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Pathology of Vascular Diseases of the Liver References /liver.html /WebPath/ Questions? * * * * * * * * * This is a case of primary biliary cirrhosis, a rare autoimmune disease (mostly of middle-aged women) that is characterized by destruction of bile ductules within the triads of the liver. Antimitochondrial antibody can be detected in serum. Seen here in a portal tract is an intense chronic inflammatory infiltrate with loss of bile ductules. Micronodular cirrhosis ensues. * Antimitochondrial antibody can be detected in serum * One of the earliest visible changes seen in the liver following extrahepatic obstruction is bile stasis. This is typically most severe in the centrilobular areas. The bile pigment is present as fine granules in the cytoplasm of hepatocytes and as very prominent bile plugs in the biliary canaliculi. Note that the biliary canaliculi between the hepatocytes are stuffed with dark green bile pigment. * The pale golden brown finely granular pigment seen here in nearly all hepatocytes is lipchrome (lipofuscin). One such deposit within a hepatocyte is marked by the arrow. This is a wear and tear pigment from the accumulation of autophagolysosomes over time. This pigment is of no real pathologic importance. * Fig 30 - ALPHA-1-ANTITRYPSIN STORAGE: The periportal hepatocytes contain very visible citoplasmic inclusions that are diastase resistant PAS positive and can be identified with immunoperoxidase method as alpa-1-antitrypsin (A1AT) material. Diastase -PAS stain should be done routinely in liver biopsies. The inclusions are almost always present in homozygous (ZZ) or heterozygous (MZ,SZ) phenotypes of A1AT deficiency cases. Non-alpha-1-antitrypsin similar inclusions are occasinally seen in other conditions,such as:post mortem material and biopsy material in cirrhosis. Immunoperoxidase reaction for A1AT will solve the problem. Fibrinogen storage also may mimic A1AT inclusions.It can be detected with immunoperoxidase reaction. Glycogen storag
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