Suppr超能文献

通过新生儿筛查鉴定 5q 型脊髓性肌萎缩症患者的综合治疗方法和实用建议。

Integrated Approaches and Practical Recommendations in Patient Care Identified with 5q Spinal Muscular Atrophy through Newborn Screening.

机构信息

Newborn Screening Reference Center, Instituto Jô Clemente (IJC), São Paulo 04040-033, Brazil.

Department of Neurology, Faculdade de Medicina, Universidade de São Paulo (FMUSP), São Paulo 05403-010, Brazil.

出版信息

Genes (Basel). 2024 Jun 29;15(7):858. doi: 10.3390/genes15070858.

Abstract

In recent years, significant progress has been made in 5q Spinal Muscular Atrophy therapeutics, emphasizing the importance of early diagnosis and intervention for better clinical outcomes. Characterized by spinal cord motor neuron degeneration, 5q-SMA leads to muscle weakness, swallowing difficulties, respiratory insufficiency, and skeletal deformities. Recognizing the pre-symptomatic phases supported by screening and confirmatory genetic tests is crucial for early diagnosis. This work addresses key considerations in implementing 5q-SMA screening within the Brazilian National Newborn Screening Program and explores Brazil's unique challenges and opportunities, including genetic tests, time-to-patient referral to specialized centers, program follow-up, and treatment algorithms. We aim to guide healthcare professionals and policymakers, facilitating global discussions, including Latin American countries, and knowledge-sharing on this critical subject to improve the care for newborns identified with 5q SMA.

摘要

近年来,5q 型脊肌萎缩症的治疗取得了重大进展,强调了早期诊断和干预对于获得更好临床结果的重要性。5q-SMA 以脊髓运动神经元变性为特征,导致肌肉无力、吞咽困难、呼吸功能不全和骨骼畸形。通过筛查和确认性遗传测试来识别有症状前阶段至关重要。这项工作解决了在巴西国家新生儿筛查计划中实施 5q-SMA 筛查的关键问题,并探讨了巴西独特的挑战和机遇,包括遗传测试、将患者转诊至专门中心的时间、计划随访和治疗算法。我们旨在为医疗保健专业人员和政策制定者提供指导,促进全球讨论,包括拉丁美洲国家,并就这一关键主题进行知识共享,以改善对被诊断为 5q-SMA 的新生儿的护理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4015/11276409/950d28384e06/genes-15-00858-g001.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验