Literature DB >> 27723100

How we manage patients with hereditary haemochromatosis.

Eva Rombout-Sestrienkova1,2, Marian G J van Kraaij2, Ger H Koek1.   

Abstract

A number of disorders cause iron overload: some are of genetic origin, such as hereditary haemochromatosis, while others are acquired, for instance due to repeated transfusions. This article reviews the treatment options for hereditary haemochromatosis, with special attention to the use of erythrocytapheresis. In general, therapy is based on the removal of excess body iron, for which ferritin levels are used to monitor the effectiveness of treatment. For many decades phlebotomy has been widely accepted as the standard treatment. Recent publications suggest that erythrocytapheresis, as a more individualized treatment, can provide a good balance between effectiveness, tolerability and costs. Other treatments like oral chelators and proton pomp inhibitors, which are used in selected patients, create the possibility to further individualize treatment of hereditary haemochromatosis. In the future, hepcidin-targeted therapy could provide a more fundamental approach to treatment.
© 2016 John Wiley & Sons Ltd.

Entities:  

Keywords:  erythrocytapheresis; hereditary haemochromatosis; phlebotomy; treatment

Mesh:

Substances:

Year:  2016        PMID: 27723100     DOI: 10.1111/bjh.14376

Source DB:  PubMed          Journal:  Br J Haematol        ISSN: 0007-1048            Impact factor:   6.998


  10 in total

Review 1.  The mechanisms of systemic iron homeostasis and etiology, diagnosis, and treatment of hereditary hemochromatosis.

Authors:  Hiroshi Kawabata
Journal:  Int J Hematol       Date:  2017-11-13       Impact factor: 2.490

Review 2.  Oxidative Stress in β-Thalassemia.

Authors:  Eitan Fibach; Mutaz Dana
Journal:  Mol Diagn Ther       Date:  2019-04       Impact factor: 4.074

Review 3.  Excess iron: considerations related to development and early growth.

Authors:  Marianne Wessling-Resnick
Journal:  Am J Clin Nutr       Date:  2017-10-25       Impact factor: 7.045

4.  Aspartate/asparagine-β-hydroxylase: a high-throughput mass spectrometric assay for discovery of small molecule inhibitors.

Authors:  Lennart Brewitz; Anthony Tumber; Inga Pfeffer; Michael A McDonough; Christopher J Schofield
Journal:  Sci Rep       Date:  2020-05-26       Impact factor: 4.379

5.  A case report of hereditary hemochromatosis caused by mutation of SLC40A1 gene.

Authors:  Xin Yin; Yu Zhang; Hui Gao; Qing-Long Jin; Xiao-Yu Wen
Journal:  Medicine (Baltimore)       Date:  2019-11       Impact factor: 1.817

6.  Runx3 regulates iron metabolism via modulation of BMP signalling.

Authors:  Hyun-Yi Kim; Jong-Min Lee; You-Soub Lee; Shujin Li; Seung-Jun Lee; Suk-Chul Bae; Han-Sung Jung
Journal:  Cell Prolif       Date:  2021-10-06       Impact factor: 6.831

Review 7.  Pathophysiology and treatment of patients with beta-thalassemia - an update.

Authors:  Eitan Fibach; Eliezer A Rachmilewitz
Journal:  F1000Res       Date:  2017-12-20

8.  Hemochromatosis, Erythrocytosis and the JAK2 p.V617F Mutation.

Authors:  Stephen E Langabeer
Journal:  EJIFCC       Date:  2017-03-08

Review 9.  Tuning the Anti(myco)bacterial Activity of 3-Hydroxy-4-pyridinone Chelators through Fluorophores.

Authors:  Maria Rangel; Tânia Moniz; André M N Silva; Andreia Leite
Journal:  Pharmaceuticals (Basel)       Date:  2018-10-20

10.  A predictive model for estimating the number of erythrocytapheresis or phlebotomy treatments for patients with naïve hereditary hemochromatosis.

Authors:  Eva Rombout-Sestrienkova; Bjorn Winkens; Marian van Kraaij; Cees Th B M van Deursen; Mirian C H Janssen; Alexander M J Rennings; Dorothea Evers; Jean-Louis Kerkhoffs; Ad Masclee; Ger H Koek
Journal:  J Clin Apher       Date:  2020-12-24       Impact factor: 2.821

  10 in total

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