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Alpha 1-PI for emphysema due to alpha 1-antitrypsin deficiency
1Division of Pulmonary and Critical Care Medicine, Hahnemann University Hospital, Philadelphia, Pennsylvania.
American Family Physician
|September 1, 1989
Summary
Alpha 1-antitrypsin deficiency is a genetic condition causing early emphysema. Weekly infusions of alpha 1-proteinase inhibitor (alpha 1-PI) are safe and effective for preventing lung disease.
Area of Science:
- Pulmonary Medicine
- Genetics
- Biochemistry
Background:
- Alpha 1-antitrypsin deficiency is an inherited condition.
- It leads to a lack of alpha 1-proteinase inhibitor (alpha 1-PI), a protective protein.
- This deficiency can cause premature pulmonary emphysema.
Purpose of the Study:
- To outline diagnostic considerations for alpha 1-antitrypsin deficiency.
- To introduce the therapeutic use of purified alpha 1-PI concentrate.
Main Methods:
- Diagnosis is considered in patients with early-onset emphysema (before age 40), especially nonsmokers.
- Evaluation includes assessing disease distribution (lower lobe predominance) and family history.
- Treatment involves weekly intravenous infusions of alpha 1-PI concentrate.
Main Results:
- Purified human alpha 1-PI concentrate is now available for therapeutic use.
- Weekly infusions effectively increase serum alpha 1-PI levels.
- The treatment is safe, with minimal adverse effects.
Conclusions:
- Alpha 1-antitrypsin deficiency requires early diagnosis for timely intervention.
- Alpha 1-PI augmentation therapy is a safe and effective strategy to prevent emphysema progression.