[Gastrectomy in congenital factor VII deficiency with substitution of factor VII concentrate].
Case report of a 57-year old man, who had a severe congenital factor VII deficiency (factor VII 2%), and who was substituted with factor VII concentrate, before undergoing a subtotal gastrectomy. The patient was previously asymptomatic. The operation and wound-healing was without complication. That the patient, despite his factor VII deficiency, showed no clinical signs of a bleeding diathesis, raises the question, whether in such a case a factor VII substitution is necessary. 3 children of the patient (2 sons aged 25 and 23 years, and an 18 year old daughter) were examined and found to be heterozygote carriers to a degree of 32%, 42% and 60% factor VII; none showed sure signs of a bleeding tendency related to factor VII deficiency.
D000293:Adolescent; D000328:Adult; D005167:Factor VII / Q000008:administration & dosage*; D005168:Factor VII Deficiency / Q000151:congenital* / Q000235:genetics; D005260:Female; D005743:Gastrectomy; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged
D005167:Factor VII
Gastrektomie bei kongenitalem Faktor-VII-Mangel unter Substitution von Faktor-VII-Konzentrat.