MUCOSAL IRON UPTAKE AND TRANSFER IN HEMOCHROMATOSIS

该项目的长期目标是开发一种非侵入性和准确的方法，用于遗传性血色素沉着症（HHC）患者的非放射性（稳定）同位素标记的铁的粘膜摄取和转移的测量。该方法的基础是摄入一个含有标记（58Fe）硫酸亚铁、不可吸收标记物氯化镝（DyCl3）和视觉标记物fd&c# 1（亮蓝色）的明胶胶囊，然后通过分析第一个视觉标记粪便的一小部分来测量初始粘膜摄取，以检测标记铁和镝。通过摄入循环红细胞中的同位素来测量吸收的标记铁的全身潴留，或在口服剂量的同时注射小剂量的57Fe转铁蛋白1-3小时后测定血浆中58Fe/57Fe的比值。拟议的i期研究的具体目的是在10例无并发症的缺铁患者中证明这种方法的可行性。建议的商业应用：这项研究将为血色素沉着症患者提供一种常规使用的方法。它在血色素沉着症的诊断、预后预测及研究等方面有广泛的应用。

The long-term objectives of this project are to develop a non-invasive and accurate method for the measurement of mucosal uptake and transfer of iron labeled with a non-radioactive (stable) isotope for general-purpose use with hereditary hemochromatosis (HHC) patients. The method is based on ingesting a single gelatin capsule containing labeled (58Fe) ferrous sulfate, the non-absorbable marker dysprosium chloride (DyCl3), and the visual marker FD&C#1 (brilliant blue) followed by the measurement of initial mucosal uptake by analysis of a small portion of the first visually marked stool for labeled iron and Dy. Whole-body retention of absorbed labeled iron is measured from either incorporation of the ingested isotope in circulating red cells, or measured of the ratio for 58Fe/57Fe in plasma 1-3 hours after injection of a small dose of 57 Fe- transferrin concurrently with the oral dose. The specific aims of the proposed phase-I research are to demonstrate the feasibility of this approach in ten patients with uncomplicated iron-deficiency. PROPOSED COMMERCIAL APPLICATIONS: This research will lead to a method for routine use in hemochromatosis patients. It has applications in confirming diagnosis, predicting prognosis, and many aspects of hemochromatosis research.