What is the role of CT scanning in the workup 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

High-resolution CT is more sensitive than chest radiography in evaluating the extent of pulmonary disease in patients with Hermansky-Pudlak syndrome. [37] Mild findings on high-resolution CT scans are paralleled by normal findings on chest radiographs. Common chest radiographic findings include reticulonodular interstitial pattern, perihilar fibrosis, and pleural thickening. High-resolution CT scans can show septal thickening, ground-glass opacities, and peribronchovascular thickening. One study noted that 82% of patients with Hermansky-Pudlak syndrome had abnormalities on high-resolution CT scans. [38]

Owing to the increase risk of bleeding complications, patients with Hermansky-Pudlak syndrome who experience physical trauma should undergo CT scanning to rule out intra-articular, intracranial, and/or internal bleeding.


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