Ricci Federica S, D'Alessandro Rossella, Vacchetti Martina, Salvalaggio Anna, Somà Alessandra, Daffunchio Giorgia, Spada Marco, Turra Renato, Bobbio Marisa, Ciuti Alessandro, Davico Chiara, Vitiello Benedetto, Mongini Tiziana E
Division of Child and Adolescent Neuropsychiatry, Department of Public Health and Pediatric Sciences, University of Turin, 10126 Turin, Italy.
Division of Pediatrics, Department of Public Health and Pediatric Sciences, University of Turin, 10126 Turin, Italy.
Children (Basel). 2022 Jul 17;9(7):1063. doi: 10.3390/children9071063.
Innovative targeted treatments for neuromuscular disorders (NMDs) can dramatically improve the course of illness. Diagnostic delay, however, is a major impediment. Here, we present a pilot project aimed at assessing the feasibility of a screening program to identify children at high risk for NMDs within the first 30 months of life. The Promoting Early Diagnosis for Neuromuscular Disorders (PEDINE) project implemented a three-step sequential screening in an area of about 300,000 people with (1) an assessment of the motor development milestones to identify "red flags" for NMDs by primary care pediatricians (PCPs) as part of the routine Health Status Check visits; (2) for the children who screened positive, a community neuropsychiatric assessment, with further referral of suspected NMD cases to (3) a hospital-based specialized tertiary care center. In the first-year feasibility study, a total of 10,032 PCP visits were conducted, and twenty children (0.2% of the total Health Status Check visits) screened positive and were referred to the community neuropsychiatrist. Of these, four had elevated creatine kinase (CK) serum levels. This pilot study shows that screening for NMDs in primary care settings is feasible and allows children at high risk for muscular disorder to be promptly identified.
针对神经肌肉疾病(NMDs)的创新靶向治疗可显著改善疾病进程。然而,诊断延迟是一个主要障碍。在此,我们介绍一个试点项目,旨在评估一项筛查计划的可行性,该计划用于在生命的前30个月内识别NMDs高危儿童。促进神经肌肉疾病早期诊断(PEDINE)项目在一个约30万人的地区实施了三步序贯筛查:(1)由初级保健儿科医生(PCP)在常规健康状况检查访视中评估运动发育里程碑,以识别NMDs的“红旗征”;(2)对于筛查呈阳性的儿童,进行社区神经精神评估,并将疑似NMDs病例进一步转诊至(3)基于医院的专业三级护理中心。在第一年的可行性研究中,共进行了10,032次PCP访视,20名儿童(占健康状况检查访视总数的0.2%)筛查呈阳性并被转诊至社区神经精神科医生处。其中,4名儿童血清肌酸激酶(CK)水平升高。这项试点研究表明,在初级保健机构中筛查NMDs是可行的,并且能够及时识别出肌肉疾病高危儿童。