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https://www.readbyqxmd.com/read/29350657/an-icet-a-survey-on-hypoparathyroidism-in-patients-with-thalassaemia-major-and-intermedia-a-preliminary-report
#1
Vincenzo De Sanctis, Ashraf T Soliman, Duran Canatan, Heba Elsedfy, Mehran Karimi, Shahina Daar, Hala Rimawi, Soteroula Christou, Nicos Skordis, Ploutarchos Tzoulis, Praveen Sobti, Shruti Kakkar, Yurdanur Kilinc, Doaa Khater, Saif A Alyaarubi, Valeriya Kaleva, Su Han Lum, Mohamed A Yassin, Forough Saki, Maha Obiedat, Salvatore Anastasi, Maria Concetta Galati, Giuseppe Raiola, Saveria Campisi, Nada Soliman, Mohamed Elshinawy, Soad Al Jaouni, Salvatore Di Maio, Yasser Wali, Ihab Zaki Elhakim, Christos Kattamis
Hypoparathyroidism (HPT) is a rare disease with leading symptoms of hypocalcemia, associated with high serum phosphorus levels and absent or inappropriately low levels of parathyroid hormone (PTH). In patients with thalassemias it is mainly attributed to transfusional iron overload, and suboptimal iron chelation therapy. The main objectives of this survey were to provide data on the prevalence, demographic and clinical features of HPT in thalassemia major (TM) and intermedia (TI) patients living in different countries, and to assess its impact in clinical medical practice...
January 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29342155/nanoscopic-x-ray-fluorescence-imaging-and-quantification-of-intracellular-key-elements-in-cryofrozen-friedreich-s-ataxia-fibroblasts
#2
Björn De Samber, Eline Meul, Brecht Laforce, Boel De Paepe, Joél Smet, Michiel De Bruyne, Riet De Rycke, Sylvain Bohic, Peter Cloetens, Rudy Van Coster, Peter Vandenabeele, Tom Vanden Berghe
Synchrotron radiation based nanoscopic X-ray fluorescence (SR nano-XRF) analysis can visualize trace level elemental distribution in a fully quantitative manner within single cells. However, in-air XRF analysis requires chemical fixation modifying the cell's chemical composition. Here, we describe first nanoscopic XRF analysis upon cryogenically frozen (-150°C) fibroblasts at the ID16A-NI 'Nano-imaging' end-station located at the European Synchrotron Radiation Facility (ESRF) in Grenoble (France). Fibroblast cells were obtained from skin biopsies from control and Friedreich's ataxia (FRDA) patients...
2018: PloS One
https://www.readbyqxmd.com/read/29338568/lactoferrin-plus-health-education-versus-total-dose-infusion-tdi-of-low-molecular-weight-lmw-iron-dextran-for-treating-iron-deficiency-anemia-ida-in-pregnancy-a-randomized-controlled-trial
#3
A M Darwish, Fouly Ha, W H Saied, E Farah
BACKGROUND: Iron deficiency anemia (IDA) is one of the most common medical disorder disturbing pregnancies particularly in low resources countries, and contributes significantly to morbidities and mortalities. Thus, early diagnosis and prompt management of IDA is highly recommended. AIM: To Test the efficacy and safety of oral lactoferrin plus health education provided by a nurse versus total dose infusion (TDI) of low-molecular weight (LMW) iron dextran for treating IDA in the second and third trimester of pregnancy...
January 16, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29335588/peripheral-iron-levels-in-children-with-attention-deficit-hyperactivity-disorder-a-systematic-review-and-meta-analysis
#4
Ping-Tao Tseng, Yu-Shian Cheng, Cheng-Fang Yen, 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, Pao-Yen Lin
There is growing recognition that the risk of attention-deficit hyperactivity disorder (ADHD) in children may be influenced by micronutrient deficiencies, including iron. We conducted this meta-analysis to examine the association between ADHD and iron levels/iron deficiency (ID). We searched for the databases of the PubMed, ScienceDirect, Cochrane CENTRAL, and ClinicalTrials.gov up to August 9th, 2017. Primary outcomes were differences in peripheral iron levels in children with ADHD versus healthy controls (HCs) and the severity of ADHD symptoms in children with/without ID (Hedges' g) and the pooled adjusted odds ratio (OR) of the association between ADHD and ID...
