日本浅层铁色素沉着症的全国性流行病学调查。

Nationwide epidemiological survey of superficial hemosiderosis in Japan.

机构信息

Saitama Medical University International Medical Center, Department of Neurology and Cerebrovascular Medicine, Hidaka, Saitama, Japan.

出版信息

J Neurol Sci. 2019 Sep 15;404:106-111. doi: 10.1016/j.jns.2019.07.019. Epub 2019 Jul 17.

DOI:10.1016/j.jns.2019.07.019

PMID:31357133

Abstract

BACKGROUND

The Japanese guideline for diagnosis and classification of superficial hemosiderosis (SHS) has recently been published, for which patient medical expenses are supported by the Ministry of Health. We sought to clarify the clinical features, method of diagnosis, and treatment for SHS in Japan.

METHODS

We sent a questionnaire survey to 792 medical institutes of the Japanese Society of Neurology, to collect information about SHS, including patients during 2017.

RESULTS

We received replies from 287 institutes (36.2%). Estimated total number of patients with SHS in 2017 was 129 at 55 institutes. All patients were diagnosed by neurologists. Among 123 patients with available data, 81 patients (63%) had "classical" type (c-SHS), 29 (24%) had "localized" type (l-SHS), and 13 patients (10%) had "atypical" type (a-SHS). Five patients with l-SHS were excluded because of lacking detailed information. There were available data for the cause of SHS in 77 patients (63%): 55 (69%) with c-SHS, 16 (55%) with l-SHS, and 6 (48%) with a-SHS. Pharmacological or surgical treatment was given at 31 institutes. Medical expense subsidies were filed for 41% of patients.

CONCLUSIONS

Using the Japanese guideline for diagnosis of SHS, over 100 patients were confirmed as having SHS with characteristic clinical features. SHS is not a rare clinical condition in Japan.

摘要

背景

日本最近发布了浅表铁性卟啉症（SHS）的诊断和分类指南，该指南规定患者的医疗费用由厚生劳动省承担。我们旨在阐明日本 SHS 的临床特征、诊断方法和治疗方法。

方法

我们向日本神经病学会的 792 家医疗机构发送了一份问卷调查，以收集 2017 年 SHS 患者的信息。

结果

我们收到了 287 家机构（36.2%）的回复。在 55 家机构中，2017 年估计有 129 名 SHS 患者。所有患者均由神经科医生诊断。在 123 名可提供数据的患者中，81 名（63%）为“经典”型（c-SHS），29 名（24%）为“局限性”型（l-SHS），13 名（10%）为“非典型”型（a-SHS）。由于缺乏详细信息，5 名 l-SHS 患者被排除在外。在 77 名（63%）患者中可获得 SHS 病因的数据：55 名（69%）c-SHS，16 名（55%）l-SHS，6 名（48%）a-SHS。31 家机构给予了药物或手术治疗。41%的患者提出了医疗费用补贴申请。