Ghannad-Zadeh Kimia, Shannon Patrick, Jobling Rebekah, Miller Elka, Chong Karen, Mathews Erin, Chitayat David, Shinar Shiri
Temerty Faculty of Medicine, University of Toronto, Toronto, Canada.
Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, University of Toronto, Toronto, Canada.
Prenat Diagn. 2025 Jun;45(6):825-828. doi: 10.1002/pd.6804. Epub 2025 May 2.
KIDINS220 plays a key role in neuronal survival, differentiation, and synaptic function. Abnormalities in its expression have been linked postnatally to neurodevelopmental disorders and SINO syndrome though prenatal presentations are rarely described. We report a novel de novo heterozygous KIDINS220 variant identified prenatally associated with bilateral ventriculomegaly, abnormal anterior horns, aqueductal stenosis, and a hypoplastic corpus callosum. This is the first prenatal case of such findings in KIDINS220, emphasizing the value of trio WES/WGS for diagnosis and counseling.
KIDINS220在神经元存活、分化及突触功能中起关键作用。其表达异常在出生后与神经发育障碍和SINO综合征相关,不过产前表现鲜有描述。我们报告了一种新的产前鉴定出的KIDINS220杂合变异,与双侧脑室扩大、前角异常、导水管狭窄及胼胝体发育不全有关。这是KIDINS220相关此类发现的首例产前病例,强调了三联体全外显子组测序/全基因组测序在诊断和咨询中的价值。