What is the role of medications in the treatment of Hermansky-Pudlak syndrome (HPS)?

Updated: Dec 13, 2019
  • Author: Jaclyn Scholtz, MD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Medications have been used to try to diminish the bleeding diathesis in patients with Hermansky-Pudlak syndrome. However, a poor response to desmopressin acetate (DDAVP) in children with Hermansky-Pudlak syndrome has been reported. [43] Hermansky-Pudlak syndrome with severe colitis has responded to infliximab. [44] The pulmonary fibrosis of Hermansky-Pudlak syndrome has been treated with pirfenidone. [45]


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