Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Sydney Genome Diagnostics, |
RCV001328129 | SCV001449221 | uncertain significance | Atypical hemolytic-uremic syndrome | 2018-05-30 | no assertion criteria provided | clinical testing | This patient is heterozygous for a variant of unknown clinical significance (VOUS), c.3530A>T (p.Tyr1177Phe), in the CFH gene. To our knowledge, this variant has not been reported in the literature or in any population databases (i.e. ExAC browser, ESP or dbSNP). In silico analysis (Alamut Visual v2.8.1) is inconclusive regarding this variant, SIFT and MutationTaster predicts it to be likely benign whereas PolyPhen2 predicts it to be possibly pathogenic. Heterozygous variants in CFH have been reported to confer susceptibility or a predisposition to atypical haemolytic uremic syndrome (aHUS) (OMIM134370). The inheritance is typically autosomal dominant and can show incomplete penetrance (Genereviews PMID: 20301541). |