ESTIMATION OF LIVER IRON CONTENT USING MRI

这个子项目是利用由NIH/NCRR资助的中心拨款提供的资源的许多研究子项目之一。子项目和调查员(PI)可能从另一个NIH来源获得了主要资金，因此可能会出现在其他CRISE条目中。列出的机构是针对中心的，而不一定是针对调查员的机构。地中海贫血患者往往依赖输血，导致潜伏的进行性铁超载。肝、脾、骨髓、心脏和内分泌腺受损，在未经治疗的患者中不可避免地导致过早的心脏和肝脏衰竭。铁络合剂地氟沙明极大地改善了这些患者的存活率和生活质量，但其每日皮下输液的要求导致了患者严重的不依从性。适当剂量的螯合治疗需要监测全身铁负荷。血清学评估是不可靠的，所以肝活检仍然是治疗监测的基石。这项研究建议使用磁共振成像(MRI)来估计肝脏铁含量。MRI具有非侵入性、价格低廉的优点，可以提供所有身体组织中铁沉积的信息。超快磁共振技术的最新进展是为心血管成像开发的，预计将比以前的方法产生更敏感和更可靠的铁估计。候选磁共振技术将在接受临床指征的肝活检的地中海贫血患者中得到验证。虽然近期的目标是减少或消除肝脏活检，但长期目标是改善对铁储存和铁超载患者消除药代动力学的了解。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Patients with thalassemia re often transfusion-dependent, leading to insidious and progressive iron overload. Liver, spleen, bone marrow, heart and endocrine glands are compromised producing inevitable premature cardiac and liver failure in untreated patients. The iron chelator, desfuroxamine, has greatly improved survival and quality of life in these patents, but its requirement for daily subcutaneous infusions leads to significant patient noncompliance. Proper dosing of chelation therapy required monitoring of systemic iron loads. Serologic estimators are not reliable, so liver biopsy remains the cornerstone of therapeutic monitoring. This study proposes to use magnetic resonance imaging (MRI) to estimate liver iron content. MRI has the advantage of being non-invasive, less expensive and can provide information about iron deposition n all body tissues. Recent advances in ultrafast MR techniques, developed for cardiovascular imaging, are expected to yield more sensitive and robust iron estimates than previous approaches. Candidate MR techniques will be validated in thalassemic patients undergoing clinically indicated liver biopsy. While the immediate goal is reduction or elimination of liver biopsies, the long term goal is improved understanding of iron storage and elimination pharmacokinetics in iron overload patients.