Primary Ciliary Dyskinesia
Conditions
Interventions
A: NaCl 7% nebulization with 0,25mg/ml quinine sulphate as taste masking agent
B: NaCl 0,9% nebulization with 0,25mg/ml quinine sulphate as taste masking agent
Sponsors
VU University medical center
Eligibility
Inclusion criteria
Inclusion criteria: * Diagnosis of primary ciliary dyskinesia * ≥ 18 years of age * Capable of performing lung function tests. * 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: * Smoking * FEV1 < 40 %. * Use of Pulmozyme or other mucolytics or non-routine antibiotics in the previous 30 days. * A decline in lung function of more than 15 % or oxyhemoglobin of < 90% after test nebulization with hypertonic saline at screening visit * Women with a current or intended pregnancy during the trial * Diagnosis of quinine sulphate allergy * Myasthenia Gravis * Lambert-Eaton syndrome * Optic neuritis * Tinnitus * Atrium fibrillation and other severe cardiac heart disease * Epilepsy * Glucose 6PD deficiency
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Primary research question: -Is there a significant difference in change in the St. George’s Respiratory Questionnaire total and sub-scores (SGRQ) within intervention periods? | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary research questions: - Are there significant differences in change in the Quality of Life score for bronchiectasis patients score (QoL-B) , lung function, lung clearance index, respiratory culture results, inflammatory parameters in sputum and blood and exacerbation frequency? - What are the effect sizes of these differences in PCD patients? | — |
Contacts
Public ContactT. Paff
Postbus 7057
Outcome results
None listed