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https://www.readbyqxmd.com/read/27928480/a-review-on-iron-chelators-in-treatment-of-iron-overload-syndromes
#1
REVIEW
Naser Mobarra, Mehrnoosh Shanaki, Hassan Ehteram, Hajar Nasiri, Mehdi Sahmani, Mohsen Saeidi, Mehdi Goudarzi, Hoda Pourkarim, Mehdi Azad
Iron chelation therapy is used to reduce iron overload development due to its deposition in various organs such as liver and heart after regular transfusion. In this review, different iron chelators implicated in treatment of iron overload in various clinical conditions have been evaluated using more up-to-date studies focusing on these therapeutic agents. Deferoxamine, Deferiprone and Deferasirox are the most important specific US FDA-approved iron chelators. Each of these chelators has their own advantages and disadvantages, various target diseases, levels of deposited iron and clinical symptoms of the afflicted patients which may affect their selection as the best modality...
October 1, 2016: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/27928477/health-related-quality-of-life-and-health-utility-values-in-beta-thalassemia-major-patients-receiving-different-types-of-iron-chelators-in-iran
#2
Meysam Seyedifar, Farid Abedin Dorkoosh, Amir Ali Hamidieh, Majid Naderi, Hossein Karami, Mehran Karimi, Masoomeh Fadaiyrayeny, Masoumeh Musavi, Sanaz Safaei, Mohammad Mahdi Ahmadian-Attari, Molouk Hadjibabaie, Abdol Majid Cheraghali, Ali Akbari Sari
Background: Thalassemia is a chronic, inherited blood disorder, which in its most severe form, causes life-threatening anemia. Thalassemia patients not only engage with difficulties of blood transfusion and iron chelating therapy but also have some social challenges and health threatening factors. There are some reports on quality of life in thalassemia patients around the world from southeast of Asia to Italy in Europe and United States. In this study, we tried to evaluate and compare Health Related Quality of life (HRQoL) and the health utility in beta thalassemia major patients receiving different types of iron chelators and living in different socio-economical situations...
October 1, 2016: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/27927768/iron-heme-bach1-axis-is-involved-in-erythroblast-adaptation-to-iron-deficiency
#3
Masahiro Kobayashi, Hiroki Kato, Hiroshi Hada, Ari Itoh-Nakadai, Tohru Fujiwara, Akihiko Muto, Yukihiro Inoguchi, Kenji Ichiyanagi, Wataru Houjou, Naohisa Tomosugi, Hiroyuki Sasaki, Hideo Harigae, Kazuhiko Igarashi
Iron plays the central role in the oxygen transport by the erythrocyte as a constituent of heme and hemoglobin. The importance of iron and heme also resides in their regulatory roles during erythroblast maturation. The transcription factor Bach1 may be involved in their regulatory roles since it is inactivated by direct binding of heme. To address whether Bach1 is involved in the responses of erythroblasts to iron status, low iron conditions that induced severe iron deficiency in mice were established. Under iron deficiency, extensive gene expression changes and mitophagy disorder were induced during maturation of erythroblasts...
December 7, 2016: Haematologica
https://www.readbyqxmd.com/read/27920908/crystal-structure-of-tris-4-4-bi-pyridine-diium-bis-1-1-3-3-tetra-cyano-2-eth-oxy-propenide-trihydrate
#4
Fatima Setifi, Arto Valkonen, Zouaoui Setifi, Sami Nummelin, Rachid Touzani, Christopher Glidewell
The title hydrated salt, C30H26N6(2+)·2C9H5N4O(-)·3H2O, was obtained as an unexpected product from the hydro-thermal reaction between potassium 1,1,3,3-tetra-cyano-2-eth-oxy-propenide, 4,4'-bi-pyridine and iron(II) sulfate hepta-hydrate. The cation lies across a twofold rotation axis in the space group I2/a with the other components all in general positions. In the cation, the H atom linking the pyridine units is disordered over two adjacent sites having occupancies of 0.66 (4) and 0.36 (4), i.e. as N-H⋯N and N⋯H-N...
September 1, 2016: Acta Crystallographica. Section E, Crystallographic Communications
https://www.readbyqxmd.com/read/27920532/the-effect-of-magnetic-nanoparticles-on-neuronal-differentiation-of-induced-pluripotent-stem-cell-derived-neural-precursors
#5
Klára Jiráková, Monika Šeneklová, Daniel Jirák, Karolína Turnovcová, Magda Vosmanská, Michal Babič, Daniel Horák, Pavel Veverka, Pavla Jendelová
INTRODUCTION: Magnetic resonance (MR) imaging is suitable for noninvasive long-term tracking. We labeled human induced pluripotent stem cell-derived neural precursors (iPSC-NPs) with two types of iron-based nanoparticles, silica-coated cobalt zinc ferrite nanoparticles (CZF) and poly-l-lysine-coated iron oxide superparamagnetic nanoparticles (PLL-coated γ-Fe2O3) and studied their effect on proliferation and neuronal differentiation. MATERIALS AND METHODS: We investigated the effect of these two contrast agents on neural precursor cell proliferation and differentiation capability...
