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Iron overload

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https://www.readbyqxmd.com/read/28935635/serpinb3-is-overexpressed-in-the-liver-in-presence-of-iron-overload
#1
Cristian Turato, Patricia Kent, Giada Sebastiani, Stefania Cannito, Elisabetta Morello, Liliana Terrin, Alessandra Biasiolo, Davide Simonato, Maurizio Parola, Kostas Pantopoulos, Patrizia Pontisso
Iron overload results in cellular toxicity, tissue injury, organ fibrosis and increased risk of neoplastic transformation. SerpinB3 is a serine protease inhibitor overexpressed in the liver in oxidative stress conditions, able to induce fibrosis and increased risk of malignant transformation. Aim of the present study was to assess the effect of iron overload on SerpinB3 expression in the liver using in vivo and in vitro models.The expression of Serpinb3 was assessed in the liver of hemojuvelin knockout mice (Hjv-/-), an established model of hereditary hemochromatosis, and of wild type control mice, following dietary or pharmacological iron manipulation...
September 20, 2017: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
https://www.readbyqxmd.com/read/28933303/cardioprotective-potential-of-iron-chelators-and-prochelators
#2
Hana Jansova, Tomas Simunek
Heart is a particularly sensitive organ to iron overload and cardiomyopathy due to the excessive cardiac iron deposition causes most deaths in disorders such as beta-thalassemia major. Free or loosely bound iron ions readily cycle between ferrous and ferric states and catalyze Haber-Weiss reaction that yields highly reactive and toxic hydroxyl radicals. Treatment with iron chelators (desferrioxamine, deferiprone, and deferasirox) substantially improved cardiovascular morbidity and mortality in iron overloaded patients...
September 20, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28932366/comparison-of-iron-chelation-effects-of-deferoxamine-deferasirox-and-combination-of-deferoxamine-and-deferiprone-on-liver-and-cardiac-t2-mri-in-thalassemia-maior
#3
Shahla Ansari, Azita Azarkeivan, Ghasem Miri-Aliabad, Saeed Yousefian, Tahereh Rostami
BACKGROUND: Cardiac complications due to iron overload are the most common cause of death in patients with thalassemia major. The aim of this study was to compare iron chelation effects of deferoxamine, deferasirox, and combination of deferoxamine and deferiprone on cardiac and liver iron load measured by T2* MRI. METHODS: In this study, 108 patients with thalassemia major aged over 10 years who had iron overload in cardiac T2* MRI were studied in terms of iron chelators efficacy on the reduction of myocardial siderosis...
2017: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/28931050/saxs-and-stability-studies-of-iron-induced-oligomers-of-bacterial-frataxin-cyay
#4
Mostafa Fekry, Wessen Alshokry, Przemysław Grela, Marek Tchórzewski, Eva-Christina Ahlgren, Christopher A Söderberg, Oleksandr Gakh, Grazia Isaya, Salam Al-Karadaghi
Frataxin is a highly conserved protein found in both prokaryotes and eukaryotes. It is involved in several central functions in cells, which include iron delivery to biochemical processes, such as heme synthesis, assembly of iron-sulfur clusters (ISC), storage of surplus iron in conditions of iron overload, and repair of ISC in aconitase. Frataxin from different organisms has been shown to undergo iron-dependent oligomerization. At least two different classes of oligomers, with different modes of oligomer packing and stabilization, have been identified...
2017: PloS One
https://www.readbyqxmd.com/read/28930842/type-4b-hereditary-hemochromatosis-associated-with-a-novel-mutation-in-the-slc40a1-gene-a-case-report-and-a-review-of-the-literature
#5
Wei Zhang, Tingxia Lv, Jian Huang, Xiaojuan Ou
RATIONALE: Hereditary hemochromatosis can be divided into HFE- and non-HFE-related based on genetic mutations in different genes. HFE-related hemochromatosis is the most common inherited genetic disease in European populations but rare in Asia-pacific region. Recently, non-HFE-related hemochromatosis has been reported in patients from the Asian countries. PATIENT CONCERNS: We report the case of a 48-year-old Chinese Han woman who presented with abnormal liver function, diabetes mellitus, hyperferritinemia, and high transferrin saturation, with severe iron overload in parenchymal cells, Kupffer cells, and periportal fibrosis on liver biopsy...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28930590/chelation-protocols-for-the-elimination-and-prevention-of-iron-overload-in-thalassaemia
#6
Annita Kolnagou, George John Kontoghiorghes
Iron overload toxicity is the main cause of mortality and morbidity in thalassaemia patients. The complete elimination and prevention of iron overload is the main aim of chelation therapy, which can be achieved by chelation protocols that can effectively remove excess iron load and maintain body iron at normal levels. Deferiprone and selected combinations with deferoxamine can be designed, adjusted and used effectively for removing all excess stored iron and for maintaining normal iron stores (NIS) in different categories of thalassaemia patients...
