What is included in the long-term monitoring 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

A variety of steps in medical care can be useful in helping patients.

A MedicAlert bracelet or other identification can be helpful in notifying caregivers of patients with Hermansky-Pudlak syndrome who have a bleeding disorder.

The avoidance of aspirin, products containing aspirin, and any other products that may compromise platelet function is important for patients.

The importance of good dental care needs to be explained to patients. The dentist must be aware that the patient has Hermansky-Pudlak syndrome because some dental procedures may require special medications and precautions.

Proper treatment of nosebleeds is important for patient care. Nasal packing should only be performed under medical supervision.

Damage to the lungs must be avoided; thus, patients must avoid smoking.

The use of gauze with petrolatum and pressure dressings on cuts or abrasions may be useful. Gelfoam may help with superficial bleeding as well. Desmopressin and platelets may be beneficial in serious bleeding complications.

Feliciano et al [42] noted steps to facilitate dental care for patients with Hermansky-Pudlak syndrome, including (1) use of eyeglasses with 99% ultraviolet filter to protect Hermansky-Pudlak syndrome patients from the unpleasant dental light stimulus, (2) an extra-soft toothbrush and conservative brushing technique, (3) use of medications with antifibrinolytic agents, and (4) local care to ensure hemostasis.


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