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Copper Accumulates in Hemosiderins in Livers of Patients with Iron Overload Syndromes

Journal of Clinical and Translational Hepatology 2015年第2期3卷 85-92页

作者： Yukiya Ono Masatoshi Ishigami Kazuhiko Hayashi Shinya Wakusawa Hisao Hayashi Kotaro Kumagai Natsuko Morotomi Tetsuji Yamashita Miwa Kawanaka Minemori Watanabe Hiroaki Ozawa Mayumi Tai Hiroaki Miyajima Kentarou Yoshioka Yoshiki Hirooka Hidemi Goto Department of Gastroenterology Nagoya University Graduate School of Medicine Nagoya Japan Department of Medical Laboratory Sciences Nagoya University Graduate School of Medicine Nagoya Japan Department of Medicine Aichi Gakuin University School of Pharmacy Nagoya Japan Digestive Disease and Lifestyle Related Disease Kagoshima University Graduate School of Medical and Dental Sciences Kagoshima Japan Department of Internal Medicine Moji Hospital Kitakyushu Japan Department of Medicine Yoshida Hospital Matsuyama Japan General Internal Medicine 2 Kawasaki Hospital Kawasaki Medical School Okayama Japan Department of Endocrinology and Diabetes Okazaki City Hospital Okazaki Japan Department of Pathology Okazaki City Hospital Okazaki Japan Department of Gastroenterology Fukushima Rohsai Hospital Fukushima Japan First Department of Medicine Hamamatsu University School of Medicine Hamamatsu Japan Department of Liver Biliary Tract and Pancreas Diseases Fujita Health University Toyoake Japan

In biology,redox reactions are essential and sometimes harmful,and therefore,iron metabolism is tightly regulated by *** the state of copper in iron overload syndromes remains unclear,we investigated whether copper metabolism is altered in these *** patients with iron overload syndromes participated in this *** clinical diagnoses were aceruloplasminemia (n=2),hemochromatosis (n=5),ferroportin disease (n=2),and receiving excess intravenous iron supplementation (n=2).Liver specimens were analyzed using a light microscope and transmission electron microscope equipped with an X-ray *** addition to a large amount of iron associated with oxygen and phosphorus,the iron-rich hemosiderins of hepatocytes and Kupffer cells contained small amounts of copper and sulfur,regardless of disease ***-dimensional imaging clearly showed that cuproproteins were distributed homogenously with iron complexes within *** stasis was unlikely in noncirrhotic *** enhanced induction of cuproproteins by excess iron may contribute to copper accumulation in *** conclusion,we have demonstrated that copper accumulates in hemosiderins in iron overload conditions,perhaps due to alterations in copper metabolism.

关键词： Aceruloplasminemia Cuproprotein induction Ferroportin disease hemochromatosis Hemosiderin Iron copper interaction Iron overload syndromes

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Hepatocellular carcinoma in thalassemia:A critical review

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World Journal of Hepatology 2010年第5期2卷 171-174页

作者： Andrea Mancuso Emergency Area Ospedali Riuniti di Sciacca(Ag)

Due to blood transfusions,thalassemics are often infected with either hepatitis C virus(HCV)or hepatitis B virus and often have *** carcinoma(HCC)has emerged in thalassemics only recently as a result of the improvement in thalassemia *** fact,a prospective study estimated an HCC incidence inβ-thalassemia of about 2%.Although data are scanty,HCC screening in thalassemics with risk factors for HCC should be carried *** treatments have some efficacy in HCV infected thalassemics despite partial contraindication to ribavirin and iron ***,there are no data on how HCV treatment translates into HCC *** data suggest that HCC treatment in thalassemics should generally have the same outcomes as in *** coexistence of severe comorbidities makes liver transplantation challenging,this therapeutic possibility should not be precluded for well selected HCCβ-thalassemia *** fact,2 transfusion dependent adult HCCβ-thalassemia patients have recently undergone successful liver transplantation with a good *** conclusion,HCC seems to be a developing issue in thalassemia and HCC screening should be carried *** treatment,including liver transplantation,can be performed in selected patients. A multidisciplinary effort is needed for management.