January 1, 2018: Frontiers in Bioscience (Landmark Edition)
https://www.readbyqxmd.com/read/28930516/new-targeted-therapies-and-diagnostic-methods-for-iron-overload-diseases
#7
Annita Kolnagou, Christina N Kontoghiorghe, George John Kontoghiorghes
Millions of people worldwide suffer from iron overload toxicity diseases such as transfusional iron overload in thalassaemia and hereditary haemochromatosis. The accumulation and presence of toxic focal iron deposits causing tissue damage can also be identified in Friedreich's ataxia, Alzheimer's, Parkinson's, renal and other diseases. Different diagnostic criteria of toxicity and therapeutic interventions apply to each disease of excess or misplaced iron. Magnetic resonance imaging relaxation times T2 and T2* for monitoring iron deposits in organs and iron biomarkers such as serum ferritin and transferrin iron saturation have contributed in the elucidation of iron toxicity mechanisms and pathways, and also the evaluation of the efficacy and mode of action of chelating drugs in the treatment of diseases related to iron overload, toxicity and metabolism...
January 1, 2018: Frontiers in Bioscience (Scholar Edition)
https://www.readbyqxmd.com/read/28929515/treatment-of-hepatitis-c-virus-infection-with-direct-acting-antiviral-drugs-is-safe-and-effective-in-patients-with-hemoglobinopathies
#8
Raffaella Origa, Maria Laura Ponti, Aldo Filosa, Alfonso Galeota Lanza, Antonio Piga, Giorgio Maria Saracco, Valeria Pinto, Antonino Picciotto, Paolo Rigano, Salvatore Madonia, Rosamaria Rosso, Domenico D'Ascola, Maria Domenica Cappellini, Roberta D'Ambrosio, Immacolata Tartaglione, Lucia De Franceschi, Barbara Gianesin, Vito Di Marco, Gian Luca Forni
Progression of liver fibrosis in patients with hemoglobinopathies is strongly related to the severity of iron overload and the presence of chronic hepatitis C virus (HCV) infection. Effective iron chelation therapy and HCV infection eradication may prevent liver complications. The European Association for the Study of the Liver guidelines recommend interferon-free regimens for the treatment of HCV infection in patients with hemoglobinopathies. However, data regarding the use of direct-acting antiviral drugs (DAAs) in this patient population are few...
September 20, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28927633/the-use-of-mri-to-monitor-iron-overload-in-scd
#9
John C Wood
No abstract text is available yet for this article.
September 6, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28924413/pseudovitelliform-maculopathy-associated-with-deferoxamine-toxicity-multimodal-imaging-and-electrophysiology-of-a-rare-entity
#10
Kelly M Bui, SriniVas R Sadda, Hani Salehi-Had
Deferoxamine is a commonly used chelating agent for secondary hemochromatosis. We report a rare retinal manifestation of deferoxamine toxicity in a 68-year-old man and provide supporting multimodal imaging and electrophysiology. The patient had iron overload related to transfusion-dependent myelodysplastic syndrome and developed a pseudovitelliform macular lesion related to deferoxamine toxicity. We also describe for the first time the worsening of this maculopathy on deferasirox, an alternative chelating agent...
