Lung clearance index for monitoring early lung disease in alpha-1-antitrypsin deficiency
Autor: | Ernst Rietschel, Nicolaus Schwerk, Matthias Griese, Winfried Baden, Georg Hülskamp, Robert Bals, Klaus Pittschieler, Thomas Köhnlein, Ludmilla Reckling, Susanne I. Fuchs, Sebastian Fähndrich, Wolfgang Gleiber, Frank Ahrens, Monika Gappa, Doris Staab |
---|---|
Rok vydání: | 2015 |
Předmět: |
Pulmonary and Respiratory Medicine
Spirometry Adult Lung Diseases Male medicine.medical_specialty Pathology Adolescent Genotype Cross-sectional study Nitrogen Vital Capacity Lung Clearance Index Gastroenterology 03 medical and health sciences Young Adult 0302 clinical medicine Internal medicine Forced Expiratory Volume alpha 1-Antitrypsin Deficiency Medicine Humans 030212 general & internal medicine Prospective Studies Young adult Prospective cohort study Child Lung Aged Alpha 1-antitrypsin deficiency medicine.diagnostic_test business.industry Middle Aged medicine.disease Peripheral Respiratory Function Tests medicine.anatomical_structure Cross-Sectional Studies 030228 respiratory system Pulmonary Emphysema Child Preschool alpha 1-Antitrypsin Female business |
Zdroj: | Respiratory medicine. 116 |
ISSN: | 1532-3064 |
Popis: | Patients with alpha-1-antitrypsin deficiency (AATD) and a PI-ZZ genotype are at high risk to develop severe emphysema during adulthood. However, little is known about early stages of emphysema and disease manifestation in other PI-types. Spirometry is commonly used for monitoring although early manifestation of emphysema is suspected within the peripheral airways that are not accessible by forced expiratory manoeuvres. We hypothesized that the Lung Clearance Index (LCI) derived from multiple breath nitrogen-washout (N2-washout) is useful to bridge this diagnostic gap. Patients from age 4 years onward and different PI-types performed N2-washout and spirometry. Results were compared to controls. 193 patients (4-79 years, 75% PI-ZZ) and 33 controls (8-60 years) were included. Mean (SD) LCI in patients was 9.1 (3.1) and 6.3 (0.6) in controls (p ≤ 0.001). 47% of adult patients with other than PI-ZZ genotypes and 39% of all patients with normal spirometry had abnormal LCIs. The LCI measured by N2-washout discriminates between patients with AATD and controls, reflects AATD related lung disease in all stages and appears to identify early peripheral lung changes in younger age than spirometry. We conclude that a normal spirometry does not exclude presence of AATD related lung disease even in genotypes other than PI-ZZ. |
Databáze: | OpenAIRE |
Externí odkaz: |