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https://www.readbyqxmd.com/read/28436960/amyloid-fibril-systems-reduce-stabilize-and-deliver-bioavailable-nanosized-iron
#1
Yi Shen, Lidija Posavec, Sreenath Bolisetty, Florentine M Hilty, Gustav Nyström, Joachim Kohlbrecher, Monika Hilbe, Antonella Rossi, Jeannine Baumgartner, Michael B Zimmermann, Raffaele Mezzenga
Iron-deficiency anaemia (IDA) is a major global public health problem. A sustainable and cost-effective strategy to reduce IDA is iron fortification of foods, but the most bioavailable fortificants cause adverse organoleptic changes in foods. Iron nanoparticles are a promising solution in food matrices, although their tendency to oxidize and rapidly aggregate in solution severely limits their use in fortification. Amyloid fibrils are protein aggregates initially known for their association with neurodegenerative disorders, but recently described in the context of biological functions in living organisms and emerging as unique biomaterial building blocks...
April 24, 2017: Nature Nanotechnology
https://www.readbyqxmd.com/read/28435994/thalassemia-minor-and-major-%C3%A2-current-management
#2
REVIEW
Ved Prakash Choudhry
Thalassemia is a common genetic disorder. It has been estimated that in India nearly 5 crore people are thalassemia carriers. They are asymptomatic and are detected on blood tests. These people are at same risk of developing iron deficiency anemia as general population and need iron therapy in the presence of iron deficiency anemia. Nearly 12,000 children with thalassemia major (Homozygous state) are born every year. These children often present with significant anemia along with hepatosplenomegaly during infancy and require early diagnosis and institution of therapy with repeated blood transfusions and chelation therapy...
April 24, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28428956/physiological-functions-of-the-cellular-prion-protein
#3
REVIEW
Andrew R Castle, Andrew C Gill
The prion protein, PrP(C), is a small, cell-surface glycoprotein notable primarily for its critical role in pathogenesis of the neurodegenerative disorders known as prion diseases. A hallmark of prion diseases is the conversion of PrP(C) into an abnormally folded isoform, which provides a template for further pathogenic conversion of PrP(C), allowing disease to spread from cell to cell and, in some circumstances, to transfer to a new host. In addition to the putative neurotoxicity caused by the misfolded form(s), loss of normal PrP(C) function could be an integral part of the neurodegenerative processes and, consequently, significant research efforts have been directed toward determining the physiological functions of PrP(C)...
2017: Frontiers in Molecular Biosciences
https://www.readbyqxmd.com/read/28427446/respiratory-chain-complex-iii-deficiency-due-to-mutated-bcs1l-a-novel-phenotype-with-encephalomyopathy-partially-phenocopied-in-a-bcs1l-mutant-mouse-model
#4
Saara Tegelberg, Nikica Tomašić, Jukka Kallijärvi, Janne Purhonen, Eskil Elmér, Eva Lindberg, David Gisselsson Nord, Maria Soller, Nicole Lesko, Anna Wedell, Helene Bruhn, Christoph Freyer, Henrik Stranneheim, Rolf Wibom, Inger Nennesmo, Anna Wredenberg, Erik A Eklund, Vineta Fellman
BACKGROUND: Mitochondrial diseases due to defective respiratory chain complex III (CIII) are relatively uncommon. The assembly of the eleven-subunit CIII is completed by the insertion of the Rieske iron-sulfur protein, a process for which BCS1L protein is indispensable. Mutations in the BCS1L gene constitute the most common diagnosed cause of CIII deficiency, and the phenotypic spectrum arising from mutations in this gene is wide. RESULTS: A case of CIII deficiency was investigated in depth to assess respiratory chain function and assembly, and brain, skeletal muscle and liver histology...
