Carol M. Rossman

Pulmonary Mucociliary Clearance in Cystic Fibrosis

Abstract Pulmonary clearance was examined by measurement of the rate of removal of a 99mTc-albumin aerosol (aerodynamic mass median diameter 3 μ) in 13 children with cystic fibrosis. Lung retention of radioactive label was monitored with a scintillation camera interfaced to a data-storage and retrieval system. The results were compared to those of nine normal adults. Overall lung retention was at all times greater in the normal subjects than in the patients (83.9 per cent at six hours after initial deposition vs. 59.6 per cent). The clearance curves of the patients had two exponentials with clearance half-times of 0.7 and 11.5 hours respectively. The mean curve for the normal subjects had a single exponential with half-times of 23.0 hours. In clinically stable patients with cystic fibrosis, clearance of inhaled aerosol from ciliated airways takes place at rapid rates, similar to those previously reported for mucociliary transport in normal adults.

Sulfur dioxide and tracheobronchial clearance in man.

Tracheobronchial clearance was measured in nine healthy, nonsmoking adults. Technetium Tc 99m albumin aerosol (mass median diameter, 3 mu; geometric standard deviation, 1.6) was inhaled as a bolus under controlled conditions to achieve reproducible deposition in large airways. Each subject was studied in three seperate three-hour experiments: twice under control conditions and once exposed to 5 ppm sulfur dioxide (pollutant exposure after aerosol inhalation). Lung retention of activity was measured using a gamma camera interfaced to a data storage and retrieval system. The study showed that (1) Both deposition and clearance were highly reproducible in individuals in repeat control studies. (2) Acute exposure to 5 ppm sulfur dioxide had no significant effect (P greater than .05) on mucocillary clearance in resting healthy subjects, except perhaps for a small transient change (P=.05) after one hour. (3) Pulmonary function tests showed a decrease in maximal midexpiratory flow (P less than .01) but no other significant changes.

Cystic fibrosis—related inhibition of mucociliaryclearance in vivo in man

Studies were performed to demonstrate possibly cystic fibrosis-related inhibition of mucociliary clearance in man. Topical application of normal serum or of CF serum did not inhibit in vivo nasal MCC. Induction of local inflammation by topical anti-IgE-reduced nasal MCC in CF subjects, but increased MCC in normal individuals. Furthermore, nasal MCC was inhibited in normal patients by CF serum but not normal serum, applied to the anti-IgE-treated nasal mucosa. These observations are consistent with the hypothesis that CF serum inhibits MCC in vivo in the inflamed mucosa.

EFFECT OF CYSTIC FIBROSIS SERUM ON NASAL MUCOCILIARY CLEARANCE

Studies were performed to detect a possible in vivo effect of the ciliary dyskinesia factor of cystic fibrosis (CF) serum. Following application of serum to the nasal mucosa of CF patients and normal subjects, nasal mucociliary clearance (MCC) was assessed by a radioisotope technique. Removal of a radioactive droplet applied to the mucocutaneous junction of the nose was quantified by means of an Anger scintillation camera interfaced to a data storage and retrieval system. Autologous serum applied to the nasal mucosa failed to inhibit MCC in normals or in CF patients, nor did CF serum inhibit MCC in normals. By contrast, an inflammatory response induced by locally applied anti-serum to human IgE retarded MCC in CF patients but not in normals: it is presumed that the local effect of anti-IgE on MCC in CF was the result of an exudation of the serum dyskinesia factor. Possibly, recurrent or persistent pulmonary infection In CF relates to a local exudation of this factor, hindering effective clearance of secretions.Supported by C.F. Foundation, MRC (Canada), Ont. Thoracic Soc., Can. TB & RD Soc.

Primary Ciliary Dyskinesia

Primary ciliary dyskinesia is a disorder characterized by chronic respiratory tract infections, abnormally positioned internal organs, and the inability to have children (infertility). The signs and symptoms of this condition are caused by abnormal cilia and flagella. Cilia are microscopic, finger-like projections that stick out from the surface of cells. They are found in the linings of the airway, the reproductive system, and other organs and tissues. Flagella are tail-like structures, similar to cilia, that propel sperm cells forward.