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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38 Citations
Background Citations
4
Methods Citations
2

38 Citations

Citation Type

Citation Type

Variable response of Hermansky-Pudlak syndrome to prophylactic administration of 1-desamino 8D-arginine in subsequent pregnancies.
CHAPTER 67 – Desmopressin (DDAVP)
Use of DDAVP in inherited and acquired platelet dysfunction
TLDR
The bleeding time response did not correlate with changes in the levels of von Willebrand factor (vWf) antigen or ristocetin cofactor activity, nor was it associated withChanges in vWf multimeric analysis or in vitro platelet aggregations following the administration of DDAVP.
Hermansky-Pudlak-Syndrom: Diagnosestellung und Therapiemöglichkeit mit DDAVP
Das Hermansky-Pudlak-Syndrom (HPS) ist eine autosomal rezessiv vererbte Krankheit, die aus einem Tyrosinase-positiven Albinismus, einer milden Blutungstendenz mit verlangerter Blutungszeit bei…
A Rare Case of Hermansky-Pudlak Syndrome Type 3
TLDR
A case of HPS type 3 in a male Puerto Rican patient who presented to the authors' clinic is reviewed, with diagnosis of dense body and melanosome deficiency.
The use of thromboelastography for the peripartum management of a patient with platelet storage pool disorder.
1‐deamino‐8‐D‐arginine vasopressin (DCAVP) increases platelet membrane expression of glycoprotein Ib in patients with disorders of platelet function and after cardiopulmonary bypass
TLDR
DDAVP increases platelet membrane GPIb expression in a variety of patients and may account for improvement in hemostasis seen in some studies.
Platelet von Willebrand factor in Hermansky‐Pudlak syndrome
The Hermansky‐Pudlak Syndrome (HPS) is an autosomal recessive inherited disorder characterized by oculocutaneous albinism, tissue accumulation of ceroid pigment, and a mild to moderate bleeding…
Clinical Experience in Disorders of Haemostasis
TLDR
Pharmacological agents such as desmopressin that are not derived from blood products offer the potential to achieve adequate haemostasis without the risks of transfusion therapy.
Hermansky–Pudlak syndrome with interstitial lung disease: A holistically worked up couplet
TLDR
An intriguing case scenario of a pair of siblings affected with this rare disorder with its associated interstitial lung disease and its associated ILD is narrated.
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References

SHOWING 1-10 OF 17 REFERENCES
Effect of 1-deamino-8-D-arginine vasopressin on the prolonged bleeding time in chronic renal failure.
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.
SHORTENING OF BLEEDING TIME BY 1-DEAMINO-8-D-ARGININE VASOPRESSIN IN VARIOUS BLEEDING DISORDERS
Deamino-8-D-arginine vasopressin shortens the bleeding time in uremia.
TLDR
It is indicated that 1-deamino-8-D-arginine vasopressin can be used for temporary correction of bleeding time and may prevent surgical bleeding in patients with uremia and factor VIII-related antigen and ristocetin cofactor.
Factor VIII and Fibrinolytic Response to Deamino‐8‐d‐Argenine Vasopressin in Normal Subjects and Dissociate Response in Some Patients with Haemophilia and von Willebrand's Disease
Summary Deamino‐8‐d‐argenine vasopressin (DDAVP) was given by intravenous infusion to normal subjects, haemophiliacs and patients with von Willebrand's disease (vWd) and the factor VIII and…
Correction of the Bleeding Time in Patients with Storage Pool Deficiency by Infusion of Cryoprecipitate
TLDR
On four occasions infusion of cryoprecipitate twice daily protected patients with Hermansky‐Pudlak syndrome and storage pool deficiency from bleeding during surgery.
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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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Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: implications for pathophysiology and therapy of von Willebrand's disease subtypes
TLDR
It is suggested that complete hemostatic correction following DDAVP can be routinely expected only in type I von Willebrand's disease, and only if factor VIII/vonWillebrand factor can be raised to normal levels.
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TLDR
Investigations on the blood of two albino women with a haemostatic defect have revealed a specific abnormality of their platelets that is suggested to be a result of such a defect of storage and release of adenine nucleotides by the platelets.
Response of Factor VIII/von Willebrand Factor to DDAVP in Healthy Subjects and Patients with Haemophilia A and von Willebrand's Disease
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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