A few patients with alpha-1 antitrypsin deficiency may develop chronic pancreatitis. The absence of antiprotease activity allows for proteolytic damage by trypsin and chymotrypsin.
Clinical features:
(1) evidence of chronic pancreatitis
(2) presence of alpha-1 antitrypsin deficiency
(3) exclusion of other causes for pancreatitis
(4) other manifestations of alpha-1 antitrypsin may be present (liver disease, emphysema, etc)
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