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Alpha-1 Antitrypsin Deficiency

by Brian Alverson, MD
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    00:01 and then this flows to the lungs and protects our lungs from what would be damage from this neutrophil elastase.

    00:05 presentation of respiratory disease that happens a little bit later in childhood and let’s focus now on alpha-1-antitrypsin deficiency.

    00:16 So, in a normal person, we make neutrophil elastase.

    00:22 This is a substrate that is secreted by neutrophils to fight infections.

    00:28 We protect our own bodies from these elastase with alpha-1-antitrypsin.

    00:35 In patients who are normal, they are making alpha-1-antitrypsin in the liver as you can see here, and then this flows to the lungs and protects our lungs from what would be damage from this neutrophil elastase.

    00:53 So the alpha-1-antitrypsin is protecting our lungs against the neutrophil elastase, which we’re using to fight off infections.

    01:00 In a patient with alpha-1-antitrypsin deficiency, the problem is they can’t get the alpha-1-antitrypsin out of the liver, it’s trapped in there, and that accrual is actually causing liver damage.

    01:14 Furthermore, because that can’t get out of the liver, the lungs now lack the protection and the neutrophil elastase is causing lung damage.

    01:28 This disease affects about one in 5,000 people.

    01:32 It’s more common in Caucasians, and the liver disease often starts in childhood with prolonged jaundice and no clear cause.

    01:42 Lung disease typically starts in adolescence or young adulthood.

    01:47 If an adolescent is a smoker, they will dramatically worsen their lung outcomes and the risk of hepatocellular cancer later in life is very real.

    01:59 So how do we manage these children? We worry about their lungs and their livers.

    02:03 We provide supportive care for their absorption of bile salts through liver dysfunction, they might have a problem and we take care of that.

    02:15 In addition, we manage their lung disease accordingly.

    02:20 Intravenous alpha-1-antitrypsin is available, but studies haven’t really gone forward yet in terms of how effective this is.

    02:28 So this is a developing area and we may see future regimens involved in the future with intravenous alpha-1-antitrypsin, but that’s a relatively new thing.


    About the Lecture

    The lecture Alpha-1 Antitrypsin Deficiency by Brian Alverson, MD is from the course Pediatric Pulmonology.


    Author of lecture Alpha-1 Antitrypsin Deficiency

     Brian Alverson, MD

    Brian Alverson, MD


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