在日本对儿科遗传性血管的全国性基于问卷的监测
Beverley Anne Yamamoto1,2, Isao Ohsawa3, Shun Toriumi4
1Graduate School of Human Sciences, Osaka University, Osaka, Japan.
概括
儿科遗传性血管炎 (HAE) 在许多日本设施中得到管理,但对有风险的亲属的查往往缺乏. 迫切需要对儿科HAE护理进行标准化.
科学领域:
- 遗传学和免疫学 遗传学和免疫学
- 罕见疾病 罕见疾病
- 儿科医疗保健 儿科医疗保健
背景情况:
- 遗传性血管炎 (HAE) 是一种罕见的遗传性疾病,会导致反复的胀发作.
- 日本儿科HAE病例的流行率和管理状况在很大程度上是未知的.
- 了解儿科HAE对于改善患者的治疗结果和生活质量至关重要.
研究的目的:
- 为了确定日本儿科HAE患者的数量.
- 评估目前对儿科HAE的管理策略.
- 确定儿科HAE护理中未得到满足的需求和挑战.
主要方法:
- 一项全国性的邮件调查问卷调查分发给了142名在HAE管理方面有经验的临床医生.
- 调查项目的重点是儿科HAE患者数量,发作史,生活质量影响,未经测试的亲属和未满足的需求.
- 分析了来自69所受访机构的数据 (49%的受访率).
主要成果:
- 25个机构 (36%的受访者) 报告说他们管理儿科HAE患者,其中大多数只管理一个病例.
- 在响应的设施中,共有26名儿科HAE患者被确定.
- 19个机构 (28%) 报告说,儿科亲属犹不决接受查,突出了早期诊断和预防方面的重大差距.
结论:
- 日本的儿科HAE管理目前是分散的,分散在许多设施.
- 相当多的HAE患者的儿科亲属没有接受查,这表明他们错过了早期干预的机会.
- 这项研究强调了日本迫切需要标准化儿科HAE管理协议和专业护理途径,以解决确定的未满足需求.
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