2016: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/27918350/a-case-of-idiopathic-pulmonary-hemosiderosis-presenting-with-signs-and-symptoms-mimicking-hemolytic-anemia
#6
Dilek Doğruel, Ayşe Erbay, Nalan Yazici, Alev Arslan, Bermal Hasbay Biçen
Idiopathic pulmonary hemosiderosis is primarily a disorder of childhood, which is characterized by hemoptysis, iron deficiency anemia, and diffuse parenchymal infiltrates on chest x-ray secondary to recurrent attacks of alveolar hemorrhage. It can be diagnosed by showing hemosiderin laden macrophages in bronchoalveolar lavage fluid after other specific causes of diffuse alveolar hemorrhage are definitely excluded. A 5-year-old male patient was admitted to our clinic with sudden-onset pallor during iron therapy given for anemia...
December 1, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27908410/elevated-serum-mda-and-depleted-non-enzymatic-antioxidants-macro-minerals-and-trace-elements-are-associated-with-bipolar-disorder
#7
Manjurul Islam Chowdhury, Maimuna Hasan, Mohammad Safiqul Islam, Md Shahid Sarwar, Mohammad Nurul Amin, S M Naim Uddin, Md Zahedur Rahaman, Sujan Banik, Md Saddam Hussain, Kazushige Yokota, Abul Hasnat
Genetic and neurobiological factors are considered to be the major causes of mood and mental disorders. However, over the past few years, increased levels of serum malondialdehyde and altered levels of various non-enzymatic antioxidants and essential minerals involved in abnormal functional activity have been identified as major contributing factors to the pathogenesis of several neurological disorders. The aim of this study was to determine the levels of the serum lipid peroxidation product malondialdehyde (MDA), antioxidants (vitamin A, E and C), macro-minerals (calcium, potassium and sodium) and trace elements (zinc, iron and selenium) in patients with bipolar disorder and to explore their role in disease progression...
January 2017: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/27905971/trends-causes-and-risk-factors-of-mortality-among-children-under-5-in-ethiopia-1990-2013-findings-from-the-global-burden-of-disease-study-2013
#8
Amare Deribew, Gizachew Assefa Tessema, Kebede Deribe, Yohannes Adama Melaku, Yihunie Lakew, Azmeraw T Amare, Semaw F Abera, Mesoud Mohammed, Abiy Hiruye, Efrem Teklay, Awoke Misganaw, Nicholas Kassebaum
BACKGROUND: Ethiopia has made remarkable progress in reducing child mortality over the last two decades. However, the under-5 mortality rate in Ethiopia is still higher than the under-5 mortality rates of several low- and middle-income countries (LMIC). On the other hand, the patterns and causes of child mortality have not been well investigated in Ethiopia. The objective of this study was to investigate the mortality trend, causes of death, and risk factors among children under 5 in Ethiopia during 1990-2013...
November 14, 2016: Population Health Metrics
https://www.readbyqxmd.com/read/27900772/hematopoietic-stem-cell-transplantation-for-people-with-%C3%A3-thalassaemia-major
#9
REVIEW
Vanitha A Jagannath, Zbys Fedorowicz, Amani Al Hajeri, Akshay Sharma
BACKGROUND: Thalassemia is an inherited autosomal recessive blood disorder, caused by mutations in globin genes or their regulatory regions. This results in a reduced rate of synthesis of one of the globin chains that make up haemoglobin. In ß-thalassaemia major there is an underproduction of ß-globin chains combined with excess of free α-globin chains. The excess free α-globin chains precipitate in red blood cells, leading to their destruction (haemolysis) and ineffective erythropoiesis...
November 30, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27896572/identification-of-novel-mutations-in-hfe-hfe2-tfr2-and-slc40a1-genes-in-chinese-patients-affected-by-hereditary-hemochromatosis
#10
Yongwei Wang, Yali Du, Gang Liu, Shanshan Guo, Bo Hou, Xianyong Jiang, Bing Han, Yanzhong Chang, Guangjun Nie
Hereditary hemochromatosis (HH) is a group of inherited iron-overload disorders associated with pathogenic defects in the genes encoding hemochromatosis (HFE), hemojuvelin (HJV/HFE2), hepcidin (HAMP), transferrin receptor 2 (TfR2), and ferroportin (FPN1/SLC40A1) proteins, and the clinical features are well described. However, there have been only a few detailed reports of HH in Chinese populations. Thus, there is insufficient patient information for population-based analyses in Chinese populations or comparative studies among different ethical groups...
