诊断时仅肝性威尔逊病患者神经症状的危险因素分析。

Analysis of risk factors for neurological symptoms in patients with purely hepatic Wilson's disease at diagnosis.

机构信息

Department of Neurology, The First Affiliated Hospital, Jinan University, Guangzhou, 610630, Guangdong, China.

Department of Neurology, College of Clinical Medicine, The First Affiliated Hospital, Guangdong Pharmaceutical University, Guangzhou, 510062, Guangdong, China.

出版信息

BMC Neurol. 2023 Feb 28;23(1):89. doi: 10.1186/s12883-023-03105-w.

DOI:10.1186/s12883-023-03105-w

PMID:36855079

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9972690/

Abstract

OBJECTIVE

To analyze and explore the risk factors for neurological symptoms in patients with purely hepatic Wilson's disease (WD) at diagnosis.

METHODS

This retrospective study was conducted at the First Affiliated Hospital of the Guangdong Pharmaceutical University on 68 patients with purely hepatic WD aged 20.6 ± 7.2 years. The physical examinations, laboratory tests, color Doppler ultrasound of the liver and spleen, and magnetic resonance imaging (MRI) of the brain were performed.

RESULTS

The elevated alanine transaminase (ALT) and aspartate transaminase (AST) levels and 24-h urinary copper level were higher in the purely hepatic WD who developed neurological symptoms (NH-WD) group than those in the purely hepatic WD (H-WD) group. Adherence to low-copper diet, and daily oral doses of penicillamine (PCA) and zinc gluconate (ZG) were lower in the NH-WD group than those in the H-WD group. Logistic regression analysis showed that insufficient doses of PCA and ZG were associated with the development of neurological symptoms in patients with purely hepatic WD at diagnosis.

CONCLUSION

The development of neurological symptoms in patients with purely hepatic WD was closely associated with insufficient doses of PCA and ZG, and the inferior efficacy of copper-chelating agents. During the course of anti-copper treatment, the patient's medical status and the efficacy of copper excretion should be closely monitored.

摘要

目的

分析并探讨初诊单纯肝型 Wilson 病（WD）患者发生神经症状的危险因素。

方法

本回顾性研究在广东药科大学附属第一医院进行，共纳入 68 例年龄为 20.6±7.2 岁的初诊单纯肝型 WD 患者。对患者进行体格检查、实验室检查、肝脏和脾脏彩色多普勒超声以及脑部磁共振成像（MRI）检查。

结果

发生神经症状（NH-WD）组的丙氨酸转氨酶（ALT）和天门冬氨酸转氨酶（AST）水平及 24 h 尿铜水平高于单纯肝型 WD（H-WD）组，NH-WD 组低铜饮食依从性及每日驱铜药物（PCA 和 ZG）服用剂量均低于 H-WD 组。Logistic 回归分析显示，PCA 和 ZG 剂量不足与初诊单纯肝型 WD 患者发生神经症状有关。