Summary
Definition
History and exam
Key diagnostic factors
- productive cough
- shortness of breath on exertion
- current cigarette smoker
- exposure to gas, fumes, and/or dust
- hepatomegaly
- ascites
- confusion
Other diagnostic factors
- age 32-41
- male gender
- wheezing
- chest hyperinflation
- scleral icterus/jaundice
- asterixis
Diagnostic investigations
1st investigations to order
- plasma AAT level
- pulmonary function testing
- chest x-ray
- chest CT
- LFTs
Investigations to consider
- phenotyping
- genotyping
- exercise testing with ABG analysis
- alpha-fetoprotein
- abdominal CT
Treatment algorithm
Contributors
Authors VIEW ALL
Associate Professor
Pulmonary and Critical Care
University of Chicago
Chicago
IL
Disclosures
DKH has served as a consultant for CSL Behring: manufacturer of AAT replacement therapy. Has lectured for CSL Behring, Grifols, and Shire: manufacturers of AAT replacement therapy. Research sponsored by Shire: manufacturer of AAT replacement therapy.
Dr D. Kyle Hogarth would like to gratefully acknowledge Dr Paul J. Hutchison, a previous contributor to this monograph. PJH declares that he has no competing interests.
Peer reviewers VIEW ALL
Assistant Professor of Medicine
David Geffen School of Medicine at UCLA
Los Angeles
CA
Disclosures
JD declares that she has no competing interests.
Chair of Quality
Director
Pulmonary Hypertension Clinic
Head
Pulmonary Education and Rehabilitation
Department of Pulmonary, Allergy and Critical Care
Cleveland Clinic Florida
Weston
FL
Disclosures
FR has been a consultant and speaker and has received funding from Baxter Healthcare and CSL Behring.
Consultant Chest Physician
Aberdeen Royal Infirmary
Aberdeen
UK
Disclosures
GPC declares that he has no competing interests.
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