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Hermansky‐pudlak syndrome: Correction of bleeding time by 1‐desamino‐8D‐arginine vasopressin
Author(s) -
Wijermans P. W.,
van Dorp D. B.
Publication year - 1989
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830300307
Subject(s) - desmopressin , vasopressin , arginine , hermansky–pudlak syndrome , medicine , hemostasis , endocrinology , gastroenterology , chemistry , biochemistry , amino acid , pulmonary fibrosis , fibrosis
The effect of the synthetic vasopressin derivative 1‐desamino‐8D‐arginine vasopressin (DDAVP = desmopressin) on bleeding time was studied in three patients with Hermansky Pudlak syndrome. A good response was observed in this type of storage pool disease. DDAVP might be useful in managing the bleeding disorder found in patients with the Hermansky‐Pudlak syndrome.