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Iron overload syndrome

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https://www.readbyqxmd.com/read/30084733/dietary-green-tea-extract-prior-to-spinal-cord-injury-prevents-hepatic-iron-overload-but-does-not-improve-chronic-hepatic-and-spinal-cord-pathology-in-rats
#1
Matthew Goodus, Andrew David Sauerbeck, Phillip G Popovich, Richard S Bruno, Dana M McTigue
Spinal cord injury (SCI) disrupts autonomic regulation of visceral organs. As a result, a leading cause of mortality in the SCI population is metabolic dysfunction, and an organ central to metabolic control is the liver. Our recent work showed that rodent SCI promotes Kupffer cell (hepatic macrophage) activation, pro-inflammatory cytokine expression and liver steatosis. These are symptoms of nonalcoholic steatohepatitis (NASH), the hepatic manifestation of metabolic syndrome, and these pre-clinical data replicate aspects of post-SCI human metabolic dysfunction...
August 7, 2018: Journal of Neurotrauma
https://www.readbyqxmd.com/read/30067867/red-blood-cell-transfusion-to-treat-or-prevent-complications-in-sickle-cell-disease-an-overview-of-cochrane-reviews
#2
REVIEW
Patricia M Fortin, Sally Hopewell, Lise J Estcourt
BACKGROUND: Globally, sickle cell disease (SCD) is one of the commonest severe monogenic disorders, due to the inheritance of two abnormal haemoglobin (beta globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Red blood cell (RBC) transfusions are used to treat complications of SCD, e.g. acute chest syndrome (ACS) (this often involves a single transfusion episode), or they can be part of a regular long-term transfusion programme to prevent SCD complications...
August 1, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29905937/iron-overload-in-patients-with-myelodysplastic-syndromes-an-updated-overview
#3
REVIEW
Nour M Moukalled, Fuad A El Rassi, Sally N Temraz, Ali T Taher
Myelodysplastic syndromes (MDS) encompass a heterogeneous group of clonal hematopoietic stem cell disorders characterized by a broad clinical spectrum related to ineffective hematopoiesis leading to unilineage or multilineage cytopenias, with a high propensity for transformation to acute myeloid leukemia. Iron overload has been recently identified as one of the important conditions complicating the management of these diverse disorders. The accumulation of iron is mainly related to chronic transfusions; however, evidence suggests a possible role for ineffective erythropoiesis and increased intestinal absorption of iron, related to altered hepcidin and growth differentiation factor-15 levels in the development of hemosiderosis in patients with MDS...
June 15, 2018: Cancer
https://www.readbyqxmd.com/read/29904950/optimizing-management-of-deferasirox-therapy-for-patients-with-transfusion-dependent-thalassaemia-and-lower-risk-myelodysplastic-syndromes
#4
REVIEW
Antonis Kattamis, Yesim Aydinok, Ali Taher
Effective iron chelation therapy is an important part of treatment in patients with transfusion-dependent thalassaemia and lower-risk myelodysplastic syndromes (MDS). Key strategies for optimizing iron chelation therapy include ensuring good adherence and preventing and managing adverse events (AEs). Good adherence to iron chelation therapy with deferoxamine and deferasirox has been linked to improved survival and/or reductions in complications related to iron overload; however, maintaining good adherence to iron chelators can be challenging...
June 14, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29903757/iron-overload-impairs-normal-hematopoietic-stem-and-progenitor-cells-through-reactive-oxygen-species-and-shortens-survival-in-myelodysplastic-syndrome-mice
#5
Xin Jin, Xiaoyuan He, Xiaoli Cao, Ping Xu, Yi Xing, Songnan Sui, Luqiao Wang, Juanxia Meng, Wenyi Lu, Rui Cui, Hongyan Ni, Mingfeng Zhao
Accumulating clinical evidence implicates a suppressive effect on hematopoiesis in myelodysplastic syndrome patients with iron overload. However, how iron overload influences hematopoiesis in myelodysplastic syndrome remains unknown. Here, the RUNX1S291fs-transduced bone marrow mononuclear cells were yielded and transplanted into lethally irradiated recipient mice together with radioprotective bone marrow cells to generate myelodysplastic syndrome mice. Eight weeks post transplantation, the recipient mice were administered intraperitoneal injection of 0...
