Department of Paediatrics, Midland Regional Hospital, Mullingar, Ireland
Department of Paediatrics, Midland Regional Hospital, Mullingar, Ireland.
BMJ Case Rep. 2022 Nov 8;15(11):e251871. doi: 10.1136/bcr-2022-251871.
We report the case of a boy with a prolonged diagnostic workup for global developmental delay alongside feeding difficulties, failure to thrive, pulmonary stenosis and macrocephaly. Following a series of diagnostic tests over the first 25 months of life, whole-exome sequencing was performed which diagnosed cardiofaciocutaenous syndrome type 3.Global developmental delay is a common presentation to general paediatric and community paediatric clinics. This prompts the search for an aetiology to describe the child's constellation of symptoms which often consists of a chromosomal microarray, neuroimaging and investigations for an inborn error of metabolism. With developments in genetic testing such as the reducing cost of clinical exome sequencing or whole-exome sequencing, could these testing strategies offer a more comprehensive first line test?This case not only demonstrates the features of cardiofaciocutaneous syndrome type 3 but the added value of modern genetic technologies in the diagnosis of children with global developmental delay.
我们报告了一例患有全球发育迟缓、喂养困难、生长不良、肺动脉瓣狭窄和大头畸形的男孩病例。在生命的头 25 个月内进行了一系列诊断性检查后,进行了全外显子组测序,诊断为 3 型心面皮肤综合征。全球发育迟缓是普通儿科和社区儿科诊所的常见表现。这促使人们寻找病因来描述孩子的一系列症状,这些症状通常包括染色体微阵列、神经影像学检查和先天性代谢缺陷的检查。随着基因检测技术的发展,如临床外显子组测序或全外显子组测序成本的降低,这些检测策略是否可以提供更全面的一线检测?这个病例不仅展示了 3 型心面皮肤综合征的特征,还展示了现代遗传技术在诊断全球发育迟缓儿童方面的附加价值。