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)

Summary

The effect of a single standard dose of intranasal DDAVP (260 μg) 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.

Keywords

1-Deamino-8-D-arginine-vasopressin - Factor VIII/von Willebrand factor - Combined deficiency of Factors V and VIII

• References

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