Hermansky‐pudlak syndrome: Correction of bleeding time by 1‐desamino‐8D‐arginine vasopressin

@article{Wijermans1989HermanskypudlakSC,
  title={Hermansky‐pudlak syndrome: Correction of bleeding time by 1‐desamino‐8D‐arginine vasopressin},
  author={Pierre W. Wijermans and D B van Dorp},
  journal={American Journal of Hematology},
  year={1989},
  volume={30}
}
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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TLDR
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TLDR
The prolonged bleeding times of 12 patients with chronic renal failure were significantly shortened at 1 and 2 h after an infusion of 0.4 microgram/kg 1-deamino-8-D-arginine vasopressin (DDAVP), suggesting that the effect of DDAVP is a temporary one.
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TLDR
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TLDR
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TLDR
The findings indicate that DDAVP might be given when biopsies or other surgical procedures must be carried out in patients with prolonged BT, however, the compound is often ineffective in Patients with thrombocytopenia or congenital platelet dysfunction.
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TLDR
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TLDR
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TLDR
It is shown that the consistency of the VIII:C response tends to decrease when repeated doses are given to healthy subjects, and this study shows that the response appears to be more prolonged in haemophiliacs.
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