April 20, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28427421/cerebrospinal-fluid-csf-biomarkers-of-iron-status-are-associated-with-csf-viral-load-antiretroviral-therapy-and-demographic-factors-in-hiv-infected-adults
#5
Stephanie M Patton, Quan Wang, Todd Hulgan, James R Connor, Peilin Jia, Zhongming Zhao, Scott L Letendre, Ronald J Ellis, William S Bush, David C Samuels, Donald R Franklin, Harpreet Kaur, Jennifer Iudicello, Igor Grant, Asha R Kallianpur
BACKGROUND: HIV-associated neurocognitive disorder (HAND) remains common, despite antiretroviral therapy (ART). HIV dysregulates iron metabolism, but cerebrospinal fluid (CSF) levels of iron and iron-transport proteins in HIV-infected (HIV+) persons are largely unknown. The objectives of this study were to characterize CSF iron-related biomarkers in HIV+ adults and explore their relationships to known predictors of HAND. METHODS: We quantified total iron, transferrin and heavy-chain (H)-ferritin by immunoassay in CSF sampled by lumbar puncture in 403 HIV+ participants in a multi-center, observational study and evaluated biomarker associations with demographic and HIV-related correlates of HAND [e...
April 21, 2017: Fluids and Barriers of the CNS
https://www.readbyqxmd.com/read/28426710/change-in-iron-metabolism-in-rats-after-renal-ischemia-reperfusion-injury
#6
Guang-Liang Xie, Lin Zhu, Yan-Min Zhang, Qian-Nan Zhang, Qing Yu
Previous studies have indicated that hepcidin, which can regulate iron efflux by binding to ferroportin-1 (FPN1) and inducing its internalization and degradation, acts as the critical factor in the regulation of iron metabolism. However, it is unknown whether hepcidin is involved in acute renal ischemia/reperfusion injury (IRI). In this study, an IRI rat model was established via right renal excision and blood interruption for 45 min in the left kidney, and iron metabolism indexes were examined to investigate the change in iron metabolism and to analyze the role of hepcidin during IRI...
2017: PloS One
https://www.readbyqxmd.com/read/28424751/levodopa-responsive-parkinsonism-in-patients-with-hemochromatosis-case-presentation-and-literature-review
#7
Tarun Girotra, Abhimanyu Mahajan, Christos Sidiropoulos
Hemochromatosis is an autosomal recessive disorder which leads to abnormal iron deposition in the parenchyma of multiple organs causing tissue damage. Accumulation of iron in the brain has been postulated to be associated with several neurodegenerative diseases including Parkinson's disease. The excess iron promotes Parkin and α-synuclein aggregation in the neurons. Excess iron has also been noted in substantia nigra on MRI especially using susceptibility weighted imaging in patients with Parkinson's disease...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28424608/blood-transcriptomic-meta-analysis-identifies-dysregulation-of-hemoglobin-and-iron-metabolism-in-parkinson-disease
#8
Jose A Santiago, Judith A Potashkin
Disrupted iron metabolism has been implicated in the pathogenesis of Parkinson's disease (PD), a progressive neurodegenerative disorder that severely affects movement and coordination, yet the molecular mechanisms underlying this association remain unknown. To this end, we performed a transcriptomic meta-analysis of four blood microarrays in PD. We observed a significant downregulation of genes related to hemoglobin including, hemoglobin delta (HBD), alpha hemoglobin stabilizing protein (ASHP), genes implicated in iron metabolism including, solute carrier family 11 member 2 (SLC11A2), ferrochelatase (FECH), and erythrocyte-specific genes including erythrocyte membrane protein (EPB42), and 5'-aminolevulinate synthase 2 (ALAS2)...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28421266/reactive-oxygen-species-mediated-t-lymphocyte-abnormalities-in-an-iron-overloaded-mouse-model-and-iron-overloaded-patients-with-myelodysplastic-syndromes
#9
Jie Chen, Wen-Yi Lu, Ming-Feng Zhao, Xiao-Li Cao, Yan-Yu Jiang, Xin Jin, Ping Xu, Ting-Ting Yuan, Yu-Chen Zhang, Xiao Chai, Juan-Xia Meng, Qing Li, Xia Xiao, Juan Mu, De-Guan Li, Ai-Ping Qi
The adverse effects of iron overload have raised more concerns as a growing number of studies reported its association with immune disorders. This study aimed to investigate alterations in the immune system by iron overload in patients with myelodysplastic syndrome (MDS) and an iron-overloaded mouse model. The peripheral blood from patients was harvested to test the effect of iron overload on the subsets of T lymphocytes, and the level of reactive oxygen species (ROS) was also evaluated. The data showed that iron-overloaded patients had a lower percentage of CD3(+) T cells and disrupted T cell subsets, concomitant with higher ROS level in lymphocytes...
