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在儿童的生长发育阶段,铜是生命所必需的微量元素,正常人体内含铜量为100 ̄150毫克。肝是铜代谢的中心。正常情况下,人体铜的排除维持动态平衡。有的儿童患常染色体遗传性疾病,导致肝内肽酶缺陷或缺乏,使铜在体内沉积,主要沉积于肝、肾、中枢神经及角膜等,从而导致肝脏器官破坏
In children’s growth and development stage, copper is essential trace elements of life, the normal human body contains 100 to 150 mg of copper. Liver is the center of copper metabolism. Under normal circumstances, the body of copper to maintain the dynamic balance of exclusion. Some children with autosomal genetic diseases, leading to defects or lack of liver peptidase, the deposition of copper in the body, mainly deposited in the liver, kidney, central nervous system and cornea, resulting in liver damage