Division of Hematology and Blood and Marrow Transplant, University of Kentucky, Lexington, KY, USA.
Markey Cancer Center, University of Kentucky, Lexington, KY, 40536, USA.
Ann Hematol. 2018 Sep;97(9):1527-1534. doi: 10.1007/s00277-018-3407-5. Epub 2018 Jun 29.
Copper is a crucial micronutrient needed by animals and humans for proper organ function and metabolic processes such as hemoglobin synthesis, as a neurotransmitter, for iron oxidation, cellular respiration, and antioxidant defense peptide amidation, and in the formation of pigments and connective tissue. Multiple factors, either hereditary or acquired, contribute to the increase in copper deficiency seen clinically over the past decades. The uptake of dietary copper into intestinal cells is via the Ctr1 transporter, located at the apical membrane aspect of intestinal cells and in most tissues. Copper is excreted from enterocytes into the blood via the Cu-ATPase, ATP7A, by trafficking the transporter towards the basolateral membrane. Zinc is another important micronutrient in animals and humans. Although zinc absorption may occur by direct interaction with the Ctr1 transporter, its absorption is slightly different. Copper deficiency affects physiologic systems such as bone marrow hematopoiesis, optic nerve function, and the nervous system in general. Detailed pathophysiology and its related diseases are explained in this manuscript. Diagnosis is made by measuring serum copper, serum ceruloplasmin, and 24-h urine copper levels. Copper deficiency anemia is treated with oral or intravenous copper replacement in the form of copper gluconate, copper sulfate, or copper chloride. Hematological manifestations are fully reversible with copper supplementation over a 4- to 12-week period. However, neurological manifestations are only partially reversible with copper supplementation.
铜是动物和人类必需的微量营养素，对于正常的器官功能和代谢过程（如血红蛋白合成）、作为神经递质、铁氧化、细胞呼吸和抗氧化防御肽酰胺化，以及在色素和结缔组织的形成中都很重要。过去几十年中，临床上观察到铜缺乏症的增加是由多种因素引起的，这些因素既有遗传性的，也有获得性的。肠道细胞对膳食铜的摄取是通过位于肠细胞顶膜侧的 Ctr1 转运体进行的，在大多数组织中也是如此。铜通过将转运体转运到基底外侧膜，从肠细胞中经 Cu-ATPase（ATP7A）分泌到血液中。锌也是动物和人类的另一种重要的微量营养素。尽管锌的吸收可能通过与 Ctr1 转运体的直接相互作用发生，但它的吸收略有不同。铜缺乏会影响生理系统，如骨髓造血、视神经功能和整个神经系统。本文详细解释了其病理生理学及其相关疾病。通过测量血清铜、血清铜蓝蛋白和 24 小时尿铜水平来诊断。铜缺乏性贫血采用口服或静脉注射葡萄糖酸铜、硫酸铜或氯化铜的形式进行铜替代治疗。在 4 到 12 周的时间内补充铜可完全逆转血液学表现。然而，铜补充仅部分逆转神经表现。
