α​1 -anti-trypsin deficiencyα​1 -anti-trypsin deficiency, is an autosomal recessive disease marked by abnormally low levels of (serum) of this major protease inhibitor (Pi) Deficiency of the enzyme leads to pulmonary emphysema (panacinar) Liver is characterised by : Presence of round to oval cytoplasmic globular inclusions in hepatocytes which on H and E stains acidophilic and indistinctly demarcated from surrounding cytoplasm. They are PAS positive and diastase resistant In most part, only distinctive feature is globules infrequently-fatty change, mallory bodies. Neonatal hepatitis with cholestasis Fatty change (steatosis) Cirrhosis may develop which can transform into heptocellular carcinoma.Following information has been added in 8th/e of RobbinsIn addition, cutaneous panniculitis, arterial aneurysm, bronchiectasis and wegner's granulomatosis can occur in α​1- antitrypsin deficiency.