2017: Digital Journal of Ophthalmology: DJO
https://www.readbyqxmd.com/read/28923493/diagnosis-and-quantification-of-the-iron-overload-through-magnetic-resonance
#11
J M Alústiza Echeverría, M C Barrera Portillo, A Guisasola Iñiguiz, A Ugarte Muño
There are different magnetic resonance techniques and models to quantify liver iron concentration. T2 relaxometry methods evaluate the iron concentration in the myocardium, and they are able to discriminate all the levels of iron overload in the liver. Signal intensity ratio methods saturate with high levels of liver overload and can not assess iron concentration in the myocardium but they are more accessible and are very standardized. This article reviews, in different clinical scenarios, when Magnetic Resonance must be used to assess iron overload in the liver and myocardium and analyzes the current challenges to optimize the aplication of the technique and to be it included in the clinical guidelines...
September 15, 2017: Radiología
https://www.readbyqxmd.com/read/28921500/growth-hormone-therapy-for-people-with-thalassaemia
#12
REVIEW
Chin Fang Ngim, Nai Ming Lai, Janet Yh Hong, Shir Ley Tan, Amutha Ramadas, Premala Muthukumarasamy, Meow-Keong Thong
BACKGROUND: Thalassaemia is a recessively-inherited blood disorder that leads to anaemia of varying severity. In those affected by the more severe forms, regular blood transfusions are required which may lead to iron overload. Accumulated iron from blood transfusions may be deposited in vital organs including the heart, liver and endocrine organs such as the pituitary glands which can affect growth hormone production. Growth hormone deficiency is one of the factors that can lead to short stature, a common complication in people with thalassaemia...
September 18, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28920821/serum-ferritin-concentration-is-not-a-reliable-biomarker-of-iron-overload-disorder-progression-or-hemochromatosis-in-the-sumatran-rhinoceros-dicerorhinus-sumatrensis
#13
Terri L Roth, Paul R Reinhart, Jennifer L Kroll
The aim of this study was to determine if ferritin is a reliable biomarker of iron overload disorder (IOD) progression and hemochromatosis in the Sumatran rhinoceros (Dicerorhinus sumatrensis) by developing a species-specific ferritin assay and testing historically banked samples collected from rhinos that did and did not die of hemochromatosis. Ferritin extracted from Sumatran rhino liver tissue was used to generate antibodies for the Enzyme Immunoassay. Historically banked Sumatran rhino serum samples (n = 298) obtained from six rhinos in US zoos (n = 290); five rhinos at the Sumatran Rhino Conservation Centre in Sungai Dusun, Malaysia (n = 5); and two rhinos in Sabah, Malaysia (n = 3) were analyzed for ferritin concentrations...
September 2017: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
https://www.readbyqxmd.com/read/28918510/acquired-hepatocerebral-degeneration-ahd-a-peculiar-neurological-impairment-in-advanced-chronic-liver-disease
#14
A Pigoni, F Iuculano, C Saetti, L Airaghi, L Burdick, S Spreafico, M Curioni, R Lombardi, L Valenti, A L Fracanzani, S Fargion
We discuss the case of a rare and often unrecognized neurologic syndrome, called Acquired Hepatocerebral Degeneration (AHD), observed in patients with advanced liver disease and portosystemic shunts. The clinical manifestations can be very heterogeneous and in our case included a combination of cerebellar and extrapyramidal signs, arisen in a period of few days. Brain Magnetic Resonance Imaging (MRI) showed, in T1-weighted images, diffuse bilateral hyper intensities in basal ganglia and biemispheric brain and cerebellar cortices, resembling paramagnetic deposits...
September 16, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28917236/dietary-intake-of-heme-iron-and-body-iron-status-are-associated-with-the-risk-of-gestational-diabetes-mellitus-a-systematic-review-and-meta-analysis
#15
Lu Zhao, Jia Lian, Jishun Tian, Ye Shen, Zhiguang Ping, Xuexian Fang, Junxia Min, Fudi Wang
BACKGROUND AND OBJECTIVES: Some potential role of iron overload in the development of diabetes mellitus have been suggested. Our study aimed to systematically assess the association between the risk of gestational diabetes mellitus (GDM) and iron intakes/body iron status. METHODS AND STUDY DESIGN: PubMed and Web of Science were searched for relevant articles. Relative risks (RR) of GDM in relation to dietary iron intakes and body iron stores were pooled with the random-effects model...
