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Alpha-l-antitrypsin deficiency

*Alpha-l- antitrypsin is a protease inhibitor
Alpha-1- Antitrypsin is a glycoprotein synthesized by the liver accounts for more than 80 % the scrum atpha-1 -globulin fraction. Patients with liver disease are usually PiZZ alleles and have serum alpha antitrypsin levels less than 2 mg/Ml , Jaundice, alcoholic stool, hepatomegaly, cirrhosis may be present
Diagnosis: *Alpha-l- antitrypsin phenotype , confirmed by liver biopsy –PAS positive intracytoplasmic granules are seen.

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