1889年Von kecklinghausen首先记载了血色病(Hemochromatosis)这个名称,其后渐有报道,直到1935年才认为本病是铁代谢障碍的结果。目前认为特发性血色病,是由于铁吸收调节缺陷,实质性铁过荷,最后导致脏器损伤及功能障碍的一种遗传性疾病。现综述如下。一、临床表现:本病多数病人起病隐匿,发病与年龄及性别有密切关系,20岁以前非常罕见,常在50岁达高峰,中数年龄55.5岁。因妇女在整个月经及妊娠期铁丢失增加,男性较女性多5倍。病情轻重与组织铁过荷程度 Von kecklinghausen first documented the name of Hemochromatosis in 1889 and later reported that it was not until 1935 that the disease was thought to be the result of an iron metabolism disorder. Currently considered idiopathic hemochromatosis, is due to iron absorption regulation defects, substantial iron overload, and finally lead to organ damage and dysfunction of a genetic disease. Are summarized below. First, the clinical manifestations: Most patients with occult onset of the disease, the incidence and age and gender are closely related, very rare before the age of 20, often reached the peak in the 50-year-old, the median age of 55.5 years. Because women increase the loss of iron throughout menstruation and gestation, men are five times more likely than women. Illness and tissue iron overload degree