Lantos John D
Department of Pediatrics, Mt Sinai School of Medicine, New York, NY 10029, USA.
Children (Basel). 2023 Jun 30;10(7):1140. doi: 10.3390/children10071140.
Genome sequencing (GS) provides exciting opportunities to rapidly identify a diagnosis in critically ill newborns and children with rare genetic conditions. Nevertheless, there are reasons to remain cautious about the use of GS. Studies to date have been mostly in highly selected populations of babies with unusual clinical presentations. GS leads to diagnoses in many such infants. More rarely, it leads to beneficial changes in management. Parents and physicians whose babies meet these criteria and for whom GS is performed both find these results useful. The concern is this: we do not know how useful such testing will be in the general population. We can speculate that a number of problems will arise as the use of GS expands. First, the percentage of cases in which a valid molecular diagnosis is made will likely go down. The number of ambiguous results or false positives will rise. Genetic counseling will become more complex and challenging. We do not know the relative cost-effectiveness of whole genome, whole exome, or targeted panels in different populations. We do not know the relative contribution of a molecular diagnosis to the decision to withdraw life support. We will have to carefully evaluate the use of such testing in order to understand whether it truly improves outcome and survival or reduces symptoms in babies who are tested. Each of these concerns will require careful study of both the technology and the ethical issues to allow us to harness the potential of these new technologies while avoiding foreseeable problems. Studies are underway to see how the tests are used in general populations. These studies should generate important information to guide clinicians and policymakers. As part of informed consent, doctors should explain to parents that genetic results are not always straightforward. Sometimes, they confirm a diagnosis that was already suspected. Sometimes, they rule out a possible diagnosis. Sometimes, the results are ambiguous and difficult to interpret. Anticipatory discussions should try to give parents a realistic understanding of the likely impact of a genetic diagnosis. Diagnostic genomic testing for newborns is a science that is still in its infancy. More research is essential in order to establish how to personalize this promising but sometimes problematic tool.
基因组测序(GS)为快速诊断患有罕见遗传疾病的危重新生儿和儿童提供了令人兴奋的机会。然而,对于GS的使用仍有理由保持谨慎。迄今为止的研究大多针对临床表现异常的高度特定婴儿群体。GS在许多此类婴儿中得出了诊断结果。更罕见的是,它导致了治疗管理上的有益改变。符合这些标准并接受GS检测的婴儿的父母和医生都认为这些结果有用。问题在于:我们不知道这种检测在普通人群中会有多大用处。我们可以推测,随着GS使用范围的扩大,会出现一些问题。首先,做出有效分子诊断的病例百分比可能会下降。模糊结果或假阳性的数量将会增加。遗传咨询将变得更加复杂且具有挑战性。我们不知道全基因组、全外显子组或靶向基因panel在不同人群中的相对成本效益。我们不知道分子诊断对撤除生命支持决策的相对贡献。我们必须仔细评估这种检测的使用情况,以了解它是否真的能改善接受检测婴儿的结局和存活率或减轻症状。这些问题中的每一个都需要对技术和伦理问题进行仔细研究,以便我们在避免可预见问题的同时充分利用这些新技术的潜力。正在进行研究以观察这些检测在普通人群中的使用情况。这些研究应能产生重要信息以指导临床医生和政策制定者。作为知情同意的一部分,医生应向父母解释基因检测结果并非总是一目了然。有时,它们证实了原本怀疑的诊断。有时,它们排除了一种可能的诊断。有时,结果模糊且难以解释。前瞻性讨论应努力让父母对基因诊断可能产生的影响有现实的认识。新生儿诊断性基因组检测仍是一门尚处于起步阶段的科学。为了确定如何将这个有前景但有时存在问题的工具个性化,更多的研究至关重要。