Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000611595 | SCV000729738 | benign | not specified | 2017-12-06 | criteria provided, single submitter | clinical testing | This variant is considered likely benign or benign based on one or more of the following criteria: it is a conservative change, it occurs at a poorly conserved position in the protein, it is predicted to be benign by multiple in silico algorithms, and/or has population frequency not consistent with disease. |
Center of Genomic medicine, |
RCV000627056 | SCV000747764 | risk factor | Aganglionic megacolon | 2017-11-20 | criteria provided, single submitter | clinical testing | This variant was identified in a patient with Hirschsprung disease and a positive familial history. The patient harbours also a variant in the SEMA3D gene, which is a VUS for this disease. |
Labcorp Genetics |
RCV001515285 | SCV001723323 | benign | Multiple endocrine neoplasia, type 2 | 2024-01-22 | criteria provided, single submitter | clinical testing | |
Breakthrough Genomics, |
RCV004717904 | SCV005317090 | benign | not provided | criteria provided, single submitter | not provided | ||
OMIM | RCV000014980 | SCV000035236 | risk factor | Hirschsprung disease, susceptibility to, 1 | 2010-07-09 | no assertion criteria provided | literature only | |
Foundation for Research in Genetics and Endocrinology, |
RCV000014980 | SCV001167343 | risk factor | Hirschsprung disease, susceptibility to, 1 | 2020-02-19 | no assertion criteria provided | clinical testing | A heterozygous variant c.73+9277T>C in intron 1 of the RET gene was detected. Presence of this allele has been identified to increase risk of Hirschprung disease by 5.7X compared to people who don't carry the variant (Virtanen et al. EJHG 2019). The allele frequency for the variant is 0.774 as given in gnomAD genomes. In summary, the variant meets our criteria to be classified as a risk factor for Hirschprung disease. |