Lung Clearance Index (LCI) is Stable in Most Primary Ciliary Dyskinesia (PCD) Patients Managed in a Specialist Centre: a Pilot Study.
Lung
; 195(4): 441-443, 2017 08.
Article
in En
| MEDLINE
| ID: mdl-28634892
ABSTRACT
Primary ciliary dyskinesia is a condition in which abnormal cilia structure or function leads to reduced mucociliary clearance and obstructive lung disease. Twenty-nine patients had lung clearance index (LCI) measured in 2009 and we attempted to perform a 5-year follow-up. Only 12 patients could be re-recruited, but in this small group LCI was stable over the 5 years, which confirms previous data showing that spirometry is also stable in these patients over the medium term. The two patients with the highest LCI in 2009 had since died, despite one having relatively preserved spirometry at the time. These data may be used to inform sample size calculations of future studies.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Outpatient Clinics, Hospital
/
Respiratory Function Tests
/
Mucociliary Clearance
/
Kartagener Syndrome
/
Lung
Type of study:
Prognostic_studies
Limits:
Humans
Language:
En
Journal:
Lung
Year:
2017
Document type:
Article
Affiliation country:
United kingdom