• Eur. J. Obstet. Gynecol. Reprod. Biol. · Sep 2002

    Case Reports

    Variable response of Hermansky-Pudlak syndrome to prophylactic administration of 1-desamino 8D-arginine in subsequent pregnancies.

    • János Zatik, Róbert Póka, Antal Borsos, and György Pfliegler.
    • Department of Obstetrics and Gynecology, Medical Faculty, Center for Health and Medical Sciences, University of Debrecen, Debrecen, Hungary. jzatik@yahoo.com
    • Eur. J. Obstet. Gynecol. Reprod. Biol. 2002 Sep 10; 104 (2): 165-6.

    AbstractHermansky-Pudlak syndrome is characterised by oculocutaneous albinism and haemorrhagic diathesis. The bleeding tendency that is associated with this autosomal recessive disease is caused by storage-pool deficiency and has been reported to be controllable by prophylactic administration of 1-desamino 8D-arginine (desmopressin, DDAVP). The DDAVP prophylaxis at the first delivery of our patient did not prevent the severe haemorrhagic sequeal requiring transfusion of packed red cells and platelets, but the same preventive measure was successful at her second childbirth. Response to prophylactic DDAVP administration varies between as well as within patients with Hermansky-Pudlak syndrome.Copyright 2002 Elsevier Science Ireland Ltd.

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