Introduction Misfolded α1-antitrypsin (AAT) deficiency results in accumlation in ER of hepatocytes normally functions to inhibit elastase without AAT elastase is over active and destroys elastic tissues results in panacinar empysema accumulation in liver results in cirrhosis AD (codominant) inheritance normal genotype is MM abnormal genoytpe is ZZ uncertain presentation in heterozygotes (MZ) Presentation Symptoms shortness of breath cirrhosis Evaluation Spirometry decreased FEV1 decreased FEV1/FVC increase in TLC Biopsy PAS+ globules in hepatocytes Treatment Medical pooled AAT given IV Surgical liver transplant