2017: Asia Pacific Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28915963/hepcidin-and-metallothioneins-as-molecular-base-for-sex-dependent-differences-in-clinical-course-of-experimental-autoimmune-encephalomyelitis-in-chronic-iron-overload
#16
Božena Ćurko-Cofek, Tanja Grubić Kezele, Vesna Barac-Latas
Multiple sclerosis is a chronic demyelinating disease of the central nervous system characterised by inflammatory and degenerative changes. It is considered that disease arises from the influence of environmental factors on genetically susceptible individuals. Recent researches, using magnetic resonance imaging, connected iron deposits in different brain regions with demyelinating process in multiple sclerosis patients. Although iron is an essential trace element important for many biological functions it could be harmful because iron excess can induce the production of reactive oxygen species, development of oxidative stress and lipid peroxidation which leads to demyelination...
September 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28914813/lactoferrin-a-natural-glycoprotein-involved-in-iron-and-inflammatory-homeostasis
#17
REVIEW
Luigi Rosa, Antimo Cutone, Maria Stefania Lepanto, Rosalba Paesano, Piera Valenti
Human lactoferrin (hLf), an iron-binding multifunctional cationic glycoprotein secreted by exocrine glands and by neutrophils, is a key element of host defenses. HLf and bovine Lf (bLf), possessing high sequence homology and identical functions, inhibit bacterial growth and biofilm dependently from iron binding ability while, independently, bacterial adhesion to and the entry into cells. In infected/inflamed host cells, bLf exerts an anti-inflammatory activity against interleukin-6 (IL-6), thus up-regulating ferroportin (Fpn) and transferrin receptor 1 (TfR1) and down-regulating ferritin (Ftn), pivotal actors of iron and inflammatory homeostasis (IIH)...
September 15, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28902419/reassessing-the-safety-concerns-of-utilizing-blood-donations-from-patients-with-hemochromatosis
#18
REVIEW
Adam C Winters, Douglas Tremblay, Suzanne Arinsburg, John Mascarenhas, Thomas D Schiano
Hereditary hemochromatosis (HH) is a genetic disorder of iron metabolism which may lead to iron overload. Clinical penetrance is low, however those afflicted may develop cirrhosis, hepatocellular carcinoma, diabetes mellitus and cardiomyopathy. Treatment involves regular phlebotomy to reduce the systemic iron burden. In many countries-including the United States-numerous blood centers do not accept donated blood obtained from HH patients during therapeutic phlebotomy and there are inconsistent positions regarding this globally...
September 13, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28889118/biotechnology-of-siderophores-in-high-impact-scientific-fields
#19
REVIEW
Luis O De Serrano
Different aspects of bacterial and fungal siderophore biotechnological applications will be discussed. Areas of application presented include, but are not limited to agriculture, medicine, pharmacology, bioremediation, biodegradation and food industry. In agriculture-related applications, siderophores could be employed to enhance plant growth due to their uptake by rhizobia. Siderophores hindered the presence of plant pathogens in biocontrol strategies. Bioremediation studies on siderophores discuss mostly the mobilization of heavy metals and radionuclides; the emulsifying effects of siderophore-producing microorganisms in oil-contaminated environments are also presented...
September 26, 2017: Biomolecular Concepts
https://www.readbyqxmd.com/read/28883221/welder-s-pulmonary-hemosiderosis-associated-with-systemic-iron-overload-following-exacerbation-of-acute-adult-t-cell-leukemia-lymphoma
#20
Naoto Imoto, Akira Shiraki, Katsuya Furukawa, Naoyuki Tange, Atsushi Murase, Masaya Hayakawa, Yosuke Iwata, Hiroshi Kosugi
Herein, we describe a 61-year-old man diagnosed with pulmonary hemosiderosis following chemotherapy for acute adult T-cell leukemia/lymphoma (ATLL). Liver and heart biopsy confirmed hemosiderosis. ATLL progressed, and the patient died from multiorgan damage. Welder's lung may have been involved in hemosiderosis and systemic iron overload. Abnormal iron metabolism or immune reactions may have influenced the clinical course, but these were not validated. Detailed analyses of family medical and lifestyle histories, and genetic examination should be performed in cases of systemic iron overload...
September 6, 2017: Journal of Clinical and Experimental Hematopathology: JCEH
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