对组织铜水平的分析作为儿科肝病的可靠诊断工具
Zohra Najimi1, Loubna ElSaboni2, Marumbo Mtegha3
1University of Manchester, Manchester, UK.
European journal of pediatrics
|December 17, 2025
概括
组织铜水平可靠地诊断儿童的威尔逊病 (WD),水平≥250μg/g表明WD. 低烯和脂肪酸有助于诊断铜含量较低的病例.
科学领域:
- 儿科肝病学 儿科肝病学
- 诊断的准确性 诊断的准确性
- 生物化学标记物 生物化学标记物
背景情况:
- 提升的组织铜是威尔逊病 (WD) 的诊断标志物.
- 肝脏铜水平的诊断截止值仍在争论中,因为在其他儿科肝脏疾病中也可能出现升高水平.
- 准确诊断儿童的WD对于及时干预至关重要.
研究的目的:
- 评估维尔森病 (WD) 儿科肝脏活检中组织铜水平的诊断准确性.
- 为了确定可靠的切断值组织铜在诊断儿童WD.
- 评估其他生化标志物和组织病理学在WD诊断中的作用.
主要方法:
- 从2012年6月到2021年10月的儿科肝脏活检数据 (儿童≤18岁) 的回顾性分析.
- 测量肝脏组织铜,血清血,24小时尿液铜.
- 使用曼-惠特尼U测试来比较WD和非威尔逊肝病 (NWLD) 组之间的铜水平.
主要成果:
- 患有WD的儿童的组织铜水平明显高于NWLD的儿童 (p < 0.0001).
- 组织铜含量≥250μg/g可靠地预测了23例未经治疗的WD病例中的22例的WD.
- 与NWLD (25%) 相比,WD (39%) 的肥胖症更为普遍,大多数NWLD病例患有与代谢功能障碍相关的肥胖性肝病.
结论:
- 组织铜≥250μg/g的切断值是诊断儿童威尔逊病的可靠指标.
- 低血清甲素和肥胖症的存在是诊断WD时需要考虑的重要参数,当组织铜水平低于250μg/g时.
- 组织铜水平应与组织病理学和生物化学一起解释,以便在儿童中全面诊断WD.
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