January 15, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29332607/the-role-of-t2-weighted-gradient-echo-in-the-diagnosis-of-tumefactive-intrahepatic-extramedullary-hematopoiesis-in-myelodysplastic-syndrome-and-diffuse-hepatic-iron-overload-a-case-report-and-review-of-the-literature
#5
Abel A Belay, Andrew M Bellizzi, Alan H Stolpen
BACKGROUND: Extramedullary hematopoiesis is the proliferation of hematopoietic cells outside bone marrow secondary to marrow hematopoiesis failure. Extramedullary hematopoiesis rarely presents as a mass-forming hepatic lesion; in this case, imaging-based differentiation from primary and metastatic hepatic neoplasms is difficult, often leading to biopsy for definitive diagnosis. We report a case of tumefactive hepatic extramedullary hematopoiesis in the setting of myelodysplastic syndrome with concurrent hepatic iron overload, and the role of T2*-weighted gradient-echo magnetic resonance imaging in differentiating extramedullary hematopoiesis from primary and metastatic hepatic lesions...
January 15, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29325618/neurodegeneration-with-brain-iron-accumulation
#6
Susan J Hayflick, Manju A Kurian, Penelope Hogarth
Neurodegeneration with brain iron accumulation (NBIA) comprises a clinically and genetically heterogeneous group of disorders affecting children and adults. These rare disorders are often first suspected when increased basal ganglia iron is observed on brain magnetic resonance imaging. For the majority of NBIA disorders the genetic basis has been delineated, and clinical testing is available. The four most common NBIA disorders include pantothenate kinase-associated neurodegeneration (PKAN) due to mutations in PANK2, phospholipase A2-associated neurodegeneration caused by mutation in PLA2G6, mitochondrial membrane protein-associated neurodegeneration from mutations in C19orf12, and beta-propeller protein-associated neurodegeneration due to mutations in WDR45...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29323501/the-pivotal-role-of-copper-in-neurodegeneration-a-new-strategy-for-the-therapy-of-neurodegenerative-disorders
#7
Roberta Giampietro, Francesco Spinelli, Marialessandra Contino, Nicola Antonio Colabufo
Copper is an essential trace element for human body since it is a cofactor of several enzymes and proteins and plays a pivotal role in several biological functions (e.g., respiration, protection from oxidative damage, iron metabolism, etc.), also including the central nervous system development and functioning (e.g. synthesis of neurotransmitters, myelination, activation of neuropeptides, etc.). Therefore, copper dysmetabolism is associated with different toxic effects, mainly represented by oxidative stress, and it has been reported in many neurodegenerative disorders, such as Wilson's disease, Menkes disease, Alzheimer's disease, Parkinson's disease, and Amyotrophic Lateral Sclerosis...
January 11, 2018: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/29318974/-understanding-the-role-of-hypoxia-inducible-factor-during-neurodegeneration-for-new-therapeutics-opportunities
#8
Amalia Merelli, Julio Cesar Garcia Rodriguez, Jaume Folch, Marcelo R Regueiro, Antoni Camins, Lazarowski Alberto
Neurodegeneration (NDG) is linked with the progressive loss of neural function with intellectual and/or motor impairment. Several diseases affecting older individuals, including Alzheimer's disease, Amyotrophic Lateral Sclerosis, Huntington's disease, Parkinson's disease, stroke, Multiple Sclerosis and many others, are the most relevant disorders associated with NDG. Since other pathologies such as refractory epilepsy, brain infections, or hereditary diseases such as "neurodegeneration with brain iron accumulation", also lead to chronic brain inflammation with loss of neural cells, NDG can be said to affect all ages...
January 10, 2018: Current Neuropharmacology
https://www.readbyqxmd.com/read/29315872/theoretical-investigations-on-the-mechanistic-aspects-of-o2-activation-by-a-biomimetic-di-nitrosyl-iron-complex
#9
Ambar Banerjee, Souloke Sen, Ankan Paul
Though Dinitrosyl-Iron complexes (DNICs) are largely believed to act as NO carriers, topical experiments on model DNICs have suggested that they can also act as Nitrating agents in presence of dioxygen. Oxygen activation by DNICs have been implicated as a possible route for protein tyrosine nitration (PTN), which leads to neurodegenerative disorders. Herein using static and dynamic theoretical techniques we unravel a previously unknown dual state mechanistic paradigm for dioxygen activation of a biomimetic nitrating DNIC complex leading to phenolic nitration...
January 7, 2018: Chemistry: a European Journal
https://www.readbyqxmd.com/read/29314418/cerebral-blood-volume-mapping-with-ferumoxytol-in-dynamic-susceptibility-contrast-perfusion-mri-comparison-to-standard-of-care
#10
Csanad G Varallyay, Eric Nesbit, Andrea Horvath, Peter Varallyay, Rongwei Fu, Seymur Gahramanov, Leslie L Muldoon, Xin Li, William D Rooney, Edward A Neuwelt
BACKGROUND: Cerebral blood volume (CBV) mapping with a dynamic susceptibility contrast (DSC) perfusion technique has become a clinical tool in diagnosing and follow-up of brain tumors. Ferumoxytol, a long-circulating iron oxide nanoparticle, has been tested for CBV mapping, but the optimal dose has not been established. PURPOSE: To compare ferumoxytol DSC of two different doses to standard of care gadoteridol by analyzing time-intensity curves and CBV maps in normal-appearing brain regions...
