Iron overload and cirrhosis in referred

HFE p.C282Y homozygosity cirrhosis hemochromatosis transferrin saturation

Journal

Canadian liver journal
ISSN: 2561-4444
Titre abrégé: Can Liver J
Pays: Canada
ID NLM: 101778326

Informations de publication

Date de publication:
2020
Historique:
received: 28 05 2019
accepted: 04 08 2019
entrez: 22 8 2022
pubmed: 4 6 2020
medline: 4 6 2020
Statut: epublish

Résumé

levated transferrin saturation (TS) is an imperfect test to identify adults with high-iron gene ( e analyzed observations of non-screening, previously untreated p.C282Y homozygotes who presented with both normal TS (<50% men, <45% women) and elevated serum ferritin (SF; men, >300 µg/L; women, >200 µg/L). Iron overload was defined as hepatocyte iron grade 3 or 4, liver iron >35 µmol/g dry weight, or iron removed by phlebotomy ≥3 g. Cirrhosis was defined as regenerating nodules of hepatocytes surrounded by bands of fibrous connective tissue. mong 917 referred p.C282Y homozygotes, 58 (33 men, 25 women) had normal TS and elevated SF (6.3% [95% CI 4.9% to 8.1%]). Of 58 patients, 14 (24.1%) underwent liver biopsy; all 14 had hepatocyte iron grade 3 or 4. Fatty infiltration was reported in 6 of 14 liver biopsies (42.9%). Liver iron was >35 µmol/g dry weight in 7 of 8 patients tested (87.5%). Iron removed by phlebotomy was ≥3 g in 75.0% (15/20) of men and 62.5% (5/8) of women. Of 58 patients, 3 (5.2%) had iron overload and cirrhosis; each also had a proven or possible non-iron liver condition that may have acted in synergy with liver iron to increase cirrhosis risk. Iron overload is common in non-screening, previously untreated

Sections du résumé

Background UNASSIGNED
levated transferrin saturation (TS) is an imperfect test to identify adults with high-iron gene (
Methods UNASSIGNED
e analyzed observations of non-screening, previously untreated p.C282Y homozygotes who presented with both normal TS (<50% men, <45% women) and elevated serum ferritin (SF; men, >300 µg/L; women, >200 µg/L). Iron overload was defined as hepatocyte iron grade 3 or 4, liver iron >35 µmol/g dry weight, or iron removed by phlebotomy ≥3 g. Cirrhosis was defined as regenerating nodules of hepatocytes surrounded by bands of fibrous connective tissue.
Results UNASSIGNED
mong 917 referred p.C282Y homozygotes, 58 (33 men, 25 women) had normal TS and elevated SF (6.3% [95% CI 4.9% to 8.1%]). Of 58 patients, 14 (24.1%) underwent liver biopsy; all 14 had hepatocyte iron grade 3 or 4. Fatty infiltration was reported in 6 of 14 liver biopsies (42.9%). Liver iron was >35 µmol/g dry weight in 7 of 8 patients tested (87.5%). Iron removed by phlebotomy was ≥3 g in 75.0% (15/20) of men and 62.5% (5/8) of women. Of 58 patients, 3 (5.2%) had iron overload and cirrhosis; each also had a proven or possible non-iron liver condition that may have acted in synergy with liver iron to increase cirrhosis risk.
Conclusions UNASSIGNED
Iron overload is common in non-screening, previously untreated

Identifiants

pubmed: 35991852
doi: 10.3138/canlivj.2019-0013
pmc: PMC9202789
doi:

Types de publication

Journal Article

Langues

eng

Pagination

188-193

Informations de copyright

Copyright © 2020 Canadian Association for the Study of the Liver.

Déclaration de conflit d'intérêts

The authors have nothing to declare.

Références

J Hepatol. 2010 Jul;53(1):3-22
pubmed: 20471131
Blood. 2003 May 1;101(9):3359-64
pubmed: 12521995
Hepatology. 2011 Jul;54(1):328-43
pubmed: 21452290
Blood. 2011 Apr 28;117(17):4590-9
pubmed: 21355094
J Pathol Bacteriol. 1962 Jul;84:53-64
pubmed: 14498313
Am J Med. 2007 Nov;120(11):999.e1-7
pubmed: 17976429
JAMA. 2013 Nov 27;310(20):2191-4
pubmed: 24141714
Am J Hematol. 2009 Nov;84(11):710-4
pubmed: 19787796
Lancet. 1997 Jan 11;349(9045):95-7
pubmed: 8996422
Cell Metab. 2005 Mar;1(3):191-200
pubmed: 16054062
Am J Hum Genet. 2009 Jan;84(1):60-5
pubmed: 19084217
N Engl J Med. 2005 Apr 28;352(17):1769-78
pubmed: 15858186
J Med Genet. 2013 Sep;50(9):593-8
pubmed: 23794717
Am J Med Sci. 2009 May;337(5):386-90
pubmed: 19440063
Nat Genet. 2009 Nov;41(11):1173-5
pubmed: 19820699
Nat Genet. 2009 Nov;41(11):1170-2
pubmed: 19820698
Am J Hum Genet. 2000 Apr;66(4):1246-58
pubmed: 10739755
Clin Gastroenterol Hepatol. 2012 Apr;10(4):412-6
pubmed: 22265917
Clin Chem. 2005 Jun;51(6):1048-52
pubmed: 15833784
Ann Hepatol. 2018 Aug 24;17(5):871-879
pubmed: 30145563
Blood Cells Mol Dis. 1999 Oct-Dec;25(5-6):374-9
pubmed: 10660486
Genet Test. 2005 Fall;9(3):231-41
pubmed: 16225403
BMC Dermatol. 2005 Nov 29;5:12
pubmed: 16316460
Blood. 1989 Nov 1;74(6):2187-93
pubmed: 2804357
Blood Cells Mol Dis. 2001 Sep-Oct;27(5):783-802
pubmed: 11783942
Liver Int. 2011 Feb;31(2):272-3
pubmed: 20738776

Auteurs

Paul C Adams (PC)

Department of Medicine, University Hospital, Western University, London, Ontario.

James C Barton (JC)

Southern Iron Disorders Center, Birmingham, Alabama, USA.
Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.

Classifications MeSH