November 28, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27895843/severe-anemia-in-malawian-children
#11
Job Cj Calis, Kamija S Phiri, E Brian Faragher, Bernard J Brabin, Imelda Bates, Luis E Cuevas, Rob J de Haan, Ajib I Phiri, Pelani Malange, Mirriam Khoka, Paul Jm Hulshof, Lisette van Lieshout, Marcel Ghm Beld, Yik Y Teo, Kirk A Rockett, Anna Richardson, Dominic P Kwiatkowski, Malcolm E Molyneux, Michaël Boele van Hensbroek
BACKGROUND: Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. METHODS: We conducted a case-control study of 381 preschool children with severe anemia (hemoglobin concentration, <5.0 g per deciliter) and 757 preschool children without severe anemia in urban and rural settings in Malawi. Causal factors previously associated with severe anemia were studied. The data were examined by multivariate analysis and structural equation modeling...
September 2016: Malawi Medical Journal: the Journal of Medical Association of Malawi
https://www.readbyqxmd.com/read/27895383/preparation-and-antioxidant-pro-oxidant-activities-of-3-monosubstituted-5-hydroxyoxindole-derivatives
#12
Daisuke Yasuda, Kyoko Takahashi, Tomoyuki Ohe, Shigeo Nakamura, Tadahiko Mashino
Antioxidant treatments have been expected to be a novel therapeutics for various oxidative stress-mediated disorders. Our previous study revealed that 5-hydroxyoxindole and its 3-phenacyl-3-hydroxy derivatives showed excellent antioxidant activities such as 1,1-diphenyl-2-picrylhydrazyl (DPPH) radical scavenging activity and lipid-peroxidation inhibitory activity. However, the DPPH radical scavenging activity of the 3,3-disubstituted derivatives was lower than that of the original 5-hydroxyoxindole. In the present study, we synthesized novel 3-monosubstituted 5-hydroxyoxindole derivatives that exhibited stronger DPPH radical scavenging activities and lipid peroxidation-inhibitory activities than the 3,3-disubstituted 5-hydroxyoxindoles...
November 2016: Journal of Clinical Biochemistry and Nutrition
https://www.readbyqxmd.com/read/27893170/safety-profiles-of-iron-chelators-in-young-patients-with-haemoglobinopathies
#13
REVIEW
Sebastian Botzenhardt, Niya Li, Esther W Chan, Chor Wing Sing, Ian C K Wong, Antje Neubert
BACKGROUND: This review describes the safety of deferoxamine (DFO), deferiprone (DFP), deferasirox (DFX), and combined therapy in young patients less than 25 years of age with haemoglobinopathies. METHOD: Searches in electronic literature databases were performed. Studies reporting adverse events associated with iron chelation therapy were included. Study and reporting quality was assessed using AHRQ Risk of Bias Assessment Tool and McMaster Quality Assessment Scale of Harms...
November 28, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27892944/veno-occlusive-disease-sinusoidal-obstruction-syndrome-after-haematopoietic-stem-cell-transplantation-middle-east-north-africa-regional-consensus-on-prevention-diagnosis-and-management
#14
A H Al Jefri, H Abujazar, A Al-Ahmari, A Al Rawas, Z Al Zahrani, A Alhejazi, M A Bekadja, A Ibrahim, M Lahoucine, S Ousia, A Bazarbachi
Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) of the liver is a serious, early complication of haematopoietic stem cell transplantation (HSCT), severe and very severe forms of which are associated with a high mortality rate. A wide variety of patient, disease and treatment-related risk factors for VOD/SOS have been identified. Several bodies have published recommendations for the diagnosis, prevention and management of VOD/SOS following HSCT. A group of regional experts have developed a consensus statement on the diagnosis, prevention and management of VOD/SOS in the Middle East and North Africa region to help in the management of HSCT patients in the region...
November 28, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27892483/down-regulation-of-coasy-the-gene-associated-with-nbia-vi-reduces-bmp-signaling-perturbs-dorso-ventral-patterning-and-alters-neuronal-development-in-zebrafish
#15
Deepak Khatri, Daniela Zizioli, Natascia Tiso, Nicola Facchinello, Sara Vezzoli, Alessandra Gianoncelli, Maurizio Memo, Eugenio Monti, Giuseppe Borsani, Dario Finazzi
Mutations in Pantothenate kinase 2 and Coenzyme A (CoA) synthase (COASY), genes involved in CoA biosynthesis, are associated with rare neurodegenerative disorders with brain iron accumulation. We showed that zebrafish pank2 gene plays an essential role in brain and vasculature development. Now we extended our study to coasy. The gene has high level of sequence identity with the human ortholog and is ubiquitously expressed from the earliest stages of development. The abrogation of its expression led to strong reduction of CoA content, high lethality and a phenotype resembling to that of dorsalized mutants...
