Utility of hepatic iron index in American patients with hereditary hemochromatosis: a multicenter study.
OBJECTIVE
A hepatic iron index (hepatic iron concentration divided by age) of more than 1.9 has been proposed as useful to identify patients with homozygous hereditary hemochromatosis (HHC). There are limited data on the diagnostic use of the hepatic iron index in patients with HHC in the United States. This study evaluated the hepatic iron index in the diagnosis of HHC in a multicenter U.S. study.


METHODS
Hepatic iron concentration was measured in 509 patients undergoing liver biopsy. The diagnosis of HHC was made using clinical, biochemical, and histopathologic criteria.


RESULTS
Fifty-five patients met criteria for HHC; hepatic iron index was > 1.9 in 51 of 55 (93%) patients with HHC but in none of 454 patients with other liver diseases; hepatic iron concentration was > 71 mumol/g dry weight in 54 of 55 patients with HHC but only 1 of the other 454 patients.


CONCLUSIONS
A hepatic iron index of > or = 1.9 can identify most U.S. patients with HHC but is < or = 1.9 in 7%. A "threshold" hepatic iron concentration of 71 mumol/g can almost always distinguish patients with HHC from patients with other liver diseases and may be a useful adjunct to the hepatic iron index in the diagnosis of HHC in the diverse U.S. population.
Kowdley
K V
KV
Department of Medicine, University of Washington School of Medicine, Seattle, USA. kkowdley@u.washington.edu
Trainer
T D
TD
Saltzman
J R
JR
Pedrosa
M
M
Krawitt
E L
EL
Knox
T A
TA
Susskind
K
K
Pratt
D
D
Bonkovsky
H L
HL
Grace
N D
ND
Kaplan
M M
MM
D000328:Adult; D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D005293:Ferritins / Q000032:analysis; D006432:Hemochromatosis / Q000175:diagnosis* / Q000235:genetics* / Q000473:pathology; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007501:Iron / Q000032:analysis* / Q000097:blood; D008099:Liver / Q000737:chemistry* / Q000473:pathology; D008107:Liver Diseases / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D015203:Reproducibility of Results; D014168:Transferrin / Q000032:analysis; D014481:United States
D014168:Transferrin; D005293:Ferritins; D007501:Iron