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Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium
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The effect of autosomal dominant polycystic kidney disease on mucociliary clearance

İrfan Kara1, İlyas Öztürk2, Adem Doğaner3

  • 1Department of Otorhinolaryngology, Medical Faculty, Kahramanmaraş Sutcu Imam University, Kahramanmaras, Turkey. drirfankara@gmail.com.

European Archives of Oto-Rhino-Laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
|February 28, 2023
PubMed

Insights

Autosomal dominant polycystic kidney disease (ADPKD) impairs nasal mucociliary clearance, showing significantly longer clearance times in patients. This study investigated the impact of ADPKD on this vital respiratory function.

Area of Science:

  • Nephrology
  • Genetics
  • Respiratory Physiology

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by mutations in PKD1 and PKD2 genes.
  • These genes encode proteins crucial for the function of cilia in renal tubule epithelial cells.
  • Cilia are also present in sinonasal epithelium, suggesting a potential link between ADPKD and sinonasal function.

Purpose of the Study:

  • To evaluate sinonasal epithelium function in ADPKD patients by measuring nasal mucociliary clearance time (NMCT).
  • To investigate the effect of ADPKD on NMCT.
  • To explore potential correlations between NMCT and estimated glomerular filtration rate (eGFR) in ADPKD patients.

Main Methods:

  • A cohort of 34 ADPKD patients and 34 age- and gender-matched controls were recruited.
  • Nasal mucociliary clearance time (NMCT) was assessed using the saccharin test.
  • Estimated glomerular filtration rate (eGFR) was calculated for all participants.

Main Results:

  • The mean age of patients and controls was comparable (approximately 47 years).
  • ADPKD patients exhibited significantly longer NMCT (903.6 s) compared to controls (580 s) (p=0.006).
  • While eGFR was lower in ADPKD patients (72.06 mL/min) than controls (99.79 mL/min) (p<0.001), no significant correlation was found between eGFR and NMCT.

Conclusions:

  • Patients with ADPKD demonstrate significantly impaired nasal mucociliary clearance.
  • The findings suggest that ADPKD affects ciliary function beyond the kidneys, impacting sinonasal health.
  • No direct correlation was observed between the severity of kidney dysfunction (eGFR) and the degree of nasal mucociliary impairment in this cohort.
Abstract

Keywords:
Autosomal dominant polycystic kidney diseaseGenetic diseaseNasal mucociliary clearanceSaccharin test

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