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Ultrastructural Liver Pathology in Patients with Minimal Liver Disease and α 1 ‐Antitrypsin Deficiency: A Comparison Between Heterozygous and Homozygous Patients
Author(s) -
Hultcrantz Rolf,
Mengarelli Silva
Publication year - 1984
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.1840040526
Subject(s) - endoplasmic reticulum , pathology , lipofuscin , parenchyma , liver disease , biology , kupffer cell , alpha 1 antitrypsin deficiency , peroxisome , liver cell , pathogenesis , cell type , ultrastructure , cell , medicine , biochemistry , immunology , gene
Abstract A light and electron microscopic investigation of liver tissue from three homozygous (Pi‐type ZZ) and three heterozygous (Pi‐type MZ) α 1 ‐antitrypsin deficiency patients is presented. All had slightly elevated levels of serum amino transferases as the only signs of liver damage. Light microscopical investigation showed minor periportal fibrosis and periodic acid‐Schiff‐positive globules in the homozygous patients, but no specific findings besides fatty changes were seen in the heterozygous patients. Electron microscopical investigation was performed on periportal and centrilobular areas separately. In the three homozygous patients, dilatation of endoplasmic reticulum was found in many periportal liver parenchymal cells. In these cells, a marked increase in peroxisomes was also noted and in some instances they showed signs of cell necrosis. Evidence of Rupffer cell heterophagy of such cells was also found, thus indicating that they had died. Only in one liver parenchymal cell from one of the three heterozygous patients was evidence of a dilated endoplasmic reticulum found. No increase in peroxisomes was found and no apparent signs of liver cell necrosis were noted. An increased amount of lipofuscin‐like material in the lysosomes was encountered in liver cells both from patients with homozygous and heterozygous ai‐antitrypsin deficiency. Based on these findings, it is suggested that cell death occurs in cells with a dilatation of the endoplasmic reticulum. If these findings are important for the pathogenesis in liver disease in α 1 ‐antitrypsin deficiency, the present study suggests that the risk to develop liver disease is less in heterozygous patients since, with one exception, alterations of the same nature were not found in their hepatocytes.