November 28, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27891065/trends-causes-and-risk-factors-of-mortality-among-children-under-5-in-ethiopia-1990-2013-findings-from-the-global-burden-of-disease-study-2013
#16
Amare Deribew, Gizachew Assefa Tessema, Kebede Deribe, Yohannes Adama Melaku, Yihunie Lakew, Azmeraw T Amare, Semaw F Abera, Mesoud Mohammed, Abiy Hiruye, Efrem Teklay, Awoke Misganaw, Nicholas Kassebaum
BACKGROUND: Ethiopia has made remarkable progress in reducing child mortality over the last two decades. However, the under-5 mortality rate in Ethiopia is still higher than the under-5 mortality rates of several low- and middle-income countries (LMIC). On the other hand, the patterns and causes of child mortality have not been well investigated in Ethiopia. The objective of this study was to investigate the mortality trend, causes of death, and risk factors among children under 5 in Ethiopia during 1990-2013...
2016: Population Health Metrics
https://www.readbyqxmd.com/read/27890316/is-there-a-dependence-between-children-s-body-weight-and-the-concentration-of-metals-in-deciduous-teeth
#17
Agnieszka Fischer, Danuta Wiechuła
Malnutrition, manifested by both overweight and underweight, can lead to serious health consequences. The subject of the study was to determine the concentration of elements such as chromium (Cr), calcium (Ca), copper (Cu), iron (Fe) and manganese (Mn) in children's deciduous teeth in relation to their body weight. The calculated body mass index (BMI) values and an application of the growth chart showed that 59% of children among the studied sample had normal weight. In 41% of children, weight disorders were observed including underweight - 28% and overweight - 12%...
September 19, 2016: Homo: Internationale Zeitschrift Für die Vergleichende Forschung Am Menschen
https://www.readbyqxmd.com/read/27871472/an-immunohistochemical-study-of-placental-syncytiotrophoblasts-in-neonatal-hemochromatosis
#18
Aiko Shimono, Yuko Imoto, Haruhiko Sakamoto, Yoichi Chiba, Koichi Matsumoto, Machi Kawauchi, Takashi Kusaka, Hirokazu Tanaka, Toshiyuki Hata, Yoshio Kushida, Masaki Ueno
INTRODUCTION: Neonatal hemochromatosis (NH) is a rare neonatal disorder that results in liver cirrhosis with hemosiderin deposition in the liver and other organs, similarly to hereditary hemochromatosis. Excess iron is transferred from the mother to fetus through the placenta in NH. We examined the expression of iron metabolism-related substances in placental syncytiotrophoblasts (STB) by immunostaining to clarify how the transfer of iron through STB increases in NH. METHODS: Immunostaining was performed using formalin-fixed, paraffin-embedded sections of placentae from three NH cases, four gestational age-matched controls, and, depending on the antibody examined, five to seven full-term controls...
December 2016: Placenta
https://www.readbyqxmd.com/read/27866575/anemia-for-the-primary-care-physician
#19
REVIEW
Darryl J Powell, Maureen Okam Achebe
Anemia denotes a reduced red blood cell (RBC) mass from any cause. The causes of anemia are numerous and due to decreased (or abnormal) erythropoesis, shortened RBC life span, or blood loss. The most common etiology of anemia is iron deficiency. A judicious work up of anemia includes evaluating the reticulocyte count and peripheral smear. The severity of illness of a patient with anemia is determined by the degree of anemia and the seriousness of the underlying disorder. Management of patients with hereditary and hemolytic anemias should involve a hematologist...
December 2016: Primary Care
https://www.readbyqxmd.com/read/27859814/black-rhinoceros-diceros-bicornis-and-domestic-horse-equus-caballus-hindgut-microflora-demonstrate-similar-fermentation-responses-to-grape-seed-extract-supplementation-in%C3%A2-vitro
#20
N F Huntley, H D Naumann, A L Kenny, M S Kerley
The domestic horse is used as a nutritional model for rhinoceros maintained under human care. The validity of this model for browsing rhinoceros has been questioned due to high prevalence of iron overload disorder (IOD) in captive black rhinoceros (Diceros bicornis), which is associated with high morbidity and mortality. Iron chelators, such as tannins, are under investigation as dietary supplements to ameliorate or prevent IOD in prone species. Polyphenolic compounds variably affect microbial fermentation, so the first objective of this experiment was to evaluate the effects of grape seed extract (GSE; a concentrated source of condensed tannins; CT) on black rhinoceros hindgut fermentation...
November 13, 2016: Journal of Animal Physiology and Animal Nutrition
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