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September 10, 2025Advances in respiratory medicineOpen Access

RSPH4A-PCDx: An Index to Predict Lung Function Decline in Primary Ciliary Dyskinesia

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Authors

GRGabriel Román-RíosGRGabriel Rosario-OrtizMRMarcos J. Ramos-Benítez

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Overview

Retrospective analysis reveals a new tool predicting lung function decline in PCD, indicating early intervention is crucial.

Key Points

  • Patients with RSPH4A-associated PCD experience significant lung function decline, with a median annual FEV1 decrease of -0.75%.
  • The median FEV1 at diagnosis for the cohort was 55%, highlighting the severity of lung impairment in this genetic condition.
  • A new index, PCDx, predicts the timeline for when patients may require lung transplant referral based on their decline.
  • Targeted tools like PCDx could improve clinical decisions and enhance management strategies for individuals with PCD.

Cite This Study

Román-Ríos et al. (2025) studied this question.

synapsesocial.com/papers/68c1ad6a54b1d3bfb60e5d9bhttps://doi.org/10.3390/arm93040027
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