April 18, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28421060/the-heme-metabolite-carbon-monoxide-facilitates-kshv-infection-by-inhibiting-tlr4-signaling-in-endothelial-cells
#10
Sara Botto, Jean K Gustin, Ashlee V Moses
Kaposi sarcoma herpesvirus (KSHV) is the etiologic agent of Kaposi sarcoma (KS) and certain rare B cell lymphoproliferative disorders. KSHV infection of endothelial cells (EC) in vitro increases expression of the inducible host-encoded enzyme heme oxygenase-1 (HO-1), which is also strongly expressed in KS tumors. HO-1 catalyzes the rate-limiting step in the conversion of heme into iron, biliverdin and the gasotransmitter carbon monoxide (CO), all of which share anti-apoptotic, anti-inflammatory, pro-survival, and tumorigenic activities...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28420988/heme-as-a-target-for-therapeutic-interventions
#11
REVIEW
Stephan Immenschuh, Vijith Vijayan, Sabina Janciauskiene, Faikah Gueler
Heme is a complex of iron and the tetrapyrrole protoporphyrin IX with essential functions in aerobic organisms. Heme is the prosthetic group of hemoproteins such as hemoglobin and myoglobin, which are crucial for reversible oxygen binding and transport. By contrast, high levels of free heme, which may occur in various pathophysiological conditions, are toxic via pro-oxidant, pro-inflammatory and cytotoxic effects. The toxicity of heme plays a major role for the pathogenesis of prototypical hemolytic disorders including sickle cell disease and malaria...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28417130/iron-isotopic-composition-of-blood-serum-in-anemia-of-chronic-kidney-disease
#12
Yulia Anoshkina, Marta Costas-Rodríguez, Marijn Speeckaert, Wim Van Biesen, Joris Delanghe, Frank Vanhaecke
Chronic kidney disease (CKD) is a general term for disorders that affect the structure and function of the kidneys. Iron deficiency (ID) and anemia occur in the vast majority of CKD patients, most of whom are elderly. However, establishing the cause of anemia in CKD, and therefore making an informed decision concerning the corresponding therapeutic treatment, is still a challenge. High-precision Fe isotopic analysis of blood serum samples of CKD patients with and without ID/anemia was performed via multi-collector inductively coupled plasma-mass spectrometry (MC-ICP-MS) for such a purpose...
April 18, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/28406842/hfe-gene-mutations-and-iron-status-in-100-healthy-polish-children
#13
Barbara Kaczorowska-Hac, Marcin Luszczyk, Jedrzej Antosiewicz, Wieslaw Ziolkowski, Elzbieta Adamkiewicz-Drozynska, Malgorzata Mysliwiec, Ewa Milosz, Jan J Kaczor
Iron participates in oxygen transport, energetic, metabolic, and immunologic processes. There are 2 main causes of iron overload: hereditary hemochromatosis which is a primary cause, is a metabolic disorder caused by mutations of genes that control iron metabolism and secondary hemochromatosis caused by multitransfusions, chronic hemolysis, and intake of iron rich food. The most common type of hereditary hemochromatosis is caused by HFE gene mutation. In this study, we analyzed iron metabolism in 100 healthy Polish children in relation to their HFE gene status...
April 12, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28401645/body-composition-and-micronutrient-deficiencies-in-patients-with-an-acute-exacerbation-of-chronic-obstructive-pulmonary-disease
#14
Charith Horadagoda, Timothy Dinihan, Mary Roberts, Kristina Kairaitis
Chronic obstructive pulmonary disease (COPD) is a multisystem disorder. Abnormal body composition (BC) and low serum micronutrient levels contribute significantly to morbidity associated with acute exacerbations of COPD (AECOPD). The BC and serum levels of selected micronutrients were recorded in prospective, consecutive patients admitted to hospital with an AECOPD in Western Sydney. 94 patients were enrolled, 43% female, the average age was 69.8 ± 8.2years(SD). Admission spirometry revealed a mean spirometric ratio of 0...
