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Iron disorders

Sven Haller, Meike W Vernooij, Joost P A Kuijer, Elna-Marie Larsson, Hans Rolf Jäger, Frederik Barkhof
Cerebral microbleeds (CMBs), also referred to as microhemorrhages, appear on magnetic resonance (MR) images as hypointense foci notably at T2*-weighted or susceptibility-weighted (SW) imaging. CMBs are detected with increasing frequency because of the more widespread use of high magnetic field strength and of newer dedicated MR imaging techniques such as three-dimensional gradient-echo T2*-weighted and SW imaging. The imaging appearance of CMBs is mainly because of changes in local magnetic susceptibility and reflects the pathologic iron accumulation, most often in perivascular macrophages, because of vasculopathy...
April 2018: Radiology
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
Shihui Hao, Bishan Liang, Qiong Huang, Shumin Dong, Zhenzhen Wu, Wanming He, Min Shi
Ferroptosis is an iron-dependent and peroxidation-driven form of cell death associated with multiple metabolic disorders and disrupted homeostasis. A number of metabolic processes and homeostasis are affected by ferroptosis. The molecules that regulate ferroptosis are involved in metabolic pathways that regulate cysteine exploitation, glutathione state, nicotinamide adenine dinucleotide phosphate function, lipid peroxidation and iron homeostasis. The present review summarizes the metabolic networks involved in ferroptosis based on previous studies, and discusses the function of ferroptosis in pathological processes, including cancer...
April 2018: Oncology Letters
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
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
Ping-Tao Tseng, Yu-Shian Cheng, Yen-Wen Chen, Brendon Stubbs, Paul Whiteley, Andre F Carvalho, Dian-Jeng Li, Tien-Yu Chen, Wei-Cheng Yang, Chia-Hung Tang, Che-Sheng Chu, Wei-Chieh Yang, Hsin-Yi Liang, Ching-Kuan Wu, Cheng-Fang Yen, Pao-Yen Lin
Autism spectrum disorder (ASD) is a common neurodevelopmental disorder, and nutritional deficiency may play a role in the development of ASD. A relationship between ASD and iron levels/iron deficiency (ID) has been reported; however, the results have been inconsistent. Therefore, we conducted this meta-analysis to examine the relationship between ASD and ID following the Meta-Analysis of Observational Studies in Epidemiology guidelines. We performed a systematic search of PubMed, ScienceDirect, Embase, ProQuest, ClinicalTrials...
February 2018: Nutrition Research
Selin Elmaoğulları, Zeyra Aycan
Abnormal uterine bleeding (AUB) is the major gynecologic complaint of adolescents admitting to hospital. Heavy menstrual bleeding (HMB) is the most common clinical presentation of AUB. Anovulatory cycles owing to immature hypothalamic-pituitary-ovarian axis is the leading etiology of HMB and there is an accompanying bleeding disorder in almost %20 of patients with HMB. Additionally, endocrine disorders such as hypothyroidism, hyperprolactinemia and polycystic ovary syndrome (PCOS) are possible causes of AUB...
February 28, 2018: Journal of Clinical Research in Pediatric Endocrinology
Bushra Moiz
Koilonychia is a nail abnormality characterized by thin, brittle, and spoon-shaped nails. It is frequently observed in chronic iron deficiency secondary to malnutrition, chronic blood loss, or malabsorption. It may also be idiopathic or related to occupation and rare systemic disorders. Presence of koilonychia should prompt investigations for iron deficiency.
March 2018: Clinical Case Reports
Josep Estadella, Laura Villamarín, Anna Feliu, Josep Perelló, Joaquim Calaf
Iron deficiency anemia is the most frequent cause of anemia world-wide and is a very common disorder in daily medical practice. Heavy menstrual bleeding (menorrhagia) and pregnancy and delivery can cause significant iron loss leading to severe anemia The aim of the present study was to characterize the population requiring intravenous iron and identify whether gynecological and obstetric iron loss are frequent indications for treatment. MATERIAL AND METHODS: Restrospective, single center study performed in a tertiary level university hospital from January 2014 to December 2016...
March 6, 2018: European Journal of Obstetrics, Gynecology, and Reproductive Biology
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
Tasneem S Alaqzam, Angela C Stanley, Pippa M Simpson, Veronica H Flood, Seema Menon
STUDY OBJECTIVE: This study sought to determine the relationship of bleeding disorders to iron deficiency anemia. Additionally, this study was undertaken to examine all current treatment modalities used in a menorrhagia clinic with respect to heavy menstrual bleeding management to identify the most effective options for menstrual management in the setting of an underlying bleeding disorder. DESIGN, SETTING, PARTICIPANT, INTERVENTION, AND MAIN OUTCOME MEASURES: Retrospective chart review of adolescent <21 years with heavy menstrual bleeding attending a multidisciplinary hematology-adolescent gynecology clinic...
