At what age is Hermansky-Pudlak syndrome (HPS) typically diagnosed?

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

This disease first manifests in childhood. At this time, albinism is evident. Its systemic manifestations unfold over time and are uncommon in childhood. Inflammatory bowel disease symptom onset occurs at age 12-30 years. The onset of pulmonary fibrosis begins in the third and fourth decades of life. Patients die of pulmonary fibrosis in their fourth or fifth decade of life.


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