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Iron overload

Y J Deng, X Y Zheng, T Tu
No abstract text is available yet for this article.
March 24, 2018: Zhonghua Xin Xue Guan Bing za Zhi
Feibi Zeng, Munenobu Nogami, Taiichiro Shirai, Yoh Zen, Takamichi Murakami
Iron overload is a major complication in blood transfusion procedures. This report presents a case of malignant lymphoma, in which the findings of bone marrow involvement were discordant between F-FDG PET imaging studies and MRI with diffusion-weighted imaging studies. In this case, the PET imaging studies were positive for malignant lymphoma, whereas the MRI studies were negative. Iron deposition in the reticuloendothelial system due to posttransfusion iron overload was considered to be the cause of the decrease in signal intensity on diffusion-weighted imaging, resulting in a false-negative finding on MRI...
March 20, 2018: Clinical Nuclear Medicine
Erdem Kucukal, Jane A Little, Umut A Gurkan
Non-adherence and deformability are the key intrinsic biomechanical features of the red blood cell (RBC), which allow it to tightly squeeze and pass through even the narrowest of microcirculatory networks. Blockage of microcirculatory flow, also known as vaso-occlusion, is a consequence of abnormal cellular adhesion to the vascular endothelium. In sickle cell disease (SCD), an inherited anaemia, even though RBCs have been shown to be heterogeneous in adhesiveness and deformability, this has not been studied in the context of physiologically relevant dynamic shear gradients at the microscale...
March 20, 2018: Integrative Biology: Quantitative Biosciences From Nano to Macro
Antonio Layoun, Macha Samba-Mondonga, Gabriela Fragoso, Annie Calvé, Manuela M Santos
Iron homeostasis is tightly regulated to provide virtually all cells in the body, particularly red blood cells, with this essential element while defending against its toxicity. The peptide hormone hepcidin is central to the control of the amount of iron absorbed from the diet and iron recycling from macrophages. Previously, we have shown that hepcidin induction in macrophages following Toll-like receptor (TLR) stimulation depends on the presence of myeloid differentiation primary response gene 88 (MyD88). In this study, we analyzed the regulation of iron metabolism in MyD88 -/- mice to further investigate MyD88 involvement in iron sensing and hepcidin induction...
2018: Frontiers in Physiology
Kalyani Raju, Srinivas Murthy Venkataramappa
Hemochromatosis is an autosomal recessive genetic disorder resulting in increased intestinal absorption of iron and eventually to iron overload. The onset of symptoms is usually seen around 40 years of age. Iron overload causes tissue damage in liver, pancreas, skin, joints, heart, and gonads. Approximately 50% of patients diagnosed with hemochromatosis will have either type 1 or type 2 diabetes mellitus (DM) because of selective beta-cell damage due to iron overload and leads to impaired insulin synthesis, release, and insulin resistance...
January 2018: International Journal of Applied and Basic Medical Research
Rachael F Grace, Paola Bianchi, Eduard J van Beers, Stefan W Eber, Bertil Glader, Hassan M Yaish, Jenny M Despotovic, Jennifer A Rothman, Mukta Sharma, Melissa M McNaull, Elisa Fermo, Kimberly Lezon-Geyda, D Holmes Morton, Ellis J Neufeld, Satheesh Chonat, Nina Kollmar, Christine M Knoll, Kevin Kuo, Janet L Kwiatkowski, Dagmar Pospíŝilová, Yves D Pastore, Alexis A Thompson, Peter E Newburger, Yaddanapudi Ravindranath, Winfred C Wang, Marcin W Wlodarski, Heng Wang, Susanne Holzhauer, Vicky R Breakey, Joachim Kunz, Sujit Sheth, Melissa J Rose, Heather A Bradeen, Nolan Neu, Dongjing Guo, Hasan Al-Sayegh, Wendy B London, Patrick G Gallagher, Alberto Zanella, Wilma Barcellini
An international, multicenter registry was established to collect retrospective and prospective clinical data on patients with pyruvate kinase (PK) deficiency, the most common glycolytic defect causing congenital non-spherocytic hemolytic anemia. Medical history and laboratory and radiologic data were retrospectively collected at enrollment in 254 patients with molecularly confirmed PK deficiency. Perinatal complications were common, including anemia requiring transfusions, hyperbilirubinemia, hydrops, and prematurity...
