hepatic A1AT deficiency
Definition: Hepatic anomalies in alpha1-antitrypsin deficiency (A1AT deficiency).
The inclusions caused by the Z form of 1-antitrypsin are periodic acid Schiff (PAS) positive and diastase resistant and are associated with neonatal hepatitis and hepatocellular carcinoma.
Electron micrograph shows the accumulation of 1-antitrypsin in the rough endoplasmic reticulum. These inclusions are composed of chains of 1-antitrypsin polymers.
Videos
Histopathology of the liver in hepatic A1AT deficiency by Washington Deceit
See also
alpha1-antitrypsin deficiency (A1AT deficiency)