Literature DB >> 10472549

[Thrombocytic alpha-delta-storage-pool-disease: shortening of bleeding time after infusion of 1-desamino-8-D-arginine vasopressin].

A Kosch1, B Kehrel, U Nowak-Göttl, J Häberle, H Jürgens.   

Abstract

BACKGROUND: The synthetic vasopressin derivate desmopressin (1-desamino-8-D-arginine vasopressin) has been reported to shorten the bleeding time in patients with hemophilia A, von Willebrand's disease and several functional platelet disorders. In addition to substitution of platelets, vasopressin is therefore used to prevent bleeding complications. CASE: We report the case of a 14-year-old female patient with prolonged bleeding time due to the rare thrombocytic alpha-delta-storage-pool-disease. When normal donor platelet substitution alone was ineffective, bleeding time was normalised after infusion of desmopressin and elective wisdom-tooth extraction was performed without significant postoperative bleeding. DISCUSSION: Infusion of desmopressin appears to be effective in shortening bleeding time in thrombocytic storage-pool-disease. Its use could prevent bleeding complications after trauma and surgical interventions and may possibly help to spare the need for platelet concentrates.

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Year:  1999        PMID: 10472549     DOI: 10.1055/s-2008-1043787

Source DB:  PubMed          Journal:  Klin Padiatr        ISSN: 0300-8630            Impact factor:   1.349


  1 in total

1.  Combined alpha-delta platelet storage pool deficiency is associated with mutations in GFI1B.

Authors:  Carlos R Ferreira; Dong Chen; Shirley M Abraham; David R Adams; Karen L Simon; May C Malicdan; Thomas C Markello; Meral Gunay-Aygun; William A Gahl
Journal:  Mol Genet Metab       Date:  2016-12-18       Impact factor: 4.797

  1 in total

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