Table 3

Advice for severe and intermediate AATD

Advice for severe AATD (homozygotes or compound heterozygotes)
 Quit smoking
 Avoid outdoor pollution and exposure to dust/irritants
 Avoid alcohol and apply appropriate diet, in case of polymerogenic mutations
 Test for AATD in first-degree relatives
 Perform regular respiratory follow-up: lung function tests, lung imaging (if necessary)
 Perform regular liver follow-up, in case of polymerogenic mutations: liver tests, abdominal ultrasounds, fibroscan
 Give augmentation therapy, if necessary
Advice for intermediate AATD (heterozygotes)
 Quit smoking
 Avoid outdoor pollution and exposure to dust/irritants
 Reduce alcohol and apply appropriate diet, in case of polymerogenic mutations
 Perform regular respiratory follow-up: lung function tests, lung imaging (if necessary)
 Perform regular liver follow-up, in case of polymerogenic mutations: liver tests, abdominal ultrasounds, fibroscan