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What is Alpha 1 antitrypsin deficiency seen in - and explain in detail?
Ans. is 'a' i.e., Emphysema o a1-anti-trvpsin deficiency :-o a1-anti-trypsin deficiency, is an autosomal recessive disease marked by abnormaliy low levels of (serum) of this major protease inhibitor (Pi )o Deficiency of the enzyme leads to pulmonary emphysema (panacinar)o Liver is characterised byPresence 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 resistantIn most part, only distinctive feature is globules infrequentlv-fatty change, mallory bodies.Neonatal hepatitis with cholestasisFatty change (steatosis)Cirrhosis may develop which can transform into hepfocellular carcinoma.