Pulmonary permeability in primary ciliary dyskinesia.

Pulmonary clearance (Pcl) of aerosolized 99mTc-DTPA was studied in fourteen patients with primary ciliary dyskinesia (PCD), (median age 23.5 yrs, range 12-44 yrs) and nine normal individuals (median age 23 yrs, range 18-27 yrs). All had never smoked. Regional Pcl was studied for arbitrarily defined central and peripheral regions of the lung using a gamma camera method, whilst total Pcl was studied by a plasma sample method. The patients with PCD had significantly reduced total Pcl compared to the normal individuals (p less than 0.05) and also significantly lower total lung capacity (TLC), vital capacity (VC), forced expiratory volume in one second (FEV1), and FEV1/VC values (p less than 0.05). There was no correlation between Pcl and FEV1/VC. It is concluded that the reduced Pcl in the PCD patients may be associated with their small lung volumes. In addition, reduced bronchial clearance of surfactant in PCD may be associated with an increased alveolar lining fluid volume and/or an impaired movement of 99mTc-DTPA along the alveolar septa to the bronchoalveolar junction, where the epithelium may be more specialized for absorption.