中华皮肤科杂志 ›› 2020, Vol. 53 ›› Issue (2): 136-138.doi: 10.35541/cjd.20190293

• 调查报告 • 上一篇    下一篇

遗传性大疱性表皮松解症367例问卷调查

陈劼1    吕军2    周迎春3    卢发燕3   

  1. 1复旦大学附属儿科医院儿童伤口造口护理门诊，上海  201102；2复旦大学公共卫生学院，上海  200032；3中国蝴蝶宝贝关爱中心，上海  200000
  • 收稿日期:2019-02-13 修回日期:2019-08-19 发布日期:2020-02-01
  • 通讯作者: 吕军 E-mail:lujun@shmu.edu.cn
  • 基金资助:
    上海市卫生和计划生育委员会科研基金（201840102）

A questionnaire survey in 367 patients with hereditary epidermolysis bullosa

Chen Jie1, Lyu Jun2, Zhou Yingchun3, Lu Fayan3   

  1. 1Pediatric Wound Ostomy Care Clinic, Children′s Hospital of Fudan University, Shanghai 201102, China; 2School of Public Health, Fudan University, Shanghai 200032, China; 3DebRA China, Shanghai 200000, China
  • Received:2019-02-13 Revised:2019-08-19 Published:2020-02-01
  • Contact: Lyu Jun E-mail:lujun@shmu.edu.cn
  • Supported by:
    Research Program of Shanghai Municipal Health Commission （201840102）

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摘要： 【摘要】 目的 调查中国遗传性大疱性表皮松解症（EB）患者的护理负担、医疗保障和合并症等情况。方法 2018年9 - 12月，对2013年1月至2018年9月在蝴蝶宝贝关爱中心登记的EB患者进行网络/电话问卷调查。结果 本次调查招募到377例EB患者或其家长参加，其中有效问卷367份，已登记的EB患者中未成年人占74.9%（275例）。每月伤口护理成本低于3 000元的患者占92%（319例），57.5%（211例）的患者报告医疗费主要靠自费。患者分布于全国的30个省、自治区，直辖市，仅西藏没有登记患者。其中居住地登记人数最多的是江苏省（49例），其次为河南（35例）、广东（27例）、河北（23例）及山东（20例）。367例患者中，168例接受基因检测。EB患者最常见的合并症是指甲脱落及瘙痒，发生率分别达66.2%和55.3%。122例（33.2%）发生残疾，其中肢体残疾占27.8%（102例）。结论 EB患者大部分得不到适宜的诊断、护理和医疗保障，合并症和残疾多发，生活质量低下。

关键词: 大疱性表皮松解, 横断面研究, 残疾人, 合并症

Abstract: 【Abstract】 Objective To investigate the nursing care burden, medical assurance and comorbidities of patients with hereditary epidermolysis bullosa （EB） in China. Methods From September to December in 2018, a cross-sectional telephone/internet-based questionnaire survey was conducted in EB patients registered at DebRA China between January 2013 and September 2018. Results Totally, 377 participants （EB patients or their parents） were enrolled into this survey, and a total of 367 valid questionnaires were collected. Of the 367 registered patients with EB, 275 （74.9%） were minors, and 319 （92%） reported wound care costs per month less than 3 000 RMB Yuan, and 211 （57.5%） reported that medical expenses were mainly paid by themselves. These patients were distributed in 30 provinces, autonomous regions or municipalities directly under the central government of China, and no patient was from Tibet. The largest number of registered patients was from Jiangsu province （49/367）, followed by Henan （35/367）, Guangdong （27/367）, Hebei （23/367） and Shandong （20/367） provinces. Of the 367 patients, 168 received genetic testing. The most common comorbidities of EB patients were nail peeling and itching, with the prevalence rate being 66.2% and 55.3% respectively. Disabilities occurred in 122 （33.2%） patients, 102 （27.8%） of which had physical disabilities. Conclusion Most of EB patients cannot obtain appropriate diagnosis, nursing care and medical assurance, comorbidities and disabilities are common, and their quality of life is very low.

Key words: Epidermolysis bullosa, Cross-sectional studies, Disabled person, Comorbidity

引用本文

陈劼 吕军 周迎春 卢发燕. 遗传性大疱性表皮松解症367例问卷调查[J]. 中华皮肤科杂志, 2020,53(2):136-138. doi:10.35541/cjd.20190293

Chen Jie, Lyu Jun, Zhou Yingchun, Lu Fayan. A questionnaire survey in 367 patients with hereditary epidermolysis bullosa[J]. Chinese Journal of Dermatology, 2020, 53(2): 136-138.doi:10.35541/cjd.20190293