Alpha-1 Antitrypsin Deficiency Q. 1 What is true about Alfa 1 antitrypsin deficiency? A Severe pulmonary disease B Liver biopsy revealing PAS positive diastase sensitive granules C ZZ genotype is associated with bad prognosis D All of the above Q. 1 What is true about Alfa 1 antitrypsin deficiency? A Severe pulmonary disease B Liver […]
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Alpha-1 Antitrypsin Deficiency ALPHA-1 ANTITRYPSIN DEFICIENCY Apha-1 antitrypsin deficiency is an autosomal recessive disorder. Alpha- 1 antitrypsin is a serine protease (mainly elastase) inhibitor produced in the liver. It produces liver disease (cirrhosis), pulmonary disease (panacinar- emphysema). Associated with bronchiestasis, cutaneous paniculitis & Wegner’s granulomatosis. Pathogenesis– Protein folding & accumulation of protein in endoplasmic reticulum.
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