title: Response of factor VIII/von Willebrand factor to intranasal DDAVP in healthy subjects and mild haemophiliacs (with observations in patients with combined deficiency of factors V and VIII).
abstract: The effect of a single standard dose of intranasal DDAVP (260 micrograms) was investigated in healthy subjects and in patients with mild deficiencies of VIII:C. Changes in FVIII/VWF activities were measured from baseline after 30, 60, 120 and 360 min of administration of the drug. Intranasal DDAVP was followed by a two-fold increase of VIII:C in both groups studied. VIIIR:AG and VIIIR:RCo increased to a lesser extent. Even though FVIII/VWF activities reached their maximum after 60-120 min, a significant increase over baseline was still observed after 360 min. The rise of VIII:C was unrelated to the body weight of the patients and was proportional to the baseline levels of this factor. In two sisters with combined deficiencies of FV/FVIII, the responses in all activities of FVIII/VWF were similar to those seen in mild hemophiliacs. Factor V did not undergo any variation. No alteration in serum osmolarity and no consistent variation in blood pressure or pulse rate were noted. It is concluded that the i.n. administration of a single high dose of DDAVP might be adopted to provide an emergency aid in bleeding patients with mild to moderate haemophilia A and to yield higher VIII:C levels in blood donors.
mesh_terms: D000281:Administration, Intranasal; D000293:Adolescent; D000328:Adult; D001127:Arginine Vasopressin / Q000008:administration & dosage*; D001779:Blood Coagulation Factors / Q000096:biosynthesis*; D001835:Body Weight; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage* / Q000009:adverse effects; D005166:Factor V Deficiency / Q000150:complications / Q000628:therapy; D005169:Factor VIII / Q000032:analysis / Q000096:biosynthesis*; D005260:Female; D006467:Hemophilia A / Q000150:complications / Q000628:therapy*; D006801:Humans; D008297:Male; D014841:von Willebrand Factor / Q000096:biosynthesis*
chemical_list: D001779:Blood Coagulation Factors; D014841:von Willebrand Factor; D001127:Arginine Vasopressin; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin