Gastrointestinal

Question 1

What is Alpha 1 Antitrypsin?

Answer 1

This is a protease inhibitor that prevents tissue damage from neutrophil elastase. It is produced by the liver and commonly found in the lungs

Question 2

Presentation of Alpha 1 Antitrypsin Disorder? Management?

Answer 2

Lungs (Emphysema): Dyspnea, cough, wheezing, decr BS, Barrel chest

Liver: cholestatic jaundice in neonates, adults–chronic hepatitis, cirrhosis, HCC

Invest: CXR (hyperinflated chest); decr serum antitrypsin protein levels

Mx: Avoid smoking, Preventative vaccine (influenza, pneumococcus); symptomatic Tx of emphysema