The relationship between alcoholism and hereditary hemochromatosis performed before the use of these dihemochromatosis remains controversial. Previous stud-agnostic tools most likely included patients with alcoies have included patients with alcoholic siderosis holic siderosis rather than hereditary hemochromatorather than hereditary hemochromatosis. In this retro-sis. [3][4][5][6][7][8][9] In this study, we have re-examined the spective study, the clinical features, iron status, alcohol prevalence of alcohol use in hereditary hemochromatohistory, liver histology, and long-term survival were re-sis using rigid criteria for the diagnosis, including the viewed in 105 homozygotes for hemochromatosis using presence of an HLA identical sibling with hemochromarigid diagnostic criteria including an HLA identical sib-tosis.ling with iron overload. Heavy alcohol consumption (ú80 g ethanol/day) was found in 15% of hemochromato-PATIENTS AND METHODS sis patients. Histological features of alcoholic liver disease (Mallory's hyaline bodies, pericentral fibrosis, polyPatient records were reviewed from a large database of 170 morphonuclear infiltrate, and fatty infiltration) were hemochromatosis homozygotes that have been documented uncommon in hemochromatosis. Hemochromatosis pa-since 1965. Family investigations at this center ranged from tients with heavy alcohol consumption had a higher no further investigation in the case of isolated proband cases prevalence of cirrhosis than hemochromatosis patients to extensive pedigree analysis involving up to 45 family memwithout heavy alcohol consumption. Hepatic iron con-bers of a single family over multiple generations. Data was centration and hepatic iron index did not significantly available on 483 family members assessed at this tertiary differ between these two hemochromatosis groups. referral center for possible hemochromatosis. The clinical feaLong-term survival was significantly reduced in patients tures of this patient cohort has been previously described. [10][11][12] with heavy alcohol consumption (mean follow-up, 9.22 From this database, homozygotes were selected that had an years). This suggests that chronic alcohol consumption HLA identical sibling with hemochromatosis to establish a has an additive hepatotoxic effect despite the paucity of hereditary disorder of iron metabolism (n Å 105). There were histological features of alcoholic liver disease. (HEPATOL-65 homozygotes excluded from this study because no HLA OGY 1996;23:724-727. ) identical sibling had been identified. The diagnosis was confirmed by liver biopsy results in all probands and most discovered siblings (n Å 89/105). No hemochromatosis patients hadThe clinical distinction between patients with heredconcomitant chronic viral hepatitis B or C on histological itary hemochromatosis and alcoholic siderosis contin-examination of the liver biopsy specimen. Liver biopsy reues to be a diagnostic problem. Clinical tools that have ports were reviewed for the presence of Mallory's hyaline, improved this diagnostic di...