Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Molecular Genetics Laboratory, |
RCV004795255 | SCV005415400 | pathogenic | KBG syndrome | 2024-11-27 | criteria provided, single submitter | clinical testing | This variant was detected in a male with intellectual disability, short stature, facial dysmorphism. The variant was confirmed to be of a de novo origin. Rare variants affecting the ANKRD11 gene are documented as a molecular cause of autosomal dominant "KBG syndrome" (OMIM:148050) (PMID:21782149;29258554;25125236;25413698). To conclude, the variant is classified as pathogenic (ACMG PVS1, PM2, PS2). |