much iron damages blood vessels, liver, hormone producing glands (pancreas, pituitary and thyroid) and the heart. It is important to know how iron damages blood vessels because most of the problems experienced by sickle cell anemia patients (stroke, kidney failure, pulmonary hypertension, heart disease) result from blood vessel damage. In this trial, iron in the liver, pancreas, and kidney will be measured noninvasively by MRI while vascular function will be measured by ultrasound and tissue Doppler. Patients will be recruited primarily from the greater Los Angeles area, although patients from greater distances will be allowed to participate.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Only
- 时间视角
- Cross Sectional
入排标准
- 年龄范围
- 13 Years 至 —（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Age > 13 years
- •Documented diagnosis of sickle cell anemia (SS, SC, Sß0, Sß+)
- •Transfused no more than 8 times in a year.
排除标准
- •Cardiac pacemaker, implantable neurostimulator or other MRI incompatible device.
- •History of extreme claustrophobia in MRI machine or other reason for inability to do MRI without sedation.
- •Inability to be positioned on the MRI table for sufficient time to complete the MRI exams.
- •Any medical or psychological condition that, in the opinion of the local investigator, would make it unsafe or ill-advised for the subject to participate.
- •Currently not receiving chronic transfusion therapy, defined as greater than 8 transfusions per year.
结局指标
主要结局
Liver iron concentration (LIC), pancreas R2*, and kidney R2*, measured by MRI
时间窗: 15 min MRI done completed during one time study visit
Liver iron concentration (LIC) will be used as a surrogate for total body iron, pancreatic iron represents a surrogate for extravascular iron deposition, and renal iron as a surrogate for chronic intravascular hemolysis. In addition, labile plasma iron will be measured in blood plasma.
次要结局
- Vascular function.(Scheduled during one time study visit)
研究者
John C. Wood
Associate Professor of Pediatrics - Division of Cardiology
Children's Hospital Los Angeles