Preferred Label : Hereditary factor VII deficiency;
ICD-11 definition : Factor VII (FVII) deficiency is a rare hereditary hemorrhagic disease caused by the
diminution or absence of this coagulation factor. The clinical expression of this
disorder is highly variable, and no consistent relationship has been found between
the severity of the hemorrhagic syndrome and the residual levels of FVII activity.;
ICD-11 synonym : Congenital proconvertin deficiency; proconvertin deficiency; Hypoproconvertinaemia;
ICD-11 inclusion : factor VII deficiency; hereditary factor VII deficiency syndrome; acquired deficiency of proconvertin; Deficiency of factor VII; hereditary factor VII deficiency disease; congenital factor VII deficiency; hereditary hypoproconvertinaemia;
Origin ID : 2034923927;
Automatic exact mappings (from CISMeF team)
Factor VII (FVII) deficiency is a rare hereditary hemorrhagic disease caused by the
diminution or absence of this coagulation factor. The clinical expression of this
disorder is highly variable, and no consistent relationship has been found between
the severity of the hemorrhagic syndrome and the residual levels of FVII activity.