June 14, 2018: Haematologica
https://www.readbyqxmd.com/read/29902097/clinical-consequences-of-iron-overload-in-patients-with-myelodysplastic-syndromes-the-case-for-iron-chelation-therapy
#6
Jamile M Shammo, Rami S Komrokji
Patients with myelodysplastic syndromes (MDS) are at increased risk of iron overload due to ineffective erythropoiesis and chronic transfusion therapy. The clinical consequences of iron overload include cardiac and/or hepatic failure, endocrinopathies, and infection risk. Areas covered: Iron chelation therapy (ICT) can help remove excess iron and ultimately reduce the clinical consequences of iron overload. The authors reviewed recent (last five years) English-language articles from PubMed on the topic of iron overload-related complications and the use of ICT (primarily deferasirox) to improve outcomes in patients with MDS...
July 2018: Expert Review of Hematology
https://www.readbyqxmd.com/read/29883686/prognostic-impact-of-elevated-pretreatment-serum-ferritin-in-patients-with-high-risk-mds-treated-with-azacitidine
#7
Kamel Laribi, Delphine Bolle, Mustapha Alani, Habib Ghnaya, Saga Le Bourdelles, Anne Besançon, Jonathan Farhi, Nathalie Denizon, Alix Baugier de Materre
Iron overload has been associated with poor overall survival in patients with higher-risk myelodysplastic syndromes after allogeneic hematopoietic stem cell transplantation, but has not been investigated in higher-risk MDS patients treated with hypomethylating agents. We evaluated the prognostic value of serum ferritin levels at diagnosis in a retrospective analysis of 48 patients with an intermediate 2 or high-risk International Prognostic Scoring System (IPSS) score treated with azacytidine. overall survival probability at 1 and 2years was 58% and 42%, respectively...
June 5, 2018: Experimental Hematology
https://www.readbyqxmd.com/read/29882374/the-interactions-between-iron-and-copper-in-genetic-iron-overload-syndromes-and-primary-copper-toxicoses-in-japan
#8
REVIEW
Yasuaki Tatsumi, Ayako Kato, Koichi Kato, Hisao Hayashi
Iron and copper are trace elements essential for health, and iron metabolism is tightly regulated by cuproproteins. Clarification of the interactions between iron and copper may provide a better understanding of the pathophysiology and treatment strategy for hemochromatosis, Wilson disease, and related disorders. The hepcidin/ferroportin system was used to classify genetic iron overload syndromes in Japan, and ceruloplasmin and ATP7B were introduced for subtyping Wilson disease into the severe hepatic and classical forms...
June 7, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29871592/hepcidin-levels-correlate-to-liver-iron-content-but-not-steatohepatitis-in-non-alcoholic-fatty-liver-disease
#9
Joel Marmur, Soheir Beshara, Gösta Eggertsen, Liselotte Onelöv, Nils Albiin, Olof Danielsson, Rolf Hultcrantz, Per Stål
BACKGROUND: One-third of patients with non-alcoholic fatty liver disease (NAFLD) develop dysmetabolic iron overload syndrome (DIOS), the pathogenesis of which is unknown. Altered production of the iron-regulatory peptide hepcidin has been reported in NAFLD, but it is unclear if this is related to iron accumulation, lipid status or steatohepatitis. METHODS: Eighty-four patients with liver disease, 54 of which had iron overload, underwent liver biopsy (n = 66) and/or magnetic resonance imaging (n = 35) for liver iron content determination...
June 5, 2018: BMC Gastroenterology
https://www.readbyqxmd.com/read/29851359/insulin-resistance-and-metabolic-syndrome-clinical-and-laboratory-associations-in-african-americans-without-diabetes-in-the-hemochromatosis-and-iron-overload-screening-study
#10
James C Barton, Jackson Clayborn Barton, Ronald T Acton
BACKGROUND: We sought to determine associations with insulin resistance (IR) and metabolic syndrome (MetS) in African Americans. METHODS: We studied African American adults without diabetes in a postscreening examination. Participants included Cases: transferrin saturation (TS) >50% and serum ferritin (SF) >300 μg/L (M), and TS >45% and SF >200 μg/L (F), regardless of HFE genotype; and Controls: TS/SF 25th to 75th percentiles and HFE wt/wt (wild type)...
May 31, 2018: Metabolic Syndrome and related Disorders
https://www.readbyqxmd.com/read/29765443/management-of-cardiac-hemochromatosis
#11
Wilbert S Aronow
Iron-overload syndromes may be hereditary or acquired. Patients may be asymptomatic early in the disease. Once heart failure develops, there is rapid deterioration. Cardiac hemochromatosis is characterized by a dilated cardiomyopathy with dilated ventricles, reduced ejection fraction, and reduced fractional shortening. Deposition of iron may occur in the entire cardiac conduction system, especially the atrioventricular node. Cardiac hemochromatosis should be considered in any patient with unexplained heart failure...
