ELLIS-VAN CREVELD SYNDROME: CLINICAL EXOME PANEL (Sec. & CNVs) - 2 genes
LabGenetics
Delivery time
3-4 weeks
Sample
Blood
Ellis-van Creveld syndrome is a congenital chondroectodermal dysplasia characterized by disproportionate dwarfism, postaxial polydactyly, ectodermal dysplasia (abnormal nails and teeth), and congenital heart defects. It is primarily caused by pathogenic variants in the EVC and EVC2 genes, which encode essential proteins in the Hedgehog signaling pathway, critical for bone, dental, and cardiac development. This panel allows for confirmation of the clinical diagnosis, definition of the autosomal recessive inheritance pattern, and facilitates genetic counseling for affected families. Genes analyzed: EVC, EVC2.