January 4, 2018: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29306510/protective-effects-of-artemisia-campestris-extract-against-gastric-acid-reflux-induced-esophageal-mucosa-injuries
#11
Mohamed-Amine Jabri, Haifa Tounsi, Afifa Abdellaoui, Lamjed Marzouki, Hichem Sebai
Artemisia campestris L. has been widely used in alternative medicine to treat digestive system diseases, particularly gastroesophageal disorders. In the present investigation, we studied the putative protective effect of Artemisia campestris aqueous extract (ACAE) against gastro-esophageal reflux (GER)-induced esophagitis in rats. The experimental ophagitis was induced by the ligation of the pylorus as well as the junction between the forestomach and the corpus. We firstly found that ACAE administration at 100, 200 and 400 mg/kg, b...
January 2, 2018: Pathophysiology: the Official Journal of the International Society for Pathophysiology
https://www.readbyqxmd.com/read/29305866/novel-and-rapid-osteoporosis-model-established-in-zebrafish-using-high-iron-stress
#12
Wenjuan Zhang, Jingjin Xu, Juhui Qiu, Cencan Xing, Xiumin Li, Bo Leng, Yi Su, Jinmei Lin, Jiaofen Lin, Xuqiao Mei, Yiqun Huang, Anming Meng, Yutian Pan, Yu Xue
Osteoporosis is a global public health concern and, it can result from numerous pathogenic mechanisms, many of which are closely related with age, nutritional disorders, endocrine imbalance, or adverse drug side effects presented by glucocorticoids, heparin, and anti-epileptics. Given its wide range etiologies, it is crucial to establish an animal model of osteoporosis for use in screening potential drugs quickly and effectively. Previous research has reported that an accumulation of elevated iron in the body is an independent risk factor for osteoporosis...
January 3, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29305416/involvement-of-hepcidin-in-iron-metabolism-dysregulation-in-gaucher-disease
#13
Thibaud Lefebvre, Niloofar Reihani, Raed Daher, Thierry Billette de Villemeur, Nadia Belmatoug, Christian Rose, Yves Colin-Aronovicz, Hervé Puy, Caroline Le Van Kim, Mélanie Franco, Zoubida Karim
Gaucher disease is an inherited deficiency of glucocerebrosidase leading to accumulation of glucosylceramide in tissues such as the spleen, liver, and bone marrow. The resulting lipid-laden macrophages lead to the appearance of Gaucher cells. Anemia associated with an unexplained hyperferritinemia is a frequent finding in Gaucher disease, but whether this pathogenesis is related to an iron metabolism disorder has remained unclear. To investigate this issue, we explored the iron status of a large cohort of 90 type I Gaucher disease patients, including 66 patients treated with enzyme replacement therapy...
January 5, 2018: Haematologica
https://www.readbyqxmd.com/read/29304922/the-appropriate-use-of-opioids-in-the-treatment-of-refractory-restless-legs-syndrome
#14
Michael H Silber, Philip M Becker, Mark J Buchfuhrer, Christopher J Earley, William G Ondo, Arthur S Walters, John W Winkelman
Restless legs syndrome (RLS) is a distinct disorder, differing from chronic pain in many ways. Refractory RLS is characterized by unresponsiveness to dopamine agonists or alpha-2-delta ligands due to inadequate efficacy, augmentation, or adverse effects. This may result in severely impaired quality of life, profound insomnia, and suicidal depression. Opioid therapy is a mainstay in the management of these patients. This article summarizes the basic science and clinical evidence in support of their use, including the positive result of a large controlled multicenter study of 306 subjects, and outlines an approach to their use in clinical practice...
January 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29301806/inherited-haemochromatosis-with-c282y-mutation-in-a-patient-with-alpha-thalassaemia-a-treatment-dilemma
#15
Mohammed Abdullah Al Qasem, Fayez Hanna, Usira S Vithanarachchi, Alhossain A Khalafallah
A Caucasian 24-year-old female patient suffers from two hereditary disorders: alpha-thalassaemia, which is prevalent in Asia and rare in Europe, and haemochromatosis, which is prevalent among northern Europe and rare in Asia. The clinical presentation and management of one of these diseases is controversial for the other. She presented 5 years ago with a clinical picture of refractory iron-deficiency anaemia secondary to menorrhagia. On treating her with the standard iron therapy, her anaemia persists although with adquate iron stores...
