1 Matching Annotations
  1. Last 7 days
    1. 8

      Patient8: Female, 41yo at diagnosis, 48yo at ERT

      CasePresentingHPOs:

      HPOsFreeText: anti rh-GAA antibodies titre = 1:1600, enzyme (μmol/g/h) = 0.23 (2-10), mobility aid required before and after ERT

      CaseNotHPOs:

      NotHPOsFreeText: NIV not required

      CaseAddInfo: 7yr disease duration, variants in trans

      CasePMIDs:

      CasePreviousTesting: same as group

      CaseGenotypingMethod: same as group

      Variant1: NM_000152.3:c.482_483del

      CAID: CA8814879, http://reg.clinicalgenome.org/redmine/projects/registry/genboree_registry/by_caid?caid=CA8814879

      gnomAD: 1.729e-05, http://exac.broadinstitute.org/variant/17-78078864-TCC-T

      Variant2: NM_000152.4(GAA):c.692+5G>T

      ClinVar: 562532, https://www.ncbi.nlm.nih.gov/clinvar/variation/526532/

      gnomAD: 8.596e-06, http://exac.broadinstitute.org/variant/17-78079698-G-T