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hepatic A1AT deficiency

Saturday 21 July 2007

Definition: Hepatic anomalies in alpha1-antitrypsin deficiency (A1AT deficiency).


Small pink-red cytoplasmic spherical granular structures seen in alpha-1 antitrypsin 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.


- Histopathology of the liver in hepatic A1AT deficiency by Washington Deceit

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See also

- alpha1-antitrypsin deficiency (A1AT deficiency)