The Hermansky-Pudlak syndrome. Variable reaction to 1-desamino-8D-arginine vasopressin for correction of the bleeding time

Ophthalmic Paediatrics and Genetics
D B van DorpG Vrensen

Abstract

The effect of the synthetic vasopressin derivative 1-desamino-8D-arginine vasopressin (DDAVP = Minrin) on bleeding time was studied in nine patients with Hermansky-Pudlak syndrome; four of them were Dutch, five were Belgian. Shortening of bleeding time was observed in four of the patients with this type of storage pool disease; in one patient the response was equivocal, in two patients the response was not dramatic and in two there was no response at all. DDAVP may be useful in managing the bleeding disorder in some patients with Hermansky-Pudlak syndrome. Therefore, every patient with this syndrome should be tested with DDAVP as a preventive measure.

References

Dec 1, 1972·British Journal of Haematology·R M HardistyK Ketsa-Ard
Aug 1, 1974·The Journal of Clinical Investigation·H J WeissH Brand
May 1, 1983·Electroencephalography and Clinical Neurophysiology·P ApkarianD Van Dorp
Jan 6, 1983·The New England Journal of Medicine·P M MannucciT S Zimmerman
Jul 15, 1983·Thrombosis Research·M I BarnhartJ M Lusher

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Citations

Jul 27, 2005·Annals of Hematology·Randi Katz, Dana Shani
Sep 11, 2002·European Journal of Obstetrics, Gynecology, and Reproductive Biology·János ZatikGyörgy Pfliegler
Aug 2, 2007·Hematology/oncology Clinics of North America·Cindy E Neunert, Janna M Journeycake
Jan 5, 2008·Haemophilia : the Official Journal of the World Federation of Hemophilia·A Coppola, G Di Minno
Aug 17, 2005·Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus·Libe GradsteinWilliam A Gahl
Feb 24, 2006·Respiration; International Review of Thoracic Diseases·Diane M PiersonKevin Leslie
Sep 16, 2004·Pediatric Blood & Cancer·Ana CordovaPedro J Santiago-Borrero

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