THE HERMANSKY-PUDLAK SYNDROME - VARIABLE REACTION TO 1-DESAMINO-8D-ARGININE VASOPRESSIN FOR CORRECTION OF THE BLEEDING-TIME

被引：18

作者：

VANDORP, DB

WIJERMANS, PW

MEIRE, F

VRENSEN, G

机构：

[1] Departments of Ophthalmology, Free University, Amsterdam

[2] Departments of Haematology, Free University, Amsterdam

[3] Department of Ophthalmology, University of Ghent

[4] Department of Morphology, The Netherlands Ophthalmic Research Institute, Amsterdam

来源：

OPHTHALMIC PAEDIATRICS AND GENETICS | 1990年 / 11卷 / 03期

关键词：

Bleeding time; DDAVP (1-desamino-8D-arginine vasopressin; Minrin); Hermansky-Pudlak syndrome; Intra- and inter-individual variability in expression; Phenotype; Storage pool disease;

D O I：

10.3109/13816819009020985

中图分类号：

R77 [眼科学];

学科分类号：

100212 ;

摘要：

The effect of the synthetic vasopressin derivative l-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. © 1990 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted.

引用

页码：237 / 244

页数：8

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