Evaluation of Functional Activity of Ciliated Epithelium in Patients with Primary Ciliary Dyskinesia

Primary ciliary dyskinesia (PCD) is a heterogeneous recessive disorder of cilia that leads to respiratory tract disorders and organ laterality defects.

Objectives

This study was designed to diagnose PCD in patients using various methods: genetic, immunofluorescence, transmission electron microscopy (TEM), and assessment of ciliary beat frequency (CBF) in ALI cultures.

Methods