Chapters Transcript Video Alpha-One Antitrypsin Deficiency and Ascending Aortic Distention Friederich Kueppers, MD discusses a study indicating a link between Alpha-One Antitrypsin Deficiency and Aortic Aneurysm. Okay my name is dr fred cooper's. I'm a professor of forensic medicine and surgery at the Lewis Katz School of Medicine at Temple University in the in the lung center. And I'm going to talk about some study that we have recently recently completed. Um I'm going to reach deals with these vascular changes that occur with editors and efficiency. The major um pathology that's associated with groups and efficiency is uh eczema and that's been has been known for a long time. Um However lately there have there have been some other um pathologies come up concerning the concerning vascular changes and um we have decided to to study this and to to look specifically at the the diameter of the ascending aorta right at the point where it comes out of the out of the last left ventricle. We have made measurements on on 51 patients within the ribs and efficiency right at the level of of the ascending aorta at the at the level of the of the primary artery. And um the we we were interested to see if there was an association with um And represent efficiency and with three with a degree of emphysema. They had 51 patients in the study. They were all they all had clips of deficiency and the next slide shows the the baby made the measurement you see in the middle here the round structure. This is the L to the ascending aorta and the structure on the on the right is the pulmonary artery. So the measurements were made at the indicated in the in the C. T. Scan here and in all of these patients. And I see and in blue to see the age distribution of the patients who had the the delegation of the delta and in yellow you see those who had emphysema but well did not have a deficiency. And you see the age distribution is different. In fact it turns out that the the aortic diameter is dilated in patients and trips and efficiency as well. And those who don't have the deficiency. But with those with the deficiency um the delegation occurs approximately 10 years earlier. As you can see here from the from the distributed from the edge distribution as it occurs in the in those who don't have a deficiency. And um the next slide shows the correlation of um mm age um in in blue for those who have benefits of deficiency and in yellow those who don't have the deficiency. And you can see that there is a significant increase in this age range here um of the of the aortic diameter. And with those who have deficiency and in those who don't have the deficiency of emphysema there's no significant increase. This slide shows the social cross cross section through a through to uh are altering. And you see the this baby line here in the around the around the vessel which is a lasting and we we think and there is sufficient evidence that in terms of deficiency um the elastin is attacked by political enzymes in particular, one that comes from white blood cells. The human local civic alas states, which breaks down the elastic structure in the in the arterial role including the the wall of the hail job. Therefore the at the vessel loses its electricity becomes more stiff and eventually bill will dilate and will form and and they can form an and of course aneurysms um are dangerous. They are like time bombs and they can rupture and this can be a devastating event. Um County 80% mortality rate um as I mentioned before and it helps in deficiency um um has first been described and as a predisposing factor to emphysema. But and then uh subsequently it became clear that the language is not the only organ involved. And the the one that we concentrate on here is how the basket changes. And as as I mentioned, the the underlying pathology um which is very similar to the pathology that leads to emphysema, namely an attack of elastic elastic enzymes on the on the and therefore leading to vascular changes and and eventually to them. Um There is someone consideration that we also should should keep in mind that there is an association and has recently been shown in good constantly uh studies that the the dilated uh Jurassic aorta is significantly associated with cerebral aneurysms. Therefore the it appears that the damage to the the vascular structure and efficiency is not limited to the to the lung and to the to the major vessels and the particularly aorta, but also to other vessels, including cerebral cerebral vasculature, which then again can lead to aneurysms and rupture. Created by Presenters Friedrich Kueppers, MD Professor, Thoracic Medicine and Surgery Clinical Interests: Regulation of A1-antitrypsin production, Inflammatory cytokines, Genetics View full profile
