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2 型神经纤维瘤病患者的生活质量和神经症状:日本全国数据库研究。

Quality of life and neurological symptoms in patients with neurofibromatosis type 2: a national database study in Japan.

机构信息

Department of Public Health and Environmental Medicine, The Jikei University School of Medicine.

出版信息

Environ Health Prev Med. 2024;29:44. doi: 10.1265/ehpm.24-00158.

DOI:10.1265/ehpm.24-00158
PMID:39198186
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11362668/
Abstract

BACKGROUND

This study examined the association between neurological symptoms and quality of life (QoL) in patients with neurofibromatosis type 2 (NF2) using a national database of all patients who newly claimed for medical expense subsidies in Japan from 2015 to 2019.

METHODS

The Japanese Ministry of Health, Labour and Welfare provided access to the "National Database of Designated Intractable Diseases of Japan" containing the "Medical Certificates of Designated Intractable Diseases" of all patients with NF2. The database included information on five items of QoL: "mobility," "self-care," "usual activities," "pain/discomfort," and "anxiety/depression." To examine the association between the presence/absence of neurological symptoms and QoL, multivariable logistic regression analyses were performed, adjusted for potential confounders.

RESULTS

Data from 187 patients (97 females and 90 males; mean (standard deviation) age, 43.1 (17.9) years) were analyzed. Overall, 31% to 55% of patients were recorded as having moderate/severe impairment of QoL. Spinal dysfunction was significantly associated with deterioration of all components of QoL, whereas speech dysfunction and hemiparesis were specifically associated with physical health-related components of QoL. Spinal dysfunction, facial nerve palsy, and age 25-64 years were significantly associated with "anxiety/depression."

CONCLUSIONS

In the present epidemiological study using a national database of NF2 in Japan, spinal dysfunction was significantly associated with deterioration of all components of QoL, including subjective and mental health-related components of QoL, whereas speech dysfunction and hemiparesis were specifically associated with physical health-related components of QoL.

摘要

背景

本研究使用日本从 2015 年至 2019 年所有新申请医疗费用补贴的患者的国家数据库,调查了神经纤维瘤病 2 型(NF2)患者的神经症状与生活质量(QoL)之间的关系。

方法

日本厚生劳动省提供了访问权限,以获取包含所有 NF2 患者的“日本指定难治性疾病国家数据库”中的“指定难治性疾病医疗证明”。该数据库包含五个 QoL 项目的信息:“移动性”、“自理”、“日常活动”、“疼痛/不适”和“焦虑/抑郁”。为了检验是否存在神经症状与 QoL 之间的关联,进行了多变量逻辑回归分析,并对潜在混杂因素进行了调整。

结果

对 187 名患者(97 名女性和 90 名男性;平均(标准差)年龄为 43.1(17.9)岁)的数据进行了分析。总体而言,31%至 55%的患者记录为存在中度/重度 QoL 受损。脊柱功能障碍与所有 QoL 成分的恶化显著相关,而言语功能障碍和偏瘫与身体健康相关的 QoL 成分具体相关。脊柱功能障碍、面神经瘫痪和 25-64 岁年龄与“焦虑/抑郁”显著相关。

结论

在本研究中,使用日本 NF2 的国家数据库进行了一项流行病学研究,结果表明脊柱功能障碍与所有 QoL 成分的恶化显著相关,包括主观和心理健康相关的 QoL 成分,而言语功能障碍和偏瘫与身体健康相关的 QoL 成分具体相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b242/11362668/3b4212aed96f/ehpm-29-044-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b242/11362668/3b4212aed96f/ehpm-29-044-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b242/11362668/3b4212aed96f/ehpm-29-044-g001.jpg

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本文引用的文献

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2
Loss of social independence in patients with neurofibromatosis type 2: a follow-up study using a national registry in Japan.神经纤维瘤病 2 型患者社会独立性丧失:一项使用日本全国登记处的随访研究。
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3
Health-related quality of life in patients with neurofibromatosis 1 in Japan: A questionnaire survey using EQ-5D-5L.
日本1型神经纤维瘤病患者的健康相关生活质量:一项使用EQ-5D-5L的问卷调查
J Dermatol. 2022 Dec;49(12):1228-1232. doi: 10.1111/1346-8138.16510. Epub 2022 Jul 3.
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Social Independence of Patients with Neurofibromatosis Type 2 in Japan: Analysis of a National Registry of Patients Receiving Medical Expense Subsidies, 2004-2013.日本 2 型神经纤维瘤病患者的社会独立状况:2004-2013 年接受医疗费用补贴的患者国家登记分析。
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Evaluation of QoL in neurofibromatosis patients: a systematic review and meta-analysis study.神经纤维瘤病患者生活质量评估:系统评价和荟萃分析研究。
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