A patient with the MZ alpha-1 antitrypsin (AAT) phenotype is at increased risk for chronic liver disease.


Forms of liver disease that may become chronic and progress to cirrhosis in patients with the MZ phenotype:

(1) nonalcoholic steatohepatitis (NASH)

(2) alcoholic hepatitis

(3) autoimmune hepatitis

(4) chronic active viral hepatitis

(5) methotrexate-induced liver disease

(6) cryptogenic liver disease


Complications of the chronic liver disease:

(1) hepatic failure

(2) hepatocellular carcinoma



(1) A person who develops unexplained chronic liver disease should be phenotyped for alpha-1 antitrypsin.

(2) A patient with the MZ phenotype should not be exposed to potentially hepatotoxic drugs or chemicals.

(3) A patient with the MZ phenotype who develops nonalcoholic steatohepatitis (NASH) should aggressively try to reverse the metabolic factors.


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