关键词： Thalassemia Hepatocellular carcinoma hemochromatosis Screening Complication Liver trans- plantation

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青铜色糖尿病(原发性血色病)——附三例家系调查报告

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西安交通大学学报(医学版) 1986年第1期 45-47页

作者：戴信刚程秦娣叶复来谈一飞冯学亮郭仁舆西安医科大学第二附属医院内科西安医科大学第一附属医院内科西安医科大学组织胚胎学教研室

本文报告了三例经肝活检铁染色证实之原发性血色病。进行了家系调查,其中二例为近亲结婚,一例为非近亲结婚,结合文献对病因和诊断方法进行了讨论。关于原发性与继发性血色病的鉴别诊断提出了一些看法。

关键词： hemochromatosis biopsy diagnosis, differential

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Iron homeostasis and H63D mutations in alcoholics with and without liver disease

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World Journal of Gastroenterology 2009年第1期15卷 106-111页

作者： Mariana Verdelho Machado Paula Ravasco Alexandra Martins Maria Rosário Almeida Maria Ermelinda Camilo Helena Cortez-Pinto Unidade de Nutrio e Metabolismo Instituto de Medicina Molecular Faculdade de Medicina da Universidade de Lisboa1649-035 Departamento de Gastrenterologia do Hospital de Santa Maria 1649-035 Lisboa Portugal Genomed Instituto de Medicina Molecular1649-035 Portugal

MM： To evaluate the prevalence of HFE gene mutation and indices of disturbed iron homeostasis in alcoholics with and without liver disease. METHODS： One hundred and fifty-three heavy drinkers （defined as alcohol consumption 〉 80 g/d for at least 5 years） were included in the study. These comprised 78 patients with liver disease [liver disease alcoholics （LDA）] in whom the presence of liver disease was confirmed by liver biopsy or clinical evidence of hepatic decompensation, and 75 subjects with no evidence of liver disease, determined by normal liver tests on two occasions [non-liver disease alcoholics （NLDA）], were consecutively enrolled. Serum markers of iron status and HFE C282Y and H63D mutations were determined. HFE genotyping was compared with data obtained in healthy blood donors from the same geographical area. RESULTS： Gender ratio was similar in both study groups. LDA patients were older than NLDA patients （52 ± 10 years vs 48 ± 11 years, P = 0.03）. One third and one fifth of the study population had serum transferrin saturation （TS） greater than 45% and 60% respectively. Serum iron levels were similar in both groups. However, LDA patients had higher TS （51 ± 27 vs 36 ± 13, P 〈 0.001） and ferritin levels （559 ± 607 ng/mL vs 159 ± 122 ng/mL, P 〈 0.001）, and lower total iron binding capacity （TIBC）（241 ± 88 μg/dL vs 279 ± 40 μg/dL, P = 0.001）. The odds ratio for having liver disease with TS greater than 45% was 2.20 （95% confidence interval （CI）： 1.37-3.54）. There was no difference in C282Y allelic frequency between the two groups. However, H63D was more frequent in LDA patients （0.25 vs 0.16, P = 0.03）. LDA patients had a greater probability of carrying at least one HFE mutation than NLDA patients （49.5% vs 31.6%, P = 0.02）. The odds ratio for LDA in patients with H63D mutation was 1.57 （95% CI： 1.02-2.40）. CONCLUSION： The present study confirms the presence of iron overload in alcoholics, which was more severe in the subset of subjects with liver disease,

关键词： Alcoholic liver disease Iron lIFE gene H63D hemochromatosis

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Pathology of hepatic iron overload

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World Journal of Gastroenterology 2007年第35期13卷 4755-4760页

作者： Yves Deugnier Bruno Turlin Liver Unit and CIC INSERM 0203 Pontchaillou University HospitalRennes 35033France Department of Pathology and INSERM U 522 Pontchaillou University HospitalRennes 35033France

