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Iron disorders

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https://www.readbyqxmd.com/read/28650631/effect-of-the-biphenyl-neolignan-honokiol-on-a%C3%AE-42-induced-toxicity-in-caenorhabditis-elegans-a%C3%AE-42-fibrillation-cholinesterase-activity-dpph-radicals-and-iron-ii-chelation
#1
Srinivas Kantham, Stephen Chan, Gawain McColl, Jared Miles, Suresh Kumar Veliyath, Girdhar Singh Deora, Satish N Dighe, Samira Khabbazi, Marie-Odile Parat, Benjamin P Ross
The biphenyl neolignan honokiol is a neuroprotectant which has been proposed as a treatment for central nervous system disorders such as Alzheimer's disease (AD). The death of cholinergic neurons in AD is attributed to multiple factors, including: accumulation and fibrillation of amyloid beta peptide (Aβ) within the brain; metal ion toxicity; and oxidative stress. In this study, we used a transgenic Caenorhabditis elegans model expressing full length Aβ42 as a convenient in vivo system for examining the effect of honokiol against Aβ-induced toxicity...
June 26, 2017: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/28648505/hypothyroidism-associated-with-parathyroid-disorders
#2
REVIEW
Giovanna Mantovani, Francesca Marta Elli, Sabrina Corbetta
Hypothyroidism may occur in association with congenital parathyroid disorders determining parathyroid hormone insufficiency, which is characterized by hypocalcemia and concomitant inappropriately low secretion of parathormone (PTH). The association is often due to loss of function of genes common to thyroid and parathyroid glands embryonic development. Hypothyroidism associated with hypoparathyroidism is generally mild and not associated with goiter; moreover, it is usually part of a multisystemic involvement not restricted to endocrine function as occurs in patients with 22q11 microdeletion/DiGeorge syndrome, the most frequent disorders...
March 2017: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28644353/extraintestinal-manifestations-of-celiac-disease-effectiveness-of-the-gluten-free-diet
#3
Hilary Jericho, Naire Sansotta, Stefano Guandalini
OBJECTIVE: The aim of the study was to evaluate the effectiveness of the gluten-free diet (GFD) on extraintestinal symptoms in pediatric and adult celiac populations at the University of Chicago. METHODS: We conducted a retrospective chart review of the University of Chicago Celiac Center clinic charts from January 2002 to October 2014. Demographics, serologic testing, intestinal biopsies, and extraintestinal symptoms at presentation, 12, 24, and >24 months were recorded...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28643901/ferric-carboxymaltose-in-patients-with-restless-legs-syndrome-and-nonanemic-iron-deficiency-a-randomized-trial
#4
Claudia Trenkwalder, Juliane Winkelmann, Wolfgang Oertel, Garth Virgin, Bernard Roubert, Anna Mezzacasa
BACKGROUND: Compromised iron status is important in restless legs syndrome pathophysiology. We compared the efficacy and tolerability of ferric carboxymaltose (single intravenous dose) versus placebo for restless legs syndrome treatment in iron-deficient nonanemic patients. METHODS: Patients with moderate to severe restless legs syndrome and serum ferritin < 75 μg/L (or serum ferritin 75-300 μg/L and transferrin saturation < 20%) were randomized to ferric carboxymaltose (1000 mg iron) or placebo...
June 23, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28643035/clinical-and-imaging-presentation-of-a%C3%A2-patient-with-beta-propeller-protein-associated-neurodegeneration-a%C3%A2-rare-and-sporadic-form-of-neurodegeneration-with-brain-iron-accumulation-nbia
#5
Elke Hattingen, Nikolaus Handke, Kirsten Cremer, Sabine Hoffjan, Guido Matthias Kukuk
Neurodegeneration with brain iron accumulation (NBIA) is a heterogeneous group of inherited neurologic disorders with iron accumulation in the basal ganglia, which share magnetic resonance (MR) imaging characteristics, histopathologic and clinical features. According to the affected basal nuclei, clinical features include extrapyramidal movement disorders and varying degrees of intellectual disability status. The most common NBIA subtype is caused by pathogenic variants in PANK2. The hallmark of MR imaging in patients with PANK2 mutations is an eye-of-the-tiger sign in the globus pallidus...
