True about alpha-1 antitrypsin deficiency, is are: A. Autosomal dominant
B. Pulmonary emphysema
C. Diastase resistant hepatic cells
D. Hepatic cells are orcein stain positive
E. Associated with berry aneurysm
B. Pulmonary emphysema
C. Diastase resistant hepatic cells
D. Hepatic cells are orcein stain positive
E. Associated with berry aneurysm
Ans. B. Pulmonary emphysema ; (C)
Diastase resistant hepatic cells :
•
alpha1-anti-trypsin deficiency,
is an autosomal recessive diseasemarked
by abnormally low levels of (serum) of this major protease inhibitor (Pi)
• Deficiency of the enzyme leads to pulmonary emphysema.
• It is characterised by
— Presence of round to oval cytoplasmic globular inclusions in hepatocytes
which on H and E stains show acidophilic and indistinctly demarcated from
surrounding cytoplasm. In most part, only distinctive feature is globules. Infrequently-fatty
change, mallory bodies
