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自1889年Von Recklinghausen首次报告血色病以来,经多年不断研究,目前已能肯定血色病的发病机制系由于机体对铁质呈进行性吸收过度,导致体内肝、心、胰以及其他器官铁质大量堆集。本病可分为特发性(家族性)血色病和继发性血色病二类。后者大多继发于某些贫血、酒精性肝硬化、高铁饮食、大量反复输血、先天性转铁蛋白缺乏、门腔静脉吻合术后和迟发型皮肤卟啉症等。本文仅就特发性血色病的一些近展作综述介绍。
Since the first report of hemochromatosis in 1889 by Von Recklinghausen, after years of continuous research, it has been confirmed that the pathogenesis of hemochromatosis is due to the body’s progressive absorption of iron, resulting in a large amount of iron in the liver, heart, pancreas and other organs Stack. The disease can be divided into idiopathic (familial) hemochromatosis and secondary hemochromatosis two categories. The latter are mostly secondary to some anemia, alcoholic cirrhosis, high-iron diet, a large number of repeated blood transfusions, congenital transferrin deficiency, portal venous anastomosis and delayed type of skin porphyria and so on. This article reviews just a few recent episodes of idiopathic hemochromatosis.