March 7, 2018: Journal of Pediatric and Adolescent Gynecology
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
Gabriel Qualhato, Simone Maria Teixeira de Sabóia-Morais, Luciana Damacena Silva, Thiago Lopes Rocha
Although iron oxide nanoparticles (IONPs) have been widely used in nanomedicine and nanoremediation, their ecotoxicological effects on aquatic organisms remain unclear. In this study, the melanomacrophage center (MMC) response and hepatic histopathologic biomarkers were investigated in female guppies, Poecilia reticulata, exposed to citrate-functionalized IONPs (γ-Fe2 O3 ) at an environmentally relevant iron concentration (0.3 mg L-1 ) over 21 days. The animals were collected at the beginning of the experiment and after 3, 7, 14, and 21 days of exposure...
February 21, 2018: Aquatic Toxicology
Sumit Sahni, Jason R Hickok, Douglas D Thomas
The chelatable iron pool (CIP) is a small but chemically significant fraction of total cellular iron. While this dynamic population of iron is limited, it is redox active and capable of generating reactive oxygen species (ROS) that can lead to oxidative stress which is associated with various pathologies. Nitric oxide (•NO), is a free radical signalling molecule that regulates numerous physiological and pathological conditions. We have previously shown that macrophages exposed to endogenously generated or exogenously administered nitric oxide (•NO) results in its interaction with CIP to form dinitrosyliron complexes with thiol containing ligands (DNICs)...
March 6, 2018: Nitric Oxide: Biology and Chemistry
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
Vera Cuartero, Javier Blasco, Gloria Subías, Joaquín García, José A Rodríguez-Velamazán, Clemens Ritter
The effect of substituting iron and zinc for cobalt in CaBaCo4 O7 was investigated using neutron diffraction and X-ray absorption spectroscopy techniques. The orthorhombic distortion present in the parent compound CaBaCo4 O7 decreases with increasing the content of either Fe or Zn. The samples CaBaCo3 ZnO7 and CaBaCo4- x Fex O7 with x ≥ 1.5 are metrically hexagonal, but much better refinements in the neutron diffraction patterns are obtained using an orthorhombic unit cell. The two types of substitution have opposite effects on the structural and magnetic properties...
March 8, 2018: Inorganic Chemistry
Jingshu Xu, Stephanie J Church, Stefano Patassini, Paul Begley, Katherine A B Kellett, Emma R L C Vardy, Richard D Unwin, Nigel M Hooper, Garth J S Cooper
Sporadic Alzheimer's disease (AD) is a neurodegenerative disorder that causes the most prevalent form of age-related dementia but its pathogenesis remains obscure. Altered regulation of metals, particularly pan-cerebral copper deficiency, and more regionally-localized perturbation of other metals, are prominent in AD brain although data on how these CNS perturbations are reflected in the peripheral bloodstream are inconsistent to date. To assess the potential use of metal dysregulation to generate biomarkers in AD, we performed a case-control study of seven essential metals and selenium, measured by inductively coupled plasma mass-spectrometry, in samples from AD and matched control cases...
March 7, 2018: Biometals: An International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine
José Teixeira, Catarina Oliveira, Fernando Cagide, Ricardo Amorim, Jorge Garrido, Fernanda Borges, Paulo J Oliveira
Pharmacological interventions targeting mitochondria present several barriers for a complete efficacy. Therefore, a new mitochondriotropic antioxidant (AntiOxBEN3 ) based on the dietary antioxidant gallic acid was developed. AntiOxBEN3 accumulated several thousand-fold inside isolated rat liver mitochondria, without causing disruption of the oxidative phosphorylation apparatus, as seen by the unchanged respiratory control ratio, phosphorylation efficiency, and transmembrane electric potential. AntiOxBEN3 showed also limited toxicity on human hepatocarcinoma cells...
December 2018: Journal of Enzyme Inhibition and Medicinal Chemistry
Mauricio Cardenas-Rodriguez, Afroditi Chatzi, Kostas Tokatlidis
Iron-sulfur clusters are ubiquitous inorganic co-factors that contribute to a wide range of cell pathways including the maintenance of DNA integrity, regulation of gene expression and protein translation, energy production, and antiviral response. Specifically, the iron-sulfur cluster biogenesis pathways include several proteins dedicated to the maturation of apoproteins in different cell compartments. Given the complexity of the biogenesis process itself, the iron-sulfur research area constitutes a very challenging and interesting field with still many unaddressed questions...
March 6, 2018: Journal of Biological Inorganic Chemistry: JBIC
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