Epidemiology

Alpha1-antitrypsin deficiency tied to high mortality

Last Updated: 2005-04-27 19:30:05 -0400 (Reuters Health)

By David Douglas

NEW YORK (Reuters Health) - People with severe alpha1-antitrypsin deficiency are at increased risk of poor health and death, particularly because of airflow obstruction, researchers report in the April issue of Chest.

"The study reminds clinicians that alpha1-antitrypsin deficiency is an important clinical problem," lead investigator Dr. James K. Stoller told Reuters Health. "That airflow obstruction is a driver of mortality reminds us that interventions to lessen the rate of lung function decline are important."

Dr. Stoller of the Cleveland Clinic Foundation, Ohio, and colleagues examined data from a national registry of patients with alpha1-antitrypsin deficiency.

Over a period of 7.2 years, a total of 204 (18.1%) of the 1129 patients died. Detailed records were available on 120 of the decedents and death certificates were available for 56 of the remaining 84.

Emphysema was the most common underlying cause of death (72%), followed by cirrhosis (10%). Malignancy was a cause of death in 3% and diverticulitis was the cause in another 3%.

Analysis indicated that excess mortality was entirely ascribable to lung and liver disease.

"Clinicians must remain vigilant in monitoring both, as has also been emphasized in the recently published American Thoracic Society/European Respiratory Society Standards document regarding the management of individuals with alpha1-antitrypsin deficiency," Dr. Stoller said.

Chest 2005;127:1196-1204.