Which pulmonary history findings are characteristic 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

Ceroid can accumulate in the pulmonary system as well. When the lungs are impaired in this fashion, shortness of breath and abnormal fatigue with exertion can occur. The disease can progress to pulmonary fibrosis, with scar tissue restricting the inflation of the lungs. [25] Patients should be asked about previous pulmonary function tests and steroid therapy. Thomas de Montpréville et al [26] noted pulmonary fibrosis in a patient with Hermansky-Pudlak syndrome related to a defect in surfactant. This patient had undergone lung transplantation.


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