June 22, 2017: Clinical Neuroradiology
https://www.readbyqxmd.com/read/28641412/anti-perovskite-li-battery-cathode-materials
#6
Kwing To Lai, Iryna Antonyshyn, Yurii Prots, Martin Valldor
Through single-step solid-state reactions, a series of novel bichalcogenides with the general composition (Li2Fe)ChO (Ch = S, Se, Te) are successfully synthesized. (Li2Fe)ChO (Ch = S, Se) possess cubic anti-perovskite crystal structures, where Fe and Li are completely disordered on a common crystallographic site (3c). According to Goldschmidt calculations, Li(+) and Fe(2+) are too small for their common atomic position and exhibit large thermal displacements in the crys-tal structure models, implying high cation mobility...
June 23, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28638652/crystal-structure-of-a-silver-cobalt-and-iron-based-phosphate-with-an-alluaudite-like-structure-ag1-655co1-64fe1-36-po4-3
#7
Adam Bouraima, Thomas Makani, Abderrazzak Assani, Mohamed Saadi, Lahcen El Ammari
The new silver-, cobalt- and iron-based phosphate, silver cobalt iron tris(ortho-phosphate), Ag1.655Co1.64Fe1.36(PO4)3, was synthesized by solid-state reactions. Its structure is isotypic to that of Na2Co2Fe(PO4)3, and belongs to the alluaudite family, with a partial cationic disorder, the Ag(I) atoms being located on an inversion centre and twofold rotation axis sites (Wyckoff positions 4a and 4e), with partial occupancies of 0.885 (2) and 0.7688 (19), respectively. One of the two P atoms in the asymmetric unit completely fills one 4e site while the Co and Fe atoms fill another 4e site, with partial occupancies of 0...
June 1, 2017: Acta Crystallographica. Section E, Crystallographic Communications
https://www.readbyqxmd.com/read/28636169/a-novel-double-nucleotide-variant-in-the-ferritin-l-iron-responsive-element-in-a-finnish-patient-with-hereditary-hyperferritinaemia-cataract-syndrome
#8
Roosa-Maria Mattila, Annele Sainio, Marketta Järveläinen, Juha Pursiheimo, Hannu Järveläinen
PURPOSE: To present a novel Finnish double nucleotide variant in the iron-responsive element (IRE) of the ferritin L-chain gene (FTL) leading to hyperferritinaemia-cataract syndrome (HHCS). METHODS: Genomic DNA extracted from peripheral blood leucocytes and synthetized with three different primers flanking the IRE in the FTL 5'-untranslated region of the FTL was used in polymerase chain reaction (PCR). Thereafter, Sanger sequencing was performed on the 487-bp and 602-bp PCR amplification products with specific primers to reveal FTL IRE mutations...
June 21, 2017: Acta Ophthalmologica
https://www.readbyqxmd.com/read/28629957/chemical-hand-warmer-packet-ingestion-a-case-of-elemental-iron-exposure
#9
Jessica L Weiland, Leighanne K Sherrow, Deepak A Jayant, Kenneth D Katz
For individuals who work outdoors in the winter or play winter sports, chemical hand warmers are becoming increasingly more commonplace because of their convenience and effectiveness. A 32-year-old woman with a history of chronic pain and bipolar disorder presented to the emergency department complaining of a "warm sensation" in her mouth and epigastrium after reportedly ingesting the partial contents of a chemical hand warmer packet containing between 5 and 8 g of elemental iron. She had been complaining of abdominal pain for approximately 1 month and was prescribed unknown antibiotics the previous day...
June 16, 2017: Wilderness & Environmental Medicine
https://www.readbyqxmd.com/read/28628361/lipocalin-2-an-emerging-player-in-iron-homeostasis-and-inflammation
#10
Xia Xiao, Beng San Yeoh, Matam Vijay-Kumar
Lipocalin 2 (Lcn2), an innate immune protein, has emerged as a critical iron regulatory protein during physiological and inflammatory conditions. As a bacteriostatic factor, Lcn2 obstructs the siderophore iron-acquiring strategy of bacteria and thus inhibits bacterial growth. As part of host nutritional immunity, Lcn2 facilitates systemic, cellular, and mucosal hypoferremia during inflammation, in addition to stabilizing the siderophore-bound labile iron pool. In this review, we summarize recent advances in understanding the interaction between Lcn2 and iron, and its effects in various inflammatory diseases...