March 16, 2018: Blood
Carles Díez-López, Josep Comín-Colet, José González-Costello
PURPOSE OF REVIEW: Iron overload cardiomyopathy (IOC) is an important predictor of prognosis in a significant number of patients with hereditary hemochromatosis and hematologic diseases. Its prevalence is increasing because of improved treatment strategies, which significantly improve life expectancy. We will review diagnosis, treatment, and recent findings in the field. RECENT FINDINGS: The development of preclinical translational disease models during the last years have helped our understanding of specific disease pathophysiological pathways that might eventually change the outcomes of these patients...
March 14, 2018: Current Opinion in Cardiology
Charikleia Kelaidi, Antonis Kattamis, Filia Apostolakou, Christos Poziopoulos, Christina Lazaropoulou, Polyxeni Delaporta, Ino Kanavaki, Ioannis Papassotiriou
BACKGROUND: Levels of the angiogenic cytokines Placental Growth Factor (PlGF) and soluble Fms-Like Tyrosine Kinase-1 (sFlt-1) and the angiogenic balance, expressed by sFlt-1/PlGF ratio, are perturbed in Sickle Cell Disease and iron overload, but they have not been evaluated in Non-Transfusion-Dependent Thalassemia (NTDT). PATIENTS AND METHODS: We measured levels of PlGF, sFlt-1 and vWF:antigen in patients with NTDT of beta thalassemia genotype, and correlated them with erythrocytic indices and markers of iron overload, inflammation and tissue hypoxia...
March 15, 2018: European Journal of Haematology
Roxanne Labranche, Guillaume Gilbert, Milena Cerny, Kim-Nhien Vu, Denis Soulières, Damien Olivié, Jean-Sébastien Billiard, Takeshi Yokoo, An Tang
Iron overload is a systemic disorder and is either primary (genetic) or secondary (exogenous iron administration). Primary iron overload is most commonly associated with hereditary hemochromatosis and secondary iron overload with ineffective erythropoiesis (predominantly caused by β-thalassemia major and sickle cell disease) that requires long-term transfusion therapy, leading to transfusional hemosiderosis. Iron overload may lead to liver cirrhosis and hepatocellular carcinoma, in addition to cardiac and endocrine complications...
March 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Ilknur Kozanoglu, Hakan Ozdogu
Sickle cell disease (SCD) is a life-threatening chronic condition primarily caused by genetic mutation. The disease is characterized by intermittent vaso-occlusive events and chronic hemolytic anemia. Acute complications in patients with SCD are difficult to manage due to the pathophysiological nature of the disease. Transfusion therapy is the cornerstone of management of acute complications and significantly reduces SCD morbidity and mortality. Red cell exchange (RCE), which is characterized by low iron accumulation and volume overload, has been widely used for transfusion therapy in recent years...
February 21, 2018: Transfusion and Apheresis Science
Carla Casu, Elizabeta Nemeth, Stefano Rivella
Hepcidin agonists are a new class of compounds that regulate blood iron levels and limit iron absorption, and could improve the treatment of hemochromatosis, β-thalassemia, polycythemia vera, and other disorders where disrupted iron homeostasis causes disease or contributes to it. Hepcidin agonists also have the potential to prevent severe complications of siderophilic infections in patients with iron overload or chronic liver disease. This review highlights the preclinical studies that support the development of hepcidin agonists for the treatment of these disorders...
March 9, 2018: Blood
Amy D Bradshaw, Kristine Y DeLeon-Pennell
No abstract text is available yet for this article.
