Elenga Narcisse
Pediatric Medicine and Surgery, Hôpital Andrée Rosemon, Rue des flamboyants, BP 6006, 97306 Cayenne Cedex, French Guiana.
Sickle Cell Reference Center, Pediatric Medicine and Surgery, Centre Hospitalier de Cayenne "Andrée Rosemon" Rue de Flamboyants, BP 6006, 97306 Cayenne Cedex, French Guiana.
Int J Pediatr. 2024 Feb 22;2024:8910397. doi: 10.1155/2024/8910397. eCollection 2024.
The primary purpose of newborn screening for sickle cell disease is to diagnose the disease before the appearance of symptoms and to initiate early treatment. To answer the question "What genetic information needs to be communicated to parents when newborn screening reveals the presence of a sickle cell trait," we conducted a survey using a self-administered online questionnaire. We received responses from 122 healthcare workers and members of sickle cell disease associations, in France and French overseas departments. Our results showed similar positions on this issue. The information conveyed is not consistent and is the result of grassroots initiatives. The negative consequences generated by this information could be reduced when this information is delivered by a multidisciplinary team, within the framework of a dedicated consultation. This information on sickle cell trait status should be given in at least three key periods: the neonatal period, early adolescence, and later adolescence, when reproductive implications become important. Neonatal screening programs should develop systems that allow referring physicians to easily access the results of neonatal screening electronically. Harmonization of practices should allow a better analysis of the consequences of this counselling on family projects.
新生儿镰状细胞病筛查的主要目的是在症状出现之前诊断疾病并启动早期治疗。为了回答“当新生儿筛查显示存在镰状细胞特征时,需要向父母传达哪些遗传信息”这一问题,我们使用自行填写的在线问卷进行了一项调查。我们收到了来自法国及法属海外省的122名医护人员和镰状细胞病协会成员的回复。我们的结果显示在这个问题上立场相似。所传达的信息不一致,是基层倡议的结果。当由多学科团队在专门咨询的框架内提供此信息时,该信息产生的负面后果可能会减少。关于镰状细胞特征状态的信息应在至少三个关键时期提供:新生儿期、青春期早期和青春期后期,此时生殖方面的影响变得重要。新生儿筛查项目应开发系统,使转诊医生能够轻松地以电子方式获取新生儿筛查结果。实践的统一应有助于更好地分析这种咨询对家庭计划的影响。