Although progress in imaging and genetics allow for a noninvasive diagnosis of most cases of genetic iron overload, liver pathology remains often useful （1） to assess prognosis by grading fibrosis and seeking for associated lesions and （2） to guide the etiological diagnosis, especially when no molecular marker is available. Then, the type of liver siderosis （parenchymal, mesenchymal or mixed） and its distribution throughout the Iobule and the liver are useful means for suggesting its etiology： HLA-linked hemochromatosis gene （HFE） hemochromatosis or other rare genetic hemochromatosis, nonhemochromatotic genetic iron overload （ferroportin disease, aceruloplasminemia）, or iron overload secondary to excessive iron supply, inflammatory syndrome, noncirrhotic chronic liver diseases including dysmetabolic iron overload syndrome, cirrhosis, and blood disorders.

关键词： Iron Liver Biopsy hemochromatosis Ferroportin HLA-linked hemochromatosis gene Hepcidin Metabolic syndrome

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Liver iron content determination by magnetic resonance imaging

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World Journal of Gastroenterology 2010年第13期16卷 1587-1597页

作者： Konstantinos Tziomalos Vassilios Perifanis 1st Propedeutic Department of Internal Medicine Medical School Aristotle University of Thessaloniki AHEPA University Hospital Thalassemia Unit Hippokration Hospital

Accurate evaluation of iron overload is necessary to establish the diagnosis of hemochromatosis and guide chelation treatment in transfusion-dependent anemia. The liver is the primary site for iron storage in patients with hemochromatosis or transfusion-dependent anemia, therefore, liver iron concentration (LIC) accurately re? ects total body iron stores. In the past 20 years, magnetic resonance imaging (MRI) has emerged as a promising method for measuring LIC in a variety of diseases. We review the potential role of MRI in LIC determination in the most important disorders that are characterized by iron overload, that is, thalassemia major, other hemoglobinopathies, acquired anemia, and hemochromatosis. Most studies have been performed in thalassemia major and MRI is currently a widely accepted method for guiding chelation treatment in these patients. However, the lack of correlation between liver and cardiac iron stores suggests that both organs should be evaluated with MRI, since cardiac disease is the leading cause of death in this population. It is also unclear which MRI method is the most accurate since there are no large studies that have directly compared the different available techniques. The role of MRI in the era of genetic diagnosis of hemochromatosis is also debated, whereas data on the accuracy of the method in other hematological and liver diseases are rather limited. However, MRI is a fast, non-invasive and relatively accurate diagnostic tool for assessing LIC, and its use is expected to increase as the role of iron in the pathogenesis of liver disease becomes clearer.

关键词： Thalassemia major Iron overload Magnetic resonance imaging Liver hemochromatosis Desferrioxamine Deferiprone Deferasirox Thalassemia inter media Myelodysplastic syndromes

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Hepcidin modulation in human diseases: From research to clinic

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World Journal of Gastroenterology 2009年第5期15卷 538-551页

作者： Alberto Piperno Raffaella Mariani Paola Trombini Domenico Girelli Department of Clinical Medicine and Prevention University of Milano-Bicocca Monza 20052 Italy Centre for Diagnosis and Treatment of hemochromatosis and Iron Overload Diseases San Gerardo Hospital Monza 20052 Italy Department of Clinical and Experimental Medicine University of Verona Verona 37100 Italy

By modulating hepcidin production, an organism controls intestinal iron absorption, iron uptake and mobilization from stores to meet body iron need. In recent years there has been important advancement in our knowledge of hepcidin regulation that also has implications for understanding the physiopathology of some human disorders. Since the discovery of hepcidin and the demonstration of its pivotal role in iron homeostasis, there has been a substantial interest in developing a reliable assay of the hormone in biological fluids. Measurement of hepcidin in biological fluids can improve our understanding of iron diseases and be a useful tool for diagnosis and clinical management of these disorders. We reviewed the literature and our own research on hepcidin to give an updated status of the situation in this rapidly evolving field.