January 4, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29301508/quality-of-life-scores-differs-between-genotypic-groups-of-patients-with-suspected-hereditary-hemochromatosis
#16
Paula Fernanda Silva Fonseca, Rodolfo Delfini Cançado, Flavio Augusto Naoum, Carla Luana Dinardo, Guilherme Henrique Hencklain Fonseca, Sandra Fatima Menosi Gualandro, José Eduardo Krieger, Alexandre Costa Pereira, Pierre Brissot, Paulo Caleb Junior Lima Santos
BACKGROUND: Hereditary hemochromatosis (HH) encompasses a group of autosomal recessive disorders mainly characterized by enhanced intestinal absorption of iron and its accumulation in parenchymal organs. HH diagnosis is based on iron biochemical and magnetic resonance imaging (MRI) assessment, and genetic testing. Questionnaires, such as SF-36 (short form health survey), have been increasingly used to assess the impact of diseases on the patient's quality of life (QL). In addition, different genotypes are identified as results of genetic tests in patients with suspected primary iron overload...
January 5, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29299437/the-protective-effect-of-hydroalcoholic-extract-of-ginger-zingiber-officinale-rosc-against-iron-induced-functional-and-histological-damages-in-rat-liver-and-kidney
#17
Firouzeh Gholampour, Fatemeh Behzadi Ghiasabadi, Seyed Mohammad Owji, Jaafar Vatanparast
Objective: Iron overload in the body is related with toxic effects and threatens the health. The aim of this study was to evaluate the protective role of hydroalcoholic extract of ginger (Zingiber officinale) against ferrous sulfate-induced hepatic and renal functional disorders and histological damages in rats. Materials and Methods: The rats were divided into four groups (n=7): Sham, Sham + G.E (ginger extract, 400 mg/kg/day for 14 days), FS (ferrous sulfate, 30 mg/kg/day for 14 days), FS+G...
November 2017: Avicenna Journal of Phytomedicine
https://www.readbyqxmd.com/read/29299089/hamartoma-of-the-spleen-splenoma-with-calcifications-in-a-child-with-beta-thalassemia-a-case-report
#18
Dario Giambelluca, Dario Picone, Giuseppe Lo Re, Salvatore Pappalardo, Placido Romeo
Splenic hamartoma (or splenoma) is a rare, benign, vascular tumor, often incidentally found at imaging, surgery or autopsy. Albeit usually asymptomatic and rare in children, when it occurs in the pediatric population it is more commonly symptomatic. We report a case of a 15-year-old girl with iron-deficiency anemia and beta-thalassemia, who had a large (10 × 8 × 7 cm) splenic lesion with calcifications, incidentally found during follow-up for splenomegaly and histologically characterized as hamartoma with calcified areas...
May 2017: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/29298483/a-metal-protein-attenuating-compound-for-pet-neuroimaging-synthesis-and-preclinical-evaluation-of-11c-pbt2
#19
Hema S Krishnan, Vadim Bernard-Gauthier, Michael Stephen Placzek, Kenneth Dahl, Vidya Narayanaswami, Elijahu Livni, Zhen Chen, Jing Yang, Thomas Lee Collier, Chongzhao Ran, Jacob M Hooker, Steven H Liang, Neil Vasdev
Dyshomeostasis or abnormal accumulation of metal ions such as copper, zinc and iron have been linked to the pathogenesis of multiple neurodegenerative disorders including Alzheimer's disease (AD) and Huntington's disease (HD). 5,7-Dichloro-2-((dimethylamino)methyl)quinolin-8-ol, PBT2, is a second generation metal protein-attenuating compound that has recently advanced in Phase II clinical trials for the treatment of AD and HD based on promising preclinical efficacy data. Herein, we report the first radiosynthesis and preclinical positron emission tomography (PET) neuroimaging evaluation of [11C]PBT2 in rodents and nonhuman primates...
January 3, 2018: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/29289598/why-should-neuroscientists-worry-about-iron-the-emerging-role-of-ferroptosis-in-the-pathophysiology-of-neuroprogressive-diseases
#20
REVIEW
Gerwyn Morris, Michael Berk, André F Carvalho, Michael Maes, Adam J Walker, Basant K Puri
Ferroptosis is a unique form of programmed death, characterised by cytosolic accumulation of iron, lipid hydroperoxides and their metabolites, and effected by the fatal peroxidation of polyunsaturated fatty acids in the plasma membrane. It is a major driver of cell death in neurodegenerative neurological diseases. Moreover, cascades underpinning ferroptosis could be active drivers of neuropathology in major psychiatric disorders. Oxidative and nitrosative stress can adversely affect mechanisms and proteins governing cellular iron homeostasis, such as the iron regulatory protein/iron response element system, and can ultimately be a source of abnormally high levels of iron and a source of lethal levels of lipid membrane peroxidation...
December 28, 2017: Behavioural Brain Research
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