鐵質過多造成血色素沉積症之研究分析

鐵質（iron）是人體細胞所需要的基本營養素，為電子傳遞鏈之重要組成及構成血紅素與肌球蛋白（myoglobulin），以攜帶氧分子。正常情況下，鐵質會與運鐵蛋白（transferrin）結合，循環全身並維持在一個恆定的狀態。若鐵質與運鐵蛋白的結合趨於飽和，會以非運鐵蛋白結合的鐵（nontransferrin bound iron, NTBI）存在，這類非運鐵蛋白結合的鐵，對正常細胞有很大的毒性，會對人體器官的細胞脂質、蛋白質與核酸，造成過氧化而形成傷害，使得器官功能受損，形成血色素沉積症（hemochromatosis）。本研究是從4位因血色素沉積症致死案例，經大體解剖後，取下各器官，做成組織切片，並以HE（Hematoxylin and Eosin）染色及普魯士藍染色（Prussian blue stain），研究鐵質會沉積在那些組織及器官，並在顯微鏡下觀察其組織病理變化，以進一步探討致死原因。綜合本研究發現，鐵離子過多會沉積在肝臟導致肝硬化；在胰臟導致糖尿病；在心臟導致心衰竭；在皮膚形成典型褐色皮膚；甚至可見褐色鐵離子顆粒沉積在胃、脾、淋巴腺、腦下垂體、腎上腺、甲狀腺和副甲狀腺等。

關鍵字

血色素沉積症；鐵質；普魯士藍

並列摘要

Iron is an essential nutrient for human cells. It is responsible for the electronic exchange and it constitutes hemoglobin and myoglobulin for carrying oxygen. Iron and transferrin will combine and maintain at a constant state in normal condition. If over-saturation occurr, it will be existed by non-transferrin bound iron (NTB), which is highly toxic to cells. The lipids, proteins and nucleic acids of cells will be damaged by peroxidation or mutation and the parenchymal cells of organs will be infiltrated by hemosiderin, the so-called hemochromatosis. In this study, we collected four autopsy cases due to hemochromatosis. We analyzed their clinical pathological changes. Prussian blue stain was performed to evaluate the iron distribution in all demonstrated organs. Our comprehensive study found that iron deposition was mainly in the liver with cirrhosis, skin with brown pigmentation, pancreas with diabetic mellitus, heart with congestive heart failure. Iron deposits were also found in the stomach, spleen, lymph nodes and other endocrine organs including pituitary gland, adrenal gland, thyroid gland and parathyroid gland.

並列關鍵字

Hemochromatosis ； Iron ； Prussian blue stain

國際替代計量

鐵質過多造成血色素沉積症之研究分析

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