June 19, 2017: Annual Review of Nutrition
https://www.readbyqxmd.com/read/28626420/restless-legs-syndrome-from-pathophysiology-to-clinical-diagnosis-and-management
#11
REVIEW
Shiyi Guo, Jinsha Huang, Haiyang Jiang, Chao Han, Jie Li, Xiaoyun Xu, Guoxin Zhang, Zhicheng Lin, Nian Xiong, Tao Wang
Restless legs syndrome (RLS), a common neurological sensorimotor disorder in western countries, has gained more and more attention in Asian countries. The prevalence of RLS is higher in older people and females. RLS is most commonly related to iron deficiency, pregnancy and uremia. The RLS symptoms show a significant circadian rhythm and a close relationship to periodic limb movements (PLMs) in clinical observations, while the pathophysiological pathways are still unknown. The diagnostic criteria have been revised in 2012 to improve the validity of RLS diagnosis...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28623611/deferiprone-rescues-behavioral-deficits-induced-by-mild-iron-exposure-in-a-mouse-model-of-alpha-synuclein-aggregation
#12
Eleonora Carboni, Lars Tatenhorst, Lars Tönges, Elisabeth Barski, Vivian Dambeck, Mathias Bähr, Paul Lingor
Parkinson's disease (PD) is the most common neurodegenerative movement disorder, and its causes remain unknown. A major hallmark of the disease is the increasing presence of aggregated alpha-synuclein (aSyn). Furthermore, there is a solid consensus on iron (Fe) accumulation in several regions of PD brains during disease progression. In our study, we focused on the interaction of Fe and aggregating aSyn in vivo in a transgenic mouse model overexpressing human aSyn bearing the A53T mutation (prnp.aSyn.A53T). We utilized a neonatal iron-feeding model to exacerbate the motor phenotype of the transgenic mouse model...
June 16, 2017: Neuromolecular Medicine
https://www.readbyqxmd.com/read/28622780/-iron-metabolism
#13
S Vaulont
Iron is an essential trace element ensuring many functions in the body. However, excess iron can be toxic with deleterious consequences on function and tissue integrity. The understanding of the molecular and cellular mechanisms allowing iron level to be kept at physiological concentration has greatly progressed in recent years, in particular with the identification of the iron-regulatory hormone, hepcidin and its receptor ferroportin, the sole iron exporter known to date. This discovery has improved our ability to diagnose and manage iron disorders and offered new therapeutic perspectives for an important class of human diseases...
May 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28621798/the-relationship-of-serum-adipokines-with-malnutrition-inflammation-score-in-hemodialysis
#14
Elham Alipoor, Ahmad Esmaillzadeh, Mitra Mahdavi-Mazdeh, Mehdi Yaseri, Narges Sadat Zahed, Mohammad Javad Hosseinzadeh-Attar
BACKGROUND: Protein-energy wasting is a prevalent disorder in hemodialysis. Zinc-α2-glycoprotein (ZAG) and adipose triglyceride lipase (ATGL) are novel adipokines with recognized lipolytic effects and proposed role in metabolic homeostasis. This study was conducted to investigate the association of ZAG and ATGL concentrations with malnutrition inflammation score (MIS) and metabolic profile of patients with hemodialysis. MATERIALS AND METHODS: Eighty-eight patients under regular hemodialysis divided based on MIS to normal to mild wasting (NMW; n=35) or moderate wasting (MW; n=53) groups...
June 16, 2017: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28620665/influence-of-iron-metabolism-on-manganese-transport-and-toxicity
#15
REVIEW
Qi Ye, Jo Eun Park, Kuljeet Gugnani, Swati Betharia, Alejandro Pino-Figueroa, Jonghan Kim
Although manganese (Mn) is critical for the proper functioning of various metabolic enzymes and cofactors, excess Mn in the brain causes neurotoxicity. While the exact transport mechanism of Mn has not been fully understood, several importers and exporters for Mn have been identified over the past decade. In addition to Mn-specific transporters, it has been demonstrated that iron transporters can mediate Mn transport in the brain and peripheral tissues. However, while the expression of iron transporters is regulated by body iron stores, whether or not disorders of iron metabolism modify Mn homeostasis has not been systematically discussed...
