Hemochromatosis-Associated Mortality in the United States from 1979 to 1992: An Analysis of Multiple-Cause Mortality Data

doi:10.1059/0003-4819-129-11_Part_2-199812011-00005

Hemochromatosis-Associated Mortality in the United States from 1979 to 1992: An Analysis of Multiple-Cause Mortality Data

Quanhe Yang, PhD;
Sharon M. McDonnell, MD, MPH;
Muin J. Khoury, MD, PhD;
Joanne Cono, MD, ScM; and
R. Gibson Parrish, MD

From the Centers for Disease Control and Prevention and Egleston Children's Hospital at Emory University, Atlanta, Georgia. Previously presented in part at Prevention 97 in Atlanta, Georgia, 20-23 March 1997. Acknowledgments: The authors thank Drs. J. David Erickson, David Mannino, Kyle Steenland, and Mary E. Cogswell for helpful comments and technical assistance. Requests for Reprints: Sharon M. McDonnell, MD, MPH, Division of International Health, Centers for Disease Control and Prevention, Mailstop C-08, 1600 Clifton Road, Atlanta, GA 30333; e-mail, sem0{at}cdc.gov. Current Author Addresses: Dr. Yang: Division of Birth Defects and Developmental Disabilities, National Center for Environmental Health, Centers for Disease Control and Prevention, Mailstop F-45, 4770 Buford Highway, Atlanta, GA 30341. Dr. McDonnell: Division of International Health, Centers for Disease Control and Prevention, Mailstop C-08, 1600 Clifton Road, Atlanta, GA 30333. Dr. Khoury: Office of Genetics and Disease Prevention, Centers for Disease Control and Prevention, Mailstop K-28, 4770 Buford Highway, Atlanta, GA 30341. Dr. Cono: Egleston Children's Hospital at Emory University, Department of General Pediatrics, 311 St. Paul Avenue, Atlanta, GA 30312. Dr. Parrish: Division of Environmental Hazards and Health Effects, National Center for Environmental Health, Centers for Disease Control and Prevention, Mailstop F-47, 4770 Buford Highway, Atlanta, GA 30341. Note: This article is one of a series of articles comprising an Annals of Internal Medicine supplement entitled “Iron Overload, Public Health, and Genetics.” To view a complete list of the articles included in this supplement, please view its Table of Contents.

Abstract

Background: Hemochromatosis, which can lead to serious chronic diseases resulting from iron overload, has an estimated prevalence of 50 to 80 cases per 10 000 persons. However, little population-based information is available on the impact of hemochromatosis on morbidity and mortality.

Objective: To evaluate trends over 14 years in deaths and medical conditions associated with hemochromatosis in the United States.

Design: We searched Multiple-Cause Mortality Files compiled by the National Center for Health Statistics for the years 1979 to 1992 for all records listing hemochromatosis. We used these data to calculate age-adjusted and age-specific mortality rates, identify medical conditions associated with a known diagnosis of hemochromatosis at death, and calculate proportionate mortality ratios for these medical conditions.

Results: The listing of hemochromatosis on death certificates increased 60% from 1979 to 1992. Decedents with hemochromatosis were 23, 13, and 5 times more likely to have liver neoplasms, liver disease, and cardiomyopathy, respectively, than were decedents without hemochromatosis. Conversely, decedents with liver neoplasms, liver disease, and cardiomyopathy were 26, 14, and 5 times more likely, respectively, to have hemochromatosis than were decedents without these conditions. Hemochromatosis was 82 times more likely in persons with the combination of liver neoplasms and diabetes and 43 times more likely in those with the combination of liver disease and diabetes than in those without these conditions.

Conclusions: Comparison of the reported prevalence of hemochromatosis among decedents with estimates of prevalence in the general U.S. population suggests that either the penetrance or the recognition of hemochromatosis, or both, is low. Nevertheless, substantial mortality resulting from liver disease, liver neoplasms, cardiomyopathy, and a combination of liver disease and diabetes in patients with hemochromatosis argues for the improved diagnosis and treatment of hemochromatosis in persons with these conditions.