PMID- 27296827
OWN - NLM
STAT- MEDLINE
DCOM- 20171218
LR  - 20180205
IS  - 1532-3064 (Electronic)
IS  - 0954-6111 (Linking)
VI  - 116
DP  - 2016 Jul
TI  - Lung clearance index for monitoring early lung disease in alpha-1-antitrypsin 
      deficiency.
PG  - 93-9
LID - S0954-6111(16)30074-9 [pii]
LID - 10.1016/j.rmed.2016.04.015 [doi]
AB  - 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.
CI  - Copyright (c) 2016. Published by Elsevier Ltd.
FAU - Fuchs, Susanne I
AU  - Fuchs SI
AD  - Department of Paediatrics and Research Institute, Marien Hospital Wesel, Germany. 
      Electronic address: susanne.fuchs@prohomine.de.
FAU - Schwerk, Nicolaus
AU  - Schwerk N
AD  - Paediatric Pulmonology, Allergology and Neonatology, Medical University Hannover, 
      Germany.
FAU - Pittschieler, Klaus
AU  - Pittschieler K
AD  - Marienklinik Bozen, Italy.
FAU - Ahrens, Frank
AU  - Ahrens F
AD  - Children's Hospital Altona, Hamburg, Germany.
FAU - Baden, Winfried
AU  - Baden W
AD  - Children's University Hospital Tubingen, Germany.
FAU - Bals, Robert
AU  - Bals R
AD  - Department 5, University of Saarland, Homburg, Germany.
FAU - Fahndrich, Sebastian
AU  - Fahndrich S
AD  - Department 5, University of Saarland, Homburg, Germany.
FAU - Gleiber, Wolfgang
AU  - Gleiber W
AD  - Department 1, University Hospital Frankfurt a.M., Germany.
FAU - Griese, Matthias
AU  - Griese M
AD  - Dr. von Haunersches Kinderspital, Medical University Munich, Germany.
FAU - Hulskamp, Georg
AU  - Hulskamp G
AD  - Department of Paediatrics, Clemenshospital Munster, Germany.
FAU - Kohnlein, Thomas
AU  - Kohnlein T
AD  - Pulmonology, Medical University Hannover, Germany.
FAU - Reckling, Ludmilla
AU  - Reckling L
AD  - Department 1, University Hospital Frankfurt a.M., Germany.
FAU - Rietschel, Ernst
AU  - Rietschel E
AD  - Cystic Fibrosis Centre, University Hospital of Cologne, Germany.
FAU - Staab, Doris
AU  - Staab D
AD  - Paediatric Pulmonologie and Allergology, Charite Berlin, Germany.
FAU - Gappa, Monika
AU  - Gappa M
AD  - Department of Paediatrics and Research Institute, Marien Hospital Wesel, Germany.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20160427
PL  - England
TA  - Respir Med
JT  - Respiratory medicine
JID - 8908438
RN  - 0 (alpha 1-Antitrypsin)
RN  - N762921K75 (Nitrogen)
RN  - alpha-1-Antitrypsin Deficiency, Autosomal Recessive
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Child
MH  - Child, Preschool
MH  - Cross-Sectional Studies
MH  - Female
MH  - Forced Expiratory Volume
MH  - Genotype
MH  - Humans
MH  - Lung/*metabolism/physiopathology
MH  - Lung Diseases/complications/*diagnosis/physiopathology
MH  - Male
MH  - Middle Aged
MH  - Nitrogen/*metabolism
MH  - Prospective Studies
MH  - Pulmonary Emphysema/complications/*diagnostic imaging/physiopathology
MH  - Respiratory Function Tests
MH  - Spirometry/methods
MH  - Vital Capacity
MH  - Young Adult
MH  - alpha 1-Antitrypsin/metabolism
MH  - alpha 1-Antitrypsin Deficiency/*complications/genetics/physiopathology
EDAT- 2016/06/15 06:00
MHDA- 2017/12/19 06:00
CRDT- 2016/06/15 06:00
PHST- 2015/09/08 00:00 [received]
PHST- 2016/03/23 00:00 [revised]
PHST- 2016/04/26 00:00 [accepted]
PHST- 2016/06/15 06:00 [entrez]
PHST- 2016/06/15 06:00 [pubmed]
PHST- 2017/12/19 06:00 [medline]
AID - S0954-6111(16)30074-9 [pii]
AID - 10.1016/j.rmed.2016.04.015 [doi]
PST - ppublish
SO  - Respir Med. 2016 Jul;116:93-9. doi: 10.1016/j.rmed.2016.04.015. Epub 2016 Apr 27.