June 16, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/28619730/consumption-of-a-high-iron-diet-disrupts-homeostatic-regulation-of-intestinal-copper-absorption-in-adolescent-mice
#16
Jung-Heun Ha, Caglar Doguer, James F Collins
High-iron feeding of rodents has been commonly used to model human iron-overload disorders. We recently noted that high-iron consumption impaired growth and caused severe systemic copper deficiency in growing rats, but the mechanism by which this occurred could not be determined due to technical limitations. In the current investigation, we thus utilized mice; first to determine if the same phenomenon occurred in another mammalian species, and secondly since we could assess in vivo copper absorption in mice...
June 15, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28617164/liver-transplantation-using-graft-from-a-donor-with-aplastic-anemia
#17
Soheila Milani, Mohsen Aliakbarian, Ebrahim Khaleghi
Liver transplantation (LT) is a life-saving procedure in patients with end-stage liver disease. The number of patients in the waiting list for LT has steadily increased over time, so removing liver from deceased donors with unusual disorders could possibly be used for transplantation. We describe a case of LT from a donor with "aplastic anemia" to a patient with liver failure due to hepatitis C. Aplastic anemia is a syndrome of bone marrow failure. Aplastic anemia is one of the most common conditions linked to transfusion dependence, and long-term repeated transfusion inevitably results in iron overload...
June 2017: Progress in Transplantation
https://www.readbyqxmd.com/read/28617058/the-assessment-of-skin-color-and-iron-levels-in-pediatric-patients-with-%C3%AE-thalassemia-major-using-a-visual-skin-color-chart
#18
Ibrahim H Bucak, Habip Almis, Samet Benli, Mehmet Turgut
Patients with β-thalassemia major (β-TM), a disease that emerges due to disorder of hemoglobin (Hb) synthesis, require life-long erythrocyte transfusion. The purpose of this study was to evaluate skin color and iron levels of patients with β-TM using a visual skin color chart. Each patient's skin color was matched on a skin color chart under a fluorescent lamp by the same physician on each occasion. Iron, iron binding capacity, ferritin and complete blood count (CBC) were studied for each patient enrolled...
June 15, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28616085/helicobacter-pylori-infection-old-and-new
#19
REVIEW
S Diaconu, A Predescu, A Moldoveanu, C S Pop, C Fierbințeanu-Braticevici
Helicobacter pylori is a spiral-shaped bacterium that grows in the digestive tract and may be present in more than half of the world's population. The clinical features of Helicobacter pylori range from asymptomatic gastritis to gastrointestinal malignancy. Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade B-cell marginal zone lymphoma and Helicobacter pylori has been detected in more than 75% of the patients with MALT lymphoma. Many tests for the detection of Helicobacter pylori are available, including antibody tests, urea breath tests, stool antigen tests and endoscopic biopsies...
April 2017: Journal of Medicine and Life
https://www.readbyqxmd.com/read/28615257/randomized-controlled-trial-assessing-the-efficacy-of-a-reusable-fish-shaped-iron-ingot-to-increase-hemoglobin-concentration-in-anemic-rural-cambodian-women
#20
Aviva I Rappaport, Kyly C Whitfield, Gwen E Chapman, Rickey Y Yada, Khin Meng Kheang, Jennie Louise, Alastair J Summerlee, Gavin R Armstrong, Timothy J Green
Background: Anemia affects 45% of women of childbearing age in Cambodia. Iron supplementation is recommended in populations in which anemia prevalence is high. However, there are issues of cost, distribution, and adherence. A potential alternative is a reusable fish-shaped iron ingot, which, when added to the cooking pot, leaches iron into the fluid in which it is prepared.Objective: We sought to determine whether there was a difference in hemoglobin concentrations in rural Cambodian anemic women (aged 18-49 y) who cooked with the iron ingot or consumed a daily iron supplement compared with a control after 1 y...
June 14, 2017: American Journal of Clinical Nutrition
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