April 2018: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/29761371/tolerability-and-efficacy-of-deferasirox-in-patients-with-transfusional-iron-overload-results-from-a-german-2-year-non-interventional-study
#12
Florian Nolte, Holger Nückel, Burkhard Schmidt, Thomas Geer, Oleg Rubanov, Holger Hebart, Andrea Jarisch, Stefan Albrecht, Christiane Johr, Christiane Schumann, Wolf-Karsten Hofmann
BACKGROUND: Iron overload (IOL) due to repetitive transfusions of packed red blood cells (pRBC) has a major impact on morbidity and mortality in patients with inherited bone marrow failure syndromes and hemoglobinopathies such as thalassemia and sickle cell disease. However, whether IOL influences the outcome of elderly patients with myeloid malignancies is not yet clear. Moreover, clinical trials have reported high drop-out rates during treatment with the oral iron chelator deferasirox (DFX)...
May 14, 2018: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/29725013/iron-overload-promotes-mitochondrial-fragmentation-in-mesenchymal-stromal-cells-from-myelodysplastic-syndrome-patients-through-activation-of-the-ampk-mff-drp1-pathway
#13
Qingqing Zheng, Youshan Zhao, Juan Guo, Sida Zhao, Chengming Fei, Chao Xiao, Dong Wu, Lingyun Wu, Xiao Li, Chunkang Chang
Iron overload (IO) has been reported to contribute to mesenchymal stromal cell (MSC) damage, but the precise mechanism has yet to be clearly elucidated. In this study, we found that IO increased cell apoptosis and lowered cell viability in MSCs, accompanied by extensive mitochondrial fragmentation and autophagy enhancement. All these effects were reactive oxygen species (ROS) dependent. In MSCs with IO, the ATP concentrations were significantly reduced due to high ROS levels and low electron respiratory chain complex (ETC) II/III activity...
May 1, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29719933/prospective-evaluation-of-the-effect-of-deferasirox-on-hematologic-response-in-transfusion-dependent-patients-with-low-risk-mds-and-iron-overload
#14
Christian Rose, Caroline Lenoir, Emmanuel Gyan, Maya Hacini, Shanti Amé, Bernadette Corront, Odile Beyne-Rauzy, Didier Adiko, Elena Loppinet, Nadia Ali-Ammar, Kamel Laribi, Eric Wattel, François Dreyfus, Claire S Roué, Stephane Cheze
OBJECTIVES: To assess the reduction of transfusions rate in transfusion-dependent patients with low-risk myelodysplastic syndrome (MDS) with iron overload treated with deferasirox. METHODS: Prospective observational study. Primary endpoint was reduction in transfusion requirements (RTR) at 3 months, (assessed on 8-week period). Secondary endpoints were hematologic improvement according to International Working Group (IWG) 2006 criteria at 3, 6, and 12 months. RESULTS: Fifty-seven patients were evaluable...
May 2, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29663858/assessment-of-liver-and-cardiac-iron-overload-using-mri-in-patients-with-chronic-anemias-in-latin-american-countries-results-from-asimila-study
#15
Rodolfo Cancado, Nora P Watman, Clarisse Lobo, Zulay Chona, Fernando Manzur, Fabiola Traina, Miriam Park, Guillermo Drelichman, Juan Pablo Zarate, Luis Marfil
OBJECTIVES: A multicenter, noninterventional, observational study was conducted in the Latin American countries including Argentina, Brazil, Colombia, Mexico, and Venezuela to assess the prevalence of liver and cardiac iron overload using magnetic resonance imaging (MRI) in patients with chronic anemias except thalassemia. METHODS: Patients aged >10 years with transfusion-dependent anemias, except thalassemia, either with <20 units of red blood cell (RBC) transfusions with serum ferritin (SF) levels >2000 ng/mL or with ≥20 units of RBC transfusions regardless of SF level in their lifetime, were enrolled...
April 17, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29659464/transitioning-patients-with-iron-overload-from-exjade-to-jadenu
#16
Sara M Tinsley, Christine M Hoehner-Cooper
Iron overload is a concern for patients who require chronic transfusions as a result of inherited or acquired anemias, including sickle cell disease, thalassemia, and myelodysplastic syndromes. Iron chelation therapy (ICT) is the primary treatment for iron overload in these patients. The ICT deferasirox, which has been available as an oral dispersible tablet for liquid suspension, is now also available as a once-daily, film-coated tablet (FCT). Deferasirox FCT allows greater convenience and may be associated with fewer gastrointestinal side effects versus the original formulation...
