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Hermansky-Pudlak syndrome: correction of bleeding time by 1-desamino-8D-arginine vasopressin.

作者信息

Wijermans P W, van Dorp D B

机构信息

Department of Hematology, Free University Hospital, Amsterdam, The Netherlands.

出版信息

Am J Hematol. 1989 Mar;30(3):154-7. doi: 10.1002/ajh.2830300307.

DOI:10.1002/ajh.2830300307
PMID:2916560
Abstract

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.

摘要

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Hermansky-Pudlak syndrome: correction of bleeding time by 1-desamino-8D-arginine vasopressin.
Am J Hematol. 1989 Mar;30(3):154-7. doi: 10.1002/ajh.2830300307.
2
The Hermansky-Pudlak syndrome. Variable reaction to 1-desamino-8D-arginine vasopressin for correction of the bleeding time.
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[Thrombocytic alpha-delta-storage-pool-disease: shortening of bleeding time after infusion of 1-desamino-8-D-arginine vasopressin].[血小板α-δ-储存池病：输注1-去氨基-8-D-精氨酸加压素后出血时间缩短]
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Consistency of responses to separate desmopressin infusions in patients with storage pool disease and isolated prolonged bleeding time.储存池病和单纯性出血时间延长患者对不同剂量去氨加压素输注反应的一致性。
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Shortening of bleeding time by 1-deamino-8-D-arginine vasopressin in various bleeding disorders.1-去氨基-8-D-精氨酸血管加压素对各种出血性疾病出血时间的缩短作用。
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Lack of effect of 1-desamino-8-D-arginine vasopressin on direct adhesion of platelets to collagen.1-去氨基-8-D-精氨酸血管加压素对血小板与胶原蛋白直接黏附无作用。
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Platelet aggregation at high shear is impaired in patients with congenital defects of platelet secretion and is corrected by DDAVP: correlation with the bleeding time.血小板分泌先天性缺陷患者在高剪切力下的血小板聚集功能受损，而1-去氨基-8-D-精氨酸加压素（DDAVP）可纠正该缺陷：与出血时间的相关性
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Poor response to desmopressin acetate (DDAVP) in children with Hermansky-Pudlak syndrome.患有Hermansky-Pudlak综合征的儿童对醋酸去氨加压素（DDAVP）反应不佳。
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Hermansky-pudlak syndrome: report of a case and review of the literature.赫尔曼斯基-普德拉克综合征：一例报告并文献复习
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