Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Molecular Genetics Department, |
RCV003985957 | SCV004801769 | likely pathogenic | CHARGE syndrome | criteria provided, single submitter | clinical testing | A previously undescribed nucleotide variant creates a premature translation stop signal p.Glu2837Ter in the CHD7 gene. The variant was observed in heterozygous state in a newborn with congenital heart defect. Loss-of-function variants are reported in patients with CHARGE syndrome, 214800. The variant is not present in population database (gnomAD no frequency). In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as likely pathogenic. |