Kartagener syndrome Primary ciliary dyskinesia
Conditions
Interventions
A Hypertonic saline (7%) inhalation bi-daily during 3 months
B Placebo: Isotonic saline (0.9%) inhalation bi-daily during 3 months
Sponsors
Vrije Universiteit Medisch Centrum
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: o Diagnosis of primary ciliary dyskinesia o * 18 years of age o Capable of performing lung function tests. o The forced expiratory volume in one second (FEV1), measured at screening, has to be within 10 % of the best value obtained during the previous six months and at least 40% of the predicted value for height, age and sex.
Exclusion criteria
Exclusion criteria: o Smoking o FEV1 < 40 %. o Use of Pulmozyme or other mucolytics or non-routine antibiotics in the previous 30 days. o A decline in lung function of more than 15 % or oxyhemoglobin of < 90% after test nebulisation with hypertonic saline at screening visit17. o Women with a current or intended pregnancy during the trial o Diagnosis of quinine sulphate allergy o Myasthenia Gravis o Lambert-Eaton syndrome o Optic neuritis o Tinnitus o Atrium fibrillation and other currently severe cardiac disease o Epilepsy o Glucose 6PD deficiency
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change in St. George's Respiratory Questionnaire score. | — |
Secondary
| Measure | Time frame |
|---|---|
| Change in Quality of Life questionnaire for bronchiectasis patients score, lung function, lung clearance index, sputum culture results, inflammatory markers in sputum and blood, exacerbation frequency. | — |
Countries
Netherlands
Outcome results
None listed