Liver transplantation normalizes serum hepcidin level and cures iron metabolism alterations in HFE hemochromatosis. - Université de Rennes Accéder directement au contenu
Article Dans Une Revue Hepatology Année : 2014

Liver transplantation normalizes serum hepcidin level and cures iron metabolism alterations in HFE hemochromatosis.

Résumé

Background: Defects in human hemochromatosis protein (HFE) cause iron overload due to reduced hepatic hepcidin secretion. Liver transplantation (LT) is a key treatment for potential complications from HFE- related hereditary hemochromatosis (HH). This study evaluated hepcidin secretion and iron burden after LT to elucidate HH pathophysiology. Methods: Patients (n=18) homozygous for the p.Cys282Tyr mutation in the HFE gene underwent LT between 1999 and 2008. Serum iron, serum hepcidin, and hepatic iron concentrations were determined before LT and at the end of follow-up (median 57 months). Mortality and causes of death were determined. Survival was compared to that of the overall patient population that received LT. Results: Before LT, serum hepcidin levels were low (0.54 ± 2.5 nmol/L; normal range: 4-30 nmol/L). After LT, 11 patients had iron evaluations; none received iron depletion therapy; all had normal transferrin saturation. The mean serum ferritin was 185 (±99) µg/L. Magnetic resonance imaging showed that iron overload was absent in 9 patients, mild in one patient with metabolic syndrome, and high (180 µmol/g) in one patient with hereditary spherocytosis unmasked after liver transplantation. At the end of follow-up, serum hepcidin was normal in 10 patients (11.12 ± 7.6 nmol/L; p<0.05) and low in one patient with iron deficiency anemia. Survival was 83% and 67% at one and 5 years, respectively. Survival was similar for patients with HH and patients that received LT for other causes. Conclusion: This study demonstrated that, in HH, LT normalized hepcidin secretion and prevented recurrence of hepatic iron overload. Survival was similar to that of patients that received LTs for other liver diseases. (HEPATOLOGY 2013.).
Fichier principal
Vignette du fichier
Liver_transplantation-13-0526-hemoTH_Revision_final.pdf (320.43 Ko) Télécharger le fichier
Origine : Fichiers produits par l'(les) auteur(s)
Loading...

Dates et versions

hal-00876626 , version 1 (05-09-2014)

Identifiants

Citer

Edouard Bardou-Jacquet, Julie Philip, Richard Lorho, Martine Ropert, Marianne Latournerie, et al.. Liver transplantation normalizes serum hepcidin level and cures iron metabolism alterations in HFE hemochromatosis.. Hepatology, 2014, 59 (3), pp.839-847. ⟨10.1002/hep.26570⟩. ⟨hal-00876626⟩
343 Consultations
1395 Téléchargements

Altmetric

Partager

Gmail Facebook X LinkedIn More