ESTIMATION OF LIVER IRON CONTENT USING MRI

本子项目是利用由NIH/NCRR资助的中心赠款提供的资源的众多研究子项目之一。子项目和研究者（PI）可能已经从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。列出的机构是中心的，不一定是研究者的机构。地中海贫血患者往往依赖输血，导致潜在的进行性铁超载。肝脏、脾脏、骨髓、心脏和内分泌腺受到损害，在未经治疗的患者中不可避免地产生过早的心脏和肝脏衰竭。铁螯合剂地呋沙明在这些专利中极大地提高了生存率和生活质量，但它要求每天皮下输注，导致患者严重不遵守。适当剂量的螯合治疗需要监测全身铁负荷。血清学估计值不可靠，因此肝活检仍然是治疗监测的基础。本研究建议使用磁共振成像（MRI）来估计肝脏铁含量。核磁共振成像具有非侵入性、较便宜的优点，可以提供有关铁沉积在所有身体组织中的信息。用于心血管成像的超快磁共振技术的最新进展有望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示的肝活检的地中海贫血患者中得到验证。虽然近期目标是减少或消除肝活检，但长期目标是提高对铁超载患者铁储存和消除药代动力学的理解。

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.