May 2018: Journal of Infusion Nursing: the Official Publication of the Infusion Nurses Society
https://www.readbyqxmd.com/read/29628397/enhanced-labile-plasma-iron-and-outcome-in-acute-myeloid-leukaemia-and-myelodysplastic-syndrome-after-allogeneic-haemopoietic-cell-transplantation-allive-a-prospective-multicentre-observational-trial
#17
Martin Wermke, Julia Eckoldt, Katharina S Götze, Stefan A Klein, Gesine Bug, Liesbeth C de Wreede, Michael Kramer, Friedrich Stölzel, Malte von Bonin, Johannes Schetelig, Michael Laniado, Verena Plodeck, Wolf-Karsten Hofmann, Gerhard Ehninger, Martin Bornhäuser, Dominik Wolf, Igor Theurl, Uwe Platzbecker
BACKGROUND: The effect of systemic iron overload on outcomes after allogeneic haemopoietic cell transplantation (HCT) has been a matter of substantial debate. We aimed to investigate the predictive value of both stored (MRI-derived liver iron content) and biologically active iron (enhanced labile plasma iron; eLPI) on post-transplantation outcomes in patients with acute myeloid leukaemia or myelodysplastic syndrome undergoing allogenic HCT. METHODS: The prospective, multicentre, observational, ALLogeneic Iron inVEstigators (ALLIVE) trial recruited patients at five centres in Germany...
May 2018: Lancet Haematology
https://www.readbyqxmd.com/read/29602612/to-chelate-or-not-to-chelate-in-mds-that-is-the-question
#18
REVIEW
Amer M Zeidan, Elizabeth A Griffiths
Myelodysplastic syndromes (MDS) are a heterogeneous group of hemopathies that exhibit physical manifestations with clinical consequences of bone marrow failure and inherent risk of progression to acute myeloid leukemia. Iron overload (IO) is common in MDS due to chronic transfusion support and disease-related alterations in iron metabolism. IO has been conclusively associated with inferior outcomes among MDS patients. Despite lack of randomized trials showing a survival impact of iron chelation therapy (ICT), ICT is recommended by experts and guidelines for select MDS patients with IO and is often used...
March 8, 2018: Blood Reviews
https://www.readbyqxmd.com/read/29511787/hypoparanet-a-database-of-chronic-hypoparathyroidism-based-on-expert-medical-surgical-centers-in-italy
#19
Gemma Marcucci, Luisella Cianferotti, Simone Parri, Paola Altieri, Emanuela Arvat, Salvatore Benvenga, Corrado Betterle, Marta Bondanelli, Marco Boscaro, Valentina Camozzi, Grazia Maria Centaro, Filomena Cetani, Iacopo Chiodini, Anna Ciampolillo, Annamaria Colao, Sabrina Corbetta, Maria Laura De Feo, Ettore Degli Uberti, Antongiulio Faggiano, Rachele Fornari, Achille Lucio Gaspari, Francesco Giorgino, Valeria Giuliani, Maurizio Iacobone, Nadia Innaro, Olga Lamacchia, Andrea Lenzi, Giovanna Mantovani, Claudio Marcocci, Laura Masi, Silvia Migliaccio, Serena Palmieri, Renato Pasquali, Giuliano Perigli, Valentina Piccini, Elisabetta Romagnoli, Rosaria Maddalena Ruggeri, Francesco Rulli, Maria Teresa Samà, Giuseppe Tomaino, Francesco Trimarchi, Maria Chiara Zatelli, Maria Luisa Brandi
Hypoparathyroidism is a rare disease characterized by low serum calcium levels and absent or deficient parathyroid hormone level. Regarding the epidemiology of chronic hypoparathyroidism, there are limited data in Italy and worldwide. Therefore, the purpose of this study was to build a unique database of patients with chronic hypoparathyroidism, derived from the databases of 16 referral centers for endocrinological diseases, affiliated with the Italian Society of Endocrinology, and four centers for endocrine surgery with expertise in hypoparathyroidism, to conduct an epidemiological analysis of chronic hypoparathyroidism in Italy...
August 2018: Calcified Tissue International
https://www.readbyqxmd.com/read/29477023/iron-overload-in-lower-international-prognostic-scoring-system-risk-patients-with-myelodysplastic-syndrome-receiving-red-blood-cell-transfusions-relation-to-infections-and-possible-benefit-of-iron-chelation-therapy
#20
Colleen A C Wong, Shannon A Y Wong, Heather A Leitch
BACKGROUND: An increased incidence of infections and infectious mortality has been reported in myelodysplastic syndromes (MDS) patients receiving red blood cell (RBC) transfusions. METHODS: We examined incidence of infections requiring antibiotics, antifungal or antiviral medications in transfused lower International Prognostic Scoring System (IPSS) risk MDS patients and whether this differed with iron chelation therapy (ICT). RESULTS: 138 transfused MDS patients were lower IPSS risk...
April 2018: Leukemia Research
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