deamino-arginine-vasopressin and Factor-VII-Deficiency

Topics: Aminocaproic Acid; Blood Transfusion; Deamino Arginine Vasopressin; Edema; Factor VII Deficiency; Factor VIIa; Hemostatics; Humans; Liver Cirrhosis, Alcoholic; Male; Middle Aged; Postoperative Hemorrhage; Recombinant Proteins; Shock; Tooth Extraction; Vitamin K

Topics: Aged; Blood Transfusion; Deamino Arginine Vasopressin; Factor V Deficiency; Factor VII Deficiency; France; Humans; Male; Plasma; Preoperative Care; Transurethral Resection of Prostate

We describe the management of tonsil or tonsil and adenoid surgery in our hospital in 10 patients with inherited bleeding disorders, over a 10-year-period. The approach to the management of the haemostatic defect is outlined in detail. All underwent successful surgery, two patients had limited secondary haemorrhage. This report demonstrates that patients with inherited bleeding disorders can safely undergo adenotonsillectomy, providing there is close liaison between surgeons and haematologists throughout the peri-operative period.

Topics: Adenoidectomy; Blood Coagulation Disorders; Child; Child, Preschool; Deamino Arginine Vasopressin; Factor VII Deficiency; Female; Hemophilia A; Hemostasis, Surgical; Humans; Male; Tonsillectomy; Tranexamic Acid; von Willebrand Diseases

We report a family with a combined factor VII Padua defect and von Willebrand's disease (vWd). The propositus is a 9-year-old child with a moderate bleeding tendency who appeared to be heterozygous for both factor VII Padua and type I vWd. The diagnosis of factor VII Padua was based on a normal factor VII antigen and factor VII activity which was low with rabbit brain thromboplastin but normal with ox brain thromboplastin. Type I vWd was diagnosed because of a concomitant decrease of von Willebrand factor antigen (vWf:Ag) and vWf ristocetin-cofactor activity (vWf:RCoF), associated with the presence of vWf multimers of all sizes in plasma and platelets. The parents were not consanguineous but came from the same isolated river Piave valley in North Eastern Italy where the factor VII Padua defect was first described. The father had the factor VII Padua defect but was clinically asymptomatic in accordance with the heterozygous state. The propositus's mother had type I vWd and was mildly symptomatic. The propositus' sisters, who were clinically asymptomatic, were both heterozygotes for factor VII Padua. The infusion of DDAVP normalized the factor VIII/vWf pattern in all patients. In the propositus, in contrast to the mother and normal subjects, showed a more rapid clearance both of vWf and factor VIII. The same pattern, albeit to a lesser degree, was also observed in the father.

Topics: Child; Deamino Arginine Vasopressin; Factor VII; Factor VII Deficiency; Female; Humans; Italy; Male; Pedigree; Thromboplastin; von Willebrand Diseases