دورية
Liver pathology in genetic hemochromatosis: A review of 135 homozygous cases and their bioclinical correlations
| العنوان: | Liver pathology in genetic hemochromatosis: A review of 135 homozygous cases and their bioclinical correlations |
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| المؤلفون: | Deugnier, Yves M., Loréal, Olivier, Turlin, Bruno, Guyader, Dominique, Jouanolle, Hervé, Moirand, Romain, Jacquelinet, Christian, Brissot, Pierre |
| المصدر: | Gastroenterology; June 1992, Vol. 102 Issue: 6 p2050-2059, 10p |
| مستخلص: | Liver pathology was assessed in 135 patients with well-defined genetic hemochromatosis ranging from mild disease to severe overload. Three lesions were clearly linked to iron-overload intensity—scarse sidero-necrosis, mild inflammation, and progressive fibrosis. Iron-free foci made of typical or dysplastic hepatocytes were found in 7.4% of the cases. An original grading allowed a reliable quantification of iron and the study of cellular and lobular distribution of iron, which permitted (a) the accurate identification of a decreasing iron gradient in hepatocytes from zone 1 to zone 3 in all cases, (b) the definition of a threshold hepatocytic/mesenchymal iron ratio related to the appearance of sideronecrosis and to the development of fibrosis, and (c) demonstration that non-iron-related factors (mainly alcoholism) could shift iron from hepatocytes to sinusoidal cells without an increase in the total liver iron amount. This study provides a dynamic view of the iron overload process and suggests that sidero-necrosis and progressive sinusoidal iron overload play a role in the development of fibrosis in human genetic hemochromatosis. |
| قاعدة البيانات: | Supplemental Index |
Full Text Finder | تدمد: | 00165085 15280012 |
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| DOI: | 10.1016/0016-5085(92)90331-R |