Normal diffusing capacity in patients with PiZ alpha(1)-antitrypsin deficiency, severe airflow obstruction, and significant radiographic emphysema

Jeffrey S Wilson; Jeffrey R Galvin

doi:10.1378/chest.118.3.867

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Normal diffusing capacity in patients with PiZ alpha(1)-antitrypsin deficiency, severe airflow obstruction, and significant radiographic emphysema

Journal article

Peer reviewed

Normal diffusing capacity in patients with PiZ alpha(1)-antitrypsin deficiency, severe airflow obstruction, and significant radiographic emphysema

Jeffrey S Wilson and Jeffrey R Galvin

Chest, Vol.118(3), pp.867-871

09/2000

DOI: 10.1378/chest.118.3.867

PMID: 10988219

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Abstract

alpha(1)-Antitrypsin deficiency is usually suspected clinically in young adults with irreversible airflow obstruction that is out of proportion to their smoking history. Many patients with alpha(1)-antitrypsin deficiency receive an initial diagnosis of asthma or chronic bronchitis. Measurement of the diffusing capacity of the lung for carbon monoxide (DLCO) has been recommended as a way to help distinguish emphysema from asthma and chronic bronchitis. In this article, we describe four patients with severe alpha(1)-antitrypsin deficiency, each of whom had a repeatedly normal DLCO despite having a significant component of fixed airway obstruction and prominent panacinar emphysema on high-resolution CT scan (HRCT). Each patient also demonstrated significant bronchodilator responsiveness, and two patients received an initial diagnosis of asthma. Potential explanations for these findings are discussed. We report these findings to illustrate the limitations of DLCO in this setting. alpha(1)-Antitrypsin deficiency should be considered in patients with fixed airway obstruction that is out of proportion to their age and smoking history, regardless of their diffusing capacity and response to bronchodilators.

Bronchodilator Agents - therapeutic use

Pulmonary Emphysema - complications

Diagnosis, Differential

Pulmonary Emphysema - physiopathology

Humans

Airway Obstruction - physiopathology

Male

Pulmonary Emphysema - drug therapy

Tomography, X-Ray Computed

Pulmonary Emphysema - diagnostic imaging

Pulmonary Diffusing Capacity - physiology

alpha 1-Antitrypsin Deficiency - blood

Airway Obstruction - complications

alpha 1-Antitrypsin Deficiency - complications

Airway Obstruction - diagnostic imaging

alpha 1-Antitrypsin Deficiency - physiopathology

Adult

Female

alpha 1-Antitrypsin - metabolism

Airway Obstruction - drug therapy

Pro-Opiomelanocortin

Details

Title: Subtitle: Normal diffusing capacity in patients with PiZ alpha(1)-antitrypsin deficiency, severe airflow obstruction, and significant radiographic emphysema
Creators: Jeffrey S Wilson - Department of Internal Medicine, University of Iowa, Iowa City, IA 52242-1081, USA. jeff-wilson@uiowa.edu
Jeffrey R Galvin
Resource Type: Journal article
Publication Details: Chest, Vol.118(3), pp.867-871
DOI: 10.1378/chest.118.3.867
PMID: 10988219
NLM abbreviation: Chest
ISSN: 0012-3692
eISSN: 1931-3543
Language: English
Date published: 09/2000
Academic Unit: Pulmonary, Critical Care, and Occupational Medicine; Internal Medicine
Record Identifier: 9984094726002771

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