Which pulmonary and cardiac 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

Auscultation of patients' lungs is important since patients may have impaired lung function. Patients with Hermansky-Pudlak syndrome have a risk of developing pulmonary fibrosis and susceptibility is linked to the alveolar epithelium. [35] A case of pulmonary alveolar proteinosis has been reported. [36]

Accumulation of ceroid in the heart can cause cardiomyopathy in these patients; thus, auscultation of the heart is important.


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