铁和锌状态对小儿肝豆状核变性预后的影响。

The effects of iron and zinc status on prognosis in pediatric Wilson's disease.

机构信息

Department of Pediatric Gastroenterology, Hepatology and Nutrition, İnönü University, Faculty of Medicine, Malatya,Turkey.

Department of Pediatric Neurology, İnönü University, Faculty of Medicine, Malatya,Turkey.

出版信息

J Trace Elem Med Biol. 2019 Sep;55:33-38. doi: 10.1016/j.jtemb.2019.05.010. Epub 2019 May 24.

DOI:10.1016/j.jtemb.2019.05.010

PMID:31345362

Abstract

OBJECTIVES

Wilson's disease (WD) is a metabolic disorder leading to hepatic and extrahepatic copper deposition. Several studies have reported that besides copper (Cu), iron (Fe) and zinc (Zn) are also accumulated at varying levels in various tissues in WD. However, there is not an adequate number of studies investigating the effects of Fe and Zn status on WD presentation and prognosis. We aimed to evaluate serum levels of ferritin (SFr), copper (SCu), and zinc (SZn) in WD and determine their role in disease presentation and prognosis.

MATERIALS-METHOD: We retrospectively reviewed the medical records of 97 pediatric patients with WD who were diagnosed and followed at İnönü University Pediatric Gastroenterology, Hepatology and Nutrition Department between January 2006 and May 2017. Serum Cu and Zn levels were analyzed by using flame atomic absorption spectrophotometer. Ferritin was analyzed by chemiluminescence immunoassay method.

RESULTS

Analysis of serum levels of the elements according to the type of presentation, there was no significant difference between the groups for ceruloplasmin. However, SCU, FSCu, SFr and 24 h urinary copper levels were significantly higher (p = 0.002, p = 0.003, p = 0.023 and p < 0.001, respectively) and SZn and CSZn levels were significantly lower (fulminant WD). p < 0.001, p < 0.001). There was a positive correlation between SFr, SCu serum levels and mortality scores (respectively, r: 0.501, 0.564 for PELD, r:0.490, 0.504 for MELD, r: 0.345, 0.374 for Dhwan), and a negative correlation between SZn level and mortality scores. (r:-0.650 for PELD, r:-0.703 for MELD, r:-0.642 for Dhwan) We used the ROC curves to determine the worst prognosis for fulminant Wilson disease. According to these limit values, we found that the sensitivity and specificity of FWD development was significantly higher. (for SZn sensitivity of 91.5%, a specificity of 100%, p=<0,001, for SCu predicted FWD development with a sensitivity of 100%, a specificity of 73.7%, p=<0,001, for SFr predicted FWD development with a sensitivity of 92.9%, a specificity of 66.2%, p < 0,001) CONCLUSION: Our study suggests that SFr, SCu, SZn levels might have prognostic importance for WD.

摘要

目的

威尔逊病（WD）是一种导致肝和肝外铜沉积的代谢紊乱。几项研究报告称，除了铜（Cu）外，铁（Fe）和锌（Zn）在 WD 中的各种组织中也以不同水平积累。然而，没有足够数量的研究调查铁和锌状态对 WD 表现和预后的影响。我们旨在评估 WD 患者血清铁蛋白（SFr）、铜（SCu）和锌（SZn）水平，并确定其在疾病表现和预后中的作用。

材料-方法：我们回顾性分析了 2006 年 1 月至 2017 年 5 月期间在因纽大学儿科胃肠病学、肝病学和营养科诊断和随访的 97 例 WD 儿科患者的病历。使用火焰原子吸收分光光度计分析血清 Cu 和 Zn 水平。用化学发光免疫分析法分析铁蛋白。

结果

根据表现类型分析元素血清水平，各组间铜蓝蛋白无显著差异。然而，SCu、FSCu、SFr 和 24 小时尿铜水平显著升高（p=0.002、p=0.003、p=0.023 和 p<0.001，分别），SZn 和 CSZn 水平显著降低（暴发性 WD）。p<0.001，p<0.001）。SFr、SCu 血清水平与死亡率评分呈正相关（分别为 PELD 的 r：0.501、0.564，MELD 的 r：0.490、0.504，Dhwan 的 r：0.345、0.374），SZn 水平与死亡率评分呈负相关。（PELD 的 r：-0.650，MELD 的 r：-0.703，Dhwan 的 r：-0.642）我们使用 ROC 曲线来确定暴发性威尔逊病的最差预后。根据这些限值，我们发现暴发性 WD 发生的敏感性和特异性显著更高。（对于 SZn，发展 FWD 的敏感性为 91.5%，特异性为 100%，p<0.001，对于 SCu，预测 FWD 发展的敏感性为 100%，特异性为 73.7%，p<0.001，对于 SFr，预测 FWD 发展的敏感性为 92.9%，特异性为 66.2%，p<0.001）