Literature DB >> 2916560

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

P W Wijermans1, D B van Dorp.   

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.

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Year:  1989        PMID： 2916560     DOI： 10.1002/ajh.2830300307

Source DB:  PubMed          Journal:  Am J Hematol        ISSN： 0361-8609            Impact factor:   10.047


  7 in total

1.  Hermansky-pudlak syndrome: report of a case and review of the literature.

Authors:  Matthew T Hurford; Christopher Sebastiano
Journal:  Int J Clin Exp Pathol       Date:  2008-01-01

2.  Identification of a novel mutation in HPS6 in a patient with hemophilia B and oculocutaneous albinism.

Authors:  Kevin J O'Brien; Jay Lozier; Andrew R Cullinane; Brigitte Osorio; Khanh Nghiem; Vladislav Speransky; Wadih M Zein; James C Mullikin; Anne T Neff; Karen L Simon; May Christine V Malicdan; William A Gahl; Lisa R Young; Bernadette R Gochuico
Journal:  Mol Genet Metab       Date:  2016-09-03       Impact factor: 4.797

3.  Ocular findings in the Hermansky-Pudlak syndrome.

Authors:  N J Izquierdo; W Townsend; I E Hussels
Journal:  Trans Am Ophthalmol Soc       Date:  1995

4.  Hermansky-Pudlak syndrome in pregnancy: A case report.

Authors:  Lydia Yusuf; Srivasavi Dukka; Etienne Ciantar
Journal:  Obstet Med       Date:  2016-08-05

5.  Hermansky-pudlak syndrome: a case report.

Authors:  Ilhami Berber; Mehmet Ali Erkurt; Irfan Kuku; Emin Kaya; Mustafa Koroglu; Ilknur Nizam; Mehmet Gul; Recep Bentli
Journal:  Case Rep Hematol       Date:  2014-02-20

6.  Clinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation.

Authors:  Souheil El-Chemaly; Kevin J O'Brien; Steven D Nathan; Gerald L Weinhouse; Hilary J Goldberg; Jean M Connors; Ye Cui; Todd L Astor; Philip C Camp; Ivan O Rosas; Merte Lemma; Vladislav Speransky; Melissa A Merideth; William A Gahl; Bernadette R Gochuico
Journal:  PLoS One       Date:  2018-03-16       Impact factor: 3.240

7.  A Rare Case of Hermansky-Pudlak Syndrome Type 3.

Authors:  Joel Alcid; Jeffrey Kim; David Bruni; Ibiyonu Lawrence
Journal:  J Hematol (Brossard)       Date:  2018-05-10
  7 in total

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