Dermatologic Manifestations of Hermansky-Pudlak Syndrome Medication

Updated: Aug 10, 2018
  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

Medications have been used to try to diminish the bleeding diathesis in patients with Hermansky-Pudlak syndrome. Poor response to desmopressin acetate (DDAVP) in children with Hermansky-Pudlak syndrome has been reported. [42] Hermansky-Pudlak syndrome with severe colitis has responded to infliximab. [43] The pulmonary fibrosis of Hermansky-Pudlak syndrome has been treated with pirfenidone. [44]

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Hemostatic Agents

Class Summary

These agents are potent inhibitors of fibrinolysis and can reverse states that are associated with excessive fibrinolysis.

Desmopressin acetate (DDAVP, Stimate, DDAVP injection)

Desmopressin acetate is a synthetic analogue of the natural pituitary hormone 8-arginine vasopressin (ADH), an antidiuretic hormone affecting renal water conservation. It is indicated for patients with hemophilia A and mild von Willebrand disease to maintain hemostasis. If used preoperatively, administer 30 minutes prior to the scheduled procedure.

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