Parent Project Muscular Dystrophy, Washington, District of Columbia, USA.
Am J Med Genet C Semin Med Genet. 2022 Jun;190(2):162-168. doi: 10.1002/ajmg.c.31993. Epub 2022 Aug 5.
Duchenne muscular dystrophy (DMD) is a progressive, fatal neuromuscular disorder typically diagnosed between 4 and 5 years of age. DMD currently has five FDA approved therapies, which has led to increased interest in newborn screening (NBS) for DMD. Our objective was to explore the perspectives and predicted practices of physicians (primarily neurologists) who will likely be responsible for the follow-up of infants identified with DMD through NBS. A short survey was developed and distributed to physicians who are responsible for providing care for patients with Duchenne at Certified Duchenne Care Centers across the USA. Twenty-seven physicians responded to statements about benefit and readiness for dystrophinopathy NBS, which care recommendations they would make at initial infant visits, and when they would recommend initiating approved therapies. Most DMD physicians indicated they see benefit in NBS (82%) and believe the DMD care community is ready for NBS in dystrophinopathies (74%). The majority of physicians would recommend multiple interventions, including genetic counseling, maternal carrier testing, referral to early intervention services, screening siblings, discussion of clinical trials, exon skipping therapies, and assessment of social and language development at initial visits. The majority of physicians also indicated they would recommend initiating approved therapies much earlier than the typical age of diagnosis.
杜氏肌营养不良症(DMD)是一种进行性、致命的神经肌肉疾病,通常在 4 至 5 岁之间确诊。目前已有五种 FDA 批准的治疗方法,这使得人们对 DMD 的新生儿筛查(NBS)产生了更大的兴趣。我们的目的是探讨可能负责通过 NBS 对 DMD 患儿进行随访的医生(主要是神经科医生)的观点和预测实践。为此,我们开发并向美国各地的认证杜氏肌营养不良症护理中心负责照顾杜氏肌营养不良症患者的医生分发了一份简短的调查问卷。有 27 名医生对关于肌营养不良症 NBS 的获益和准备情况、他们将在初次婴儿就诊时提出哪些护理建议以及何时建议开始使用批准的治疗方法的陈述做出了回应。大多数 DMD 医生表示他们认为 NBS 具有益处(82%),并认为 DMD 护理界已经为肌营养不良症 NBS 做好了准备(74%)。大多数医生会建议多种干预措施,包括遗传咨询、母亲携带者检测、转介早期干预服务、筛查兄弟姐妹、讨论临床试验、外显子跳跃治疗,以及在初次就诊时评估社会和语言发育情况。大多数医生还表示,他们会建议更早地开始使用批准的治疗方法,而不是典型的诊断年龄。