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Updated: Jul 7, 2026

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
07:35

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring

Published on: June 23, 2015

Autosomal dominant polycystic kidney disease is associated with an increased prevalence of radiographic

James A Driscoll1, Sanjeev Bhalla, Helen Liapis

  • 1Department of Medicine, Washington University School of Medicine, 660 S. Euclid Ave, St. Louis, MO 63110, USA.

Chest
|February 12, 2008
PubMed

Insights

Autosomal dominant polycystic kidney disease (ADPKD) is linked to a higher prevalence of bronchiectasis, a lung condition. This suggests a potential airway manifestation in ADPKD patients due to primary cilia dysfunction.

Area of Science:

  • Pulmonary Medicine
  • Nephrology
  • Genetics

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder with known extrarenal manifestations but no established pulmonary features.
  • The disease is linked to polycystin-1 and polycystin-2 protein dysfunction, affecting primary cilia in renal epithelial cells.
  • The role of polycystin-1 in airway epithelial cells and its potential impact on lung structure in ADPKD remained unexplored.

Purpose of the Study:

  • To investigate the expression of polycystin-1 in the motile cilia of airway epithelial cells.
  • To evaluate lung structure and identify potential pulmonary abnormalities in patients with ADPKD.

Main Methods:

  • Immunostaining of airway epithelium from non-ADPKD individuals to detect polycystin-1.
  • Histopathological examination of lung tissue from ADPKD patients.
  • Retrospective review of CT scans from 95 ADPKD patients and 95 controls to assess bronchiectasis prevalence.

Main Results:

  • Polycystin-1 was found in the motile cilia of normal airway epithelial cells.
  • Histologic evidence of bronchiectasis was observed in one ADPKD patient's lung tissue.
  • ADPKD patients showed a threefold higher prevalence of bronchiectasis on CT scans compared to controls (37% vs. 13%, p=0.002).

Conclusions:

  • Radiographic bronchiectasis is a previously unrecognized manifestation of ADPKD.
  • The findings suggest a potential increased risk of airway disease in patients with primary cilia-associated disorders like ADPKD.
Abstract

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