March 9, 2018: American Journal of Physiology. Heart and Circulatory Physiology
Mather A Khan, Norma A Castro-Guerrero, Samuel A McInturf, Nga T Nguyen, Ashley N Dame, Jiaojiao Wang, Rebecca K Bindbeutel, Trupti Joshi, Silvia S Jurisson, Dmitri A Nusinow, David G Mendoza-Cozatl
The OLIGOPEPTIDE TRANSPORTER 3 (OPT3) has recently been identified as a component of the systemic network mediating iron (Fe) deficiency responses in Arabidopsis. Reduced expression of OPT3 induces an over accumulation of Fe in roots and leaves, due in part by an elevated expression of the IRON-REGULATED TRANSPORTER 1 (IRT1). Here we show however, that opt3 leaves display a transcriptional program consistent with an Fe overload, suggesting that Fe excess is properly sensed in opt3 leaves and that the OPT3-mediated shoot-to-root signaling is critical to prevent a systemic Fe overload...
March 8, 2018: Plant, Cell & Environment
Naveen L Pereira, Martha Grogan, G William Dec
Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies...
March 13, 2018: Journal of the American College of Cardiology
Naveen L Pereira, Martha Grogan, G William Dec
Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies...
March 13, 2018: Journal of the American College of Cardiology
Huimin Lin, Caixia Fu, Stephan Kannengiesser, Shu Cheng, Jun Shen, Haipeng Dong, Fuhua Yan
BACKGROUND: The coexistence of hepatic iron and fat is common in patients with hyperferritinemia, which plays an interactive and aggressive role in the progression of diseases (fibrosis, cirrhosis, and hepatocellular carcinomas). PURPOSE: To evaluate a modified high-speed T2 -corrected multi-echo, single voxel spectroscopy sequence (HISTOV) for liver iron concentration (LIC) quantification in patients with hyperferritinemia, with simultaneous fat fraction (FF) estimation...
March 7, 2018: Journal of Magnetic Resonance Imaging: JMRI
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...
March 6, 2018: Calcified Tissue International
Shenglong Cao, Ya Hua, Richard F Keep, Neeraj Chaudhary, Guohua Xi
BACKGROUND AND PURPOSE: Brain iron overload is a key factor causing brain injury after intracerebral hemorrhage (ICH). This study quantified brain iron levels after ICH with magnetic resonance imaging R2* mapping. The effect of minocycline on iron overload and ICH-induced brain injury in aged rats was also determined. METHODS: Aged (18 months old) male Fischer 344 rats had an intracerebral injection of autologous blood or saline, and brain iron levels were measured by magnetic resonance imaging R2* mapping...
March 6, 2018: Stroke; a Journal of Cerebral Circulation
Alessia Pepe, Maria R Gamberini, Massimiliano Missere, Laura Pistoia, Maurizio Mangione, Liana Cuccia, Anna Spasiano, Silvia Maffei, Christian Cadeddu, Massimo Midiri, Caterina Borgna, Antonella Meloni
We assessed whether male gender was associated with a higher risk of cardiac iron accumulation and fibrosis, heart dysfunction and complications in a large, multicentre cohort of thalassaemia major (TM) patients, in order to optimize the timing in cardiac follow-up. We considered 1711 TM patients (899 females, 31·09 ± 9·08 years), enrolled in the Myocardial Iron Overload in Thalassaemia Network. Clinical/instrumental data are recorded from birth to the first Cardiovascular Magnetic Resonance Imaging scan...
March 2018: British Journal of Haematology
Sara Pelucchi, Raffaella Mariani, Giulia Ravasi, Irene Pelloni, Massimo Marano, Lucio Tremolizzo, Massimo Alessio, Alberto Piperno
INTRODUCTION: Aceruloplasminemia is an ultra-rare hereditary disorder characterized by iron-restricted microcytic anemia and tissue iron overload associated with diabetes, retinal and progressive neurological degeneration. We describe genotypes and phenotypes at diagnosis, and disease evolution of seven Italian patients. METHODS: Anagraphical, biochemical, genetic, clinical and instrumental data were collected at diagnosis and during a long-term follow-up. Mutations, ferroxidase activity and Western Blot analysis of ceruloplasmin were performed according to standard protocols...
February 24, 2018: Parkinsonism & related Disorders
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