关键词： Hepcidin Iron homeostasis Erythropoiesis hemochromatosis Anemia Liver diseases

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Liver cirrhosis as a consequence of iron overload caused by hereditary nonspherocytic hemolytic anemia

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World Journal of Gastroenterology 2005年第8期11卷 1241-1244页

作者： Philip Hilgard Guido Gerken Department of Gastroenterology and Hepatology

Nonspherocytic hereditary anemias are occasionally accompanied by significant iron overload but the significance for the development of chronic liver disease is not clear. We described two cases of patients with chronic liver d isease and severeiron overload due to chronic hereditary hemolysis. Both patients have had signs of liver cirrhosis and severe hemolysis since childhood. A hereditary pyruvate kinase deficiency (PKD) was discovered as the underlying reason for the *** of the pyruvate kinase gene showed a mutation within exon 11. Liver histology in both patients revealed cirrhosis and a severe iron overload but primary hemochromatosis was excluded by HFE-gene *** iron reduction therapy with desferrioxamine led to significant decrease of serum ferritin and sustained clinical improvement. PKD-induced hemolysis may cause severe iron overload even in the absence of HFE-genotype abnormalities. This secondary iron overload can lead to chronic liver disease and cirrhosis. Therefore, the iron metabolism of PKD patients has to be closely monitored and iron overload should be consequently treated.

关键词： hemochromatosis Pyruvate kinase deficiency Liver cirrhosis Iron overload Desferrioxamine

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Porphyria cutanea tarda as a complication of therapy for chronic hepatitis C

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World Journal of Gastroenterology 2008年第38期14卷 5913-5915页

作者： James Azim Heather McCurdy Richard H Moseley Division of Gastroenterology Department of Internal Medicine Department of Veterans Affairs Medical Center and University of Michigan School of Medicine Ann ArborMichigan United States

There is a strong association between porphyria cutanea tarda (PCT) and chronic viral hepatitis C. Therapy for chronic viral hepatitis C may improve PCT. However, there are only a few reports of the de novo development of PCT during therapy for chronic viral hepatitis C. We describe the development of PCT in a 56-year-old patient with chronic viral hepatitis C after 12 wk of peginterferon/ribavirin therapy. In addition, the patient was homozygous for the H63D hereditary hemochromatosis gene (HFE ) mutation. The association of PCT with chronic viral hepatitis C and the possible role of hepatic iron overload and ribavirin-induced hemolytic anemia in the development of PCT during therapy for chronic viral hepatitis C are discussed.

关键词： Porphyria Hepatitis C virus Ribavirin Pegylated interferon hemochromatosis

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Ceruloplasmin-ferroportin system of iron traffic in vertebrates

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World Journal of Biological Chemistry 2014年第2期5卷 204-215页

作者： Giovanni Musci Fabio Polticelli Maria Carmela Bonaccorsi di Patti Department of Biosciences and Territory University of Molise Pesche 86090 Italy Department of Sciences University Roma Tre00146 Rome Italy National Institute of Nuclear Physics Roma Tre Section 00146 Rome Italy Department of Biochemical Sciences Sapienza University of Rome 00185 Rome Italy

Safe trafficking of iron across the cell membrane is a delicate process that requires specific protein carriers. While many proteins involved in iron uptake by cells are known, only one cellular iron export protein has been identified in mammals: ferroportin(SLC40A1). Ceruloplasmin is a multicopper enzyme endowed with ferroxidase activity that is found as a soluble isoform in plasma or as a membrane-associated isoform in specific cell types. According to the currently accepted view, ferrous iron transported out of the cell by ferroportin would be safely oxidized by ceruloplasmin to facilitate loading on transferrin. Therefore, the ceruloplasminferroportin system represents the main pathway for cellular iron egress and it is responsible for physiological regulation of cellular iron levels. The most recent findings regarding the structural and functional features of ceruloplasmin and ferroportin and their relationship will be described in this review.

关键词： Iron homeostasis Ferroportin Ceruloplasmin hemochromatosis Copper

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