Iron overload in cirrhosis -: HFE genotypes and outcome after liver transplantation

被引:61
作者
Brandhagen, DJ
Alvarez, W
Therneau, TM
Kruckeberg, KE
Thibodeau, SN
Ludwig, J
Porayko, MK
机构
[1] Thomas Jefferson Med Ctr, Philadelphia, PA 19107 USA
[2] Mayo Clin & Mayo Fdn, Rochester, MN 55905 USA
关键词
D O I
10.1002/hep.510310227
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Previously, we found appreciable hepatic iron deposition in one third of our patients undergoing liver transplantation (LTx) with approximately 10% of cases having quantifiable iron in the range of that seen in hereditary hemochromatosis (HHC). The aim of this study was to compare clinical outcome in liver transplant patients with and without iron overload. We also sought to determine the prevalence of HFE mutations in liver transplant patients with iron overload. Of 456 consecutive liver transplants, 41 explants had an hepatic iron index (HII) greater than 1.9, and these cases were compared to 41 matched liver transplant recipients without increased hepatic iron. Posttransplantation complications, along with patient and graft survival were monitored. HFE gene testing was performed using DNA-based techniques. Kaplan-Meier 5-year patient survival after LTx was significantly lower in cases with hepatic iron overload compared to matched controls without iron excess (48% vs. 77%; P = .045), Fatal infections (especially fungal) were more common in patients with iron overload (24% vs. 7%; P = .03). Of the 41 patients with a liver explant HII greater than 1.9, only 4 were C282Y homozygotes. Patients with severe hepatic explant iron overload undergoing LTx have a reduced survival compared to liver transplant recipients without explant iron excess. The reduced survival was attributable mainly to fatal bacterial and fungal infections. Despite the iron overload, HFE gene mutations were uncommon in patients with hepatic explant hemosiderosis.
引用
收藏
页码:456 / 460
页数:5
相关论文
共 50 条
  • [1] BALLART IJ, 1986, BLOOD, V67, P105
  • [2] Genetic and clinical description of hemochromatosis probands and heterozygotes: Evidence that multiple genes linked to the major histocompatibility complex are responsible for hemochromatosis
    Barton, JC
    Shih, WWH
    SawadaHirai, R
    Acton, RT
    Harmon, L
    Rivers, C
    Rothenberg, BE
    [J]. BLOOD CELLS MOLECULES AND DISEASES, 1997, 23 (08) : 135 - 145
  • [3] VALUE OF HEPATIC IRON MEASUREMENTS IN EARLY HEMOCHROMATOSIS AND DETERMINATION OF THE CRITICAL IRON LEVEL ASSOCIATED WITH FIBROSIS
    BASSETT, ML
    HALLIDAY, JW
    POWELL, LW
    [J]. HEPATOLOGY, 1986, 6 (01) : 24 - 29
  • [4] Beutler Ernest, 1996, Blood Cells Molecules and Diseases, V22, P187, DOI 10.1006/bcmd.1996.0027
  • [5] Mutations in the MHC class I-like candidate gene for hemochromatosis in French patients
    Borot, N
    Roth, MP
    Malfroy, L
    Demangel, C
    Vinel, JP
    Pascal, JP
    Coppin, H
    [J]. IMMUNOGENETICS, 1997, 45 (05) : 320 - 324
  • [6] Iron overload in cirrhosis -: HFE genotypes and outcome after liver transplantation
    Brandhagen, DJ
    Alvarez, W
    Therneau, TM
    Kruckeberg, KE
    Thibodeau, SN
    Ludwig, J
    Porayko, MK
    [J]. HEPATOLOGY, 2000, 31 (02) : 456 - 460
  • [7] Prevalence of HFE gene mutations in normal blood donors and in patients with iron overload.
    Brandhagen, DJ
    Fairbanks, VF
    Baldus, WP
    Smith, CI
    Kruckeberg, KE
    Thibodeau, SN
    [J]. GASTROENTEROLOGY, 1998, 114 (04) : A1214 - A1214
  • [8] THE IMMUNE-SYSTEM IN HEREDITARY HEMOCHROMATOSIS - A QUANTITATIVE AND FUNCTIONAL ASSESSMENT OF THE CELLULAR ARM
    BRYAN, CF
    LEECH, SH
    KUMAR, P
    GAUMER, R
    BOZELKA, B
    MORGAN, J
    [J]. AMERICAN JOURNAL OF THE MEDICAL SCIENCES, 1991, 301 (01) : 55 - 61
  • [9] HEMOCHROMATOSIS, IRON, AND SEPTICEMIA CAUSED BY VIBRIO-VULNIFICUS
    BULLEN, JJ
    SPALDING, PB
    WARD, CG
    GUTTERIDGE, JMC
    [J]. ARCHIVES OF INTERNAL MEDICINE, 1991, 151 (08) : 1606 - 1609
  • [10] CANTINIEAUX B, 1987, EUR J HAEMATOL, V39, P28