April 12, 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28400547/anemia-in-thyroid-diseases
#15
Ewelina Szczepanek-Parulska, Aleksandra Hernik, Marek Ruchała
Anemia is a frequent, although often underestimated, clinical condition accompanying thyroid diseases. In spite of the fact that anemia and thyroid dysfunction often occur simultaneously, the causative relationship between these two disorders remains ambiguous. Thyroid hormones stimulate erythrocytes precursors proliferation directly, as well as via erythropoietin production enhancement, whereas iron-deficient anemia negatively influences thyroid hormonal status. Thus, different forms of anemia might emerge in the course of thyroid dysfunction...
March 28, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/28399703/value-of-speckle-tracking-echocardiography-for-detection-of-clinically-silent-left-ventricular-dysfunction-in-patients-with-%C3%AE-thalassemia
#16
Mozhgan Parsaee, Sedigheh Saedi, Pegah Joghataei, Azita Azarkeivan, Zahra Alizadeh Sani
OBJECTIVE: β-Thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia requiring chronic transfusion therapy. Cardiac involvement is the main cause of death in patients with thalassemia major. The narrow border is between overt myocardial dysfunction and clinically silent left ventricular (LV) dysfunction in patients with thalassemia. Therefore, we need novel parameters in different imaging techniques to discover cardiac involvement in an early and subtle stage...
April 12, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28393990/-restless-legs-syndrome-long-term-follow-up-of-a-series-of-patients
#17
Marcelo Miranda C, Lorena Hudson A
BACKGROUND: Restless legs syndrome (RLS) affects 10% of the general population. AIM: To analyze a series of patients with a minimum follow-up period of four years, treated during an interval of 14 years. MATERIAL AND METHODS: Retrospective analysis of medical records of 200 patients assessed and followed by the authors at a private outpatient clinic. RESULTS: Fifty patients aged 25 to 90 years (34 females), who had a mean follow-up of 6,3 years (range 4-14 years), were selected...
December 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/28389699/ion-channelopathies-and-migraine-pathogenesis
#18
REVIEW
Cassie L Albury, Shani Stuart, Larisa M Haupt, Lyn R Griffiths
Migraine is a common neurological disorder that affects approximately 12-20% of the general adult population. Migraine pathogenesis is complex and not wholly understood. Molecular genetic investigations, imaging and biochemical studies, have unveiled a number of interconnected neurological pathways which seem to have a cause and effect component integral to its cause. Much weight of migraine attack initiation can be placed on the initial trigger and the pathways involved in its neuronal counter reaction. Ion channels play a large role in the generation, portrayal and mitigation of the brains response to external triggers...
April 7, 2017: Molecular Genetics and Genomics: MGG
https://www.readbyqxmd.com/read/28385785/imatinib-and-spironolactone-suppress-hepcidin-expression
#19
Katarzyna Mleczko-Sanecka, Ana Rita da Silva, Debora Call, Joana Neves, Nikolai Schmeer, Georg Damm, Daniel Seehofer, Martina U Muckenthaler
Disorders of iron metabolism are largely attributed to an excessive or insufficient expression of hepcidin, the master regulator of systemic iron homeostasis. Here, we investigated whether drugs targeting genetic regulators of hepcidin can affect iron homeostasis. We focused our efforts on drugs approved for clinical use to enable repositioning strategies and/or to reveal iron-related side effects of widely prescribed therapeutics. To identify hepcidin-modulating therapeutics, we reevaluated data generated by a genome-wide RNAi screen for hepcidin regulators...
April 6, 2017: Haematologica
https://www.readbyqxmd.com/read/28382129/labyrinthectomy-and-vestibular-neurectomy-for-intractable-vertiginous-symptoms
#20
Alfredo Vega Alarcón, Lourdes Olivia Vales Hidalgo, Rodrigo Jácome Arévalo, Marite Palma Diaz
Introduction Labyrinthectomy and vestibular neurectomy are considered the surgical procedures with the highest possibility of controlling medically untreatable incapacitating vertigo. Ironically, after 100 years of the introduction of both transmastoid labyrinthectomy and vestibular neurectomy, the choice of which procedure to use rests primarily on the evaluation of the hearing and of the surgical morbidity. Objective To review surgical labyrinthectomy and vestibular neurectomy for the treatment of incapacitating vestibular disorders...
April 2017: International Archives of Otorhinolaryngology
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