title: Liver fibrosis in genetic hemochromatosis. Respective roles of iron and non-iron-related factors in 127 homozygous patients.
abstract: A retrospective study of 127 patients with untreated homozygous genetic hemochromatosis (HGH) was conducted to evaluate the respective roles of iron overload and non-iron-related factors in the development of hepatic fibrosis in HGH. Twenty-seven percent of the patients had cirrhosis, 21% had liver fibrosis and 52% had no fibrosis (prefibrotic group). The mean value of liver iron concentration was increased significantly (p < 0.001) in cirrhotic (378 +/- 144 mumol/g dry wt.) and in fibrotic (331 +/- 168) subjects compared to prefibrotic (237 +/- 108) patients. Of 13 patients with liver iron concentration > or = 500, 12 had liver fibrosis or cirrhosis, versus 48/134 with liver iron concentration < 500. Chronic alcoholic men exhibited hepatic fibrosis or cirrhosis more frequently than non-alcoholic men (p < 0.001). Non-alcoholic men had hepatic fibrosis or cirrhosis more often than non-alcoholic women (p < 0.05). Cirrhotic and fibrotic patients were significantly older than prefibrotic patients whilst a significant correlation between age and liver iron concentration was found in younger patients only. These results suggest that the iron overload threshold necessary to induce fibrosis is modulated by non-iron-related factors such as alcoholism, sex and age. The development of fibrosis in HGH with liver iron concentration < 500 mumol/g is frequent and must lead to a search for associated non-iron-related fibrogenic factors.
authors: Clinique des Maladies du Foie, CHRU Pontchaillou, Rennes, France.
authors: O
authors: O
authors: Loréal
authors: Y
authors: Y
authors: Deugnier
authors: R
authors: R
authors: Moirand
authors: L
authors: L
authors: Lauvin
authors: D
authors: D
authors: Guyader
authors: H
authors: H
authors: Jouanolle
authors: B
authors: B
authors: Turlin
authors: G
authors: G
authors: Lescoat
authors: P
authors: P
authors: Brissot
mesh_terms: D000328:Adult; D000367:Age Factors; D000437:Alcoholism / Q000150:complications; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006432:Hemochromatosis / Q000150:complications / Q000235:genetics*; D006509:Hepatitis B / Q000150:complications; D006579:Heterozygote; D006801:Humans; D007501:Iron / Q000378:metabolism / Q000502:physiology*; D008099:Liver / Q000378:metabolism; D008103:Liver Cirrhosis / Q000139:chemically induced / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012727:Sex Characteristics
chemical_list: D006680:HLA Antigens; D007501:Iron