ArticleRespiratory medicine case reports2024
Hermansky-Pudlak Syndrome: An unusual pattern of pulmonary fibrosis.
Article in Respiratory medicine case reports, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
1 citing paper in PubMed.
- Usual Interstitial Pneumonia Pattern and Mycobacteria Lung Diseases: A Case Series.Infectious disease reports · 2025Article
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Authors and funding
3 authors.
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Abstract
Hermansky-Pudlak Syndrome is a rare genetic cause of pulmonary fibrosis, associated with albinism, nystagmus, and a bleeding diathesis. Histologically, Hermansky-Pudlak Syndrome Pulmonary Fibrosis (HPS-PF) typically resembles usual interstitial pneumonia (UIP), however radiologically this is not always the case with a range of features described in the current literature. HPS-PF typically occurs earlier in life than idiopathic pulmonary fibrosis (IPF) and there is limited evidence to support the use of antifibrotic therapy. Given the rarity and potential clinical outcomes of the disease, further research is required. This may be aided by the inclusion of patient with HPS-PF in registry databases.
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