铁代谢紊乱，驱铜治疗虽可改善但不能使 Wilson 病患者的铁代谢恢复正常。
Iron metabolism is disturbed and anti-copper treatment improves but does not normalize iron metabolism in Wilson's disease.
机构信息
Faculty of Medicine, Collegium Medicum, Cardinal Stefan Wyszyński University in Warsaw, Wóycickiego Str. 1/3, 01-938, Warsaw, Poland.
Second Department of Neurology, Institute of Psychiatry and Neurology, Sobieskiego Str. 9, 02-957, Warsaw, Poland.
出版信息
Biometals. 2021 Apr;34(2):407-414. doi: 10.1007/s10534-021-00289-x. Epub 2021 Feb 8.
Wilson's disease (WD) is a rare hereditary disorder of copper metabolism. Some data suggest that iron metabolism is disturbed in WD and this may affect the course of the disease. The current study aimed to determine whether anti-copper treatment could affect iron metabolism in WD. One hundred thirty-eight WD patients and 102 controls were examined. Serum ceruloplasmin and copper were measured by colorimetric enzyme assay or atomic adsorption spectroscopy, respectively. Routine and non-routine parameters of iron metabolism were measured by standard laboratory methods or enzyme immunoassay, respectively. WD patients, both newly diagnosed and treated, had less serum copper and ceruloplasmin than controls (90.0, 63.0, 22.0 mg/dL, respectively, p < 0.001); in the treated patients blood copper and ceruloplasmin were lower than in untreated patients (p < 0.001). Untreated patients (n = 39) had a higher median blood iron (126.0 vs 103.5 ug/dL, p < 0.05), ferritin (158.9 vs 47.5 ng/mL, p < 0.001), hepcidin (32, 6 vs 12.1 ng/mL, p < 0.001) and sTfR (0.8 vs. 0.7 ug/mL, p < 0.001) and lower blood transferrin (2.4 vs. 2.7 g/L, p < 0.001), TIBC (303.0 vs 338.0 ug/dL, p < 0.001), hemoglobin (13.1 vs 13.9 g/dL, p < 0.01) and RBC (4.3 vs. 4.6, p < 0.002) than controls. Treated patients (n = 99) had a significantly lower median iron (88.0 vs. 126.0 ug/dL, p < 0.001), ferritin (77.0 vs. 158.9 ng/mL, p < 0.005) and hepcidin (16.7 vs. 32.6 ng/mL, p < 001) and higher transferrin (2.8 vs. 2.4 g/L, p < 0.005), TIBC (336.0 vs 303.0 ug/dL, p < 0.001), RBC (4.8 vs. 4.3 M/L, p < 0.001) and hemoglobin (14.4 vs. 13.1 g/dL, p < 0.001) than untreated; the median iron (p < 0.005) was lower, and ferritin (p < 0.005), RBC (p < 0.005) and hepcidin (p < 0.002) were higher in them than in the control group. Changes in copper metabolism are accompanied by changes in iron metabolism in WD. Anti-copper treatment improves but does not normalize iron metabolism.
威尔逊病 (WD) 是一种罕见的遗传性铜代谢紊乱。一些数据表明 WD 中存在铁代谢紊乱，这可能会影响疾病的进程。本研究旨在确定抗铜治疗是否会影响 WD 中的铁代谢。检查了 138 名 WD 患者和 102 名对照者。分别通过比色酶法或原子吸收光谱法测定血清铜蓝蛋白和铜。分别通过标准实验室方法或酶免疫测定法测定铁代谢的常规和非常规参数。与对照组相比，新诊断和治疗的 WD 患者的血清铜和铜蓝蛋白均较低（分别为 90.0、63.0 和 22.0mg/dL，p<0.001）；在治疗患者中，血铜和铜蓝蛋白低于未治疗患者（p<0.001）。未治疗的患者（n=39）的中位血铁（126.0 与 103.5ug/dL，p<0.05）、铁蛋白（158.9 与 47.5ng/mL，p<0.001）、hepcidin（32、6 与 12.1ng/mL，p<0.001）和 sTfR（0.8 与 0.7ug/mL，p<0.001）较高，转铁蛋白（2.4 与 2.7g/L，p<0.001）、总铁结合力（303.0 与 338.0ug/dL，p<0.001）、血红蛋白（13.1 与 13.9g/dL，p<0.01）和红细胞（4.3 与 4.6，p<0.002）较低与对照组相比。治疗的患者（n=99）的中位铁（88.0 与 126.0ug/dL，p<0.001）、铁蛋白（77.0 与 158.9ng/mL，p<0.005）和 hepcidin（16.7 与 32.6ng/mL，p<0.001）较低，转铁蛋白（2.8 与 2.4g/L，p<0.005）、总铁结合力（336.0 与 303.0ug/dL，p<0.001）、红细胞（4.8 与 4.3M/L，p<0.001）和血红蛋白（14.4 与 13.1g/dL，p<0.001）较高与未治疗组相比；中位铁（p<0.005）较低，铁蛋白（p<0.005）、红细胞（p<0.005）和 hepcidin（p<0.002）较高与对照组相比。铜代谢的变化伴随着 WD 中铁代谢的变化。抗铜治疗可改善但不能使铁代谢正常化。
Zotero 插件