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https://www.readbyqxmd.com/read/28221264/efficacy-and-safety-of-combined-oral-chelation-with-deferiprone-and-deferasirox-in-children-with-%C3%AE-thalassemia-major-an-experience-from-north-india
#1
Nupur Parakh, Jagdish Chandra, Sunita Sharma, Bhavna Dhingra, Rajesh Jain, DeoNath Mahto
OBJECTIVE: A combination of desferrioxamine with either deferiprone (DFP) or deferasirox (DFX) for patients with β-thalassemia major who do not achieve negative iron balance with monotherapy has been studied widely. However, poor compliance resulting from the need for parentral administration of desferrioxamine and its cost necessicitates combining 2 oral chelators. METHODS: A prospective study was conducted in patients with transfusion-dependent β-thalassemia major in a tertiary care center over 2 years...
February 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28218487/radiolabelled-polymeric-materials-for-imaging-and-treatment-of-cancer-quo-vadis
#2
REVIEW
Kritee Pant, Ondřej Sedláček, Robin A Nadar, Martin Hrubý, Holger Stephan
Owing to their tunable blood circulation time and suitable plasma stability, polymer-based nanomaterials hold a great potential for designing and utilising multifunctional nanocarriers for efficient imaging and effective treatment of cancer. When tagged with appropriate radionuclides, they may allow for specific detection (diagnosis) as well as the destruction of tumours (therapy) or even customization of materials, aiming to both diagnosis and therapy (theranostic approach). This review provides an overview of recent developments of radiolabelled polymeric nanomaterials (natural and synthetic polymers) for molecular imaging of cancer, specifically, applying nuclear techniques such as positron emission tomography (PET) and single-photon emission computed tomography (SPECT)...
February 20, 2017: Advanced Healthcare Materials
https://www.readbyqxmd.com/read/28218005/evaluation-of-cardiac-and-hepatic-iron-overload-in-thalassemia-major-patients-with-t2-magnetic-resonance-imaging
#3
Pustika Amalia Wahidiyat, Felix Liauw, Damayanti Sekarsari, Siti Ayu Putriasih, Vasili Berdoukas, Dudley J Pennell
OBJECTIVES: Recent advancements have promoted the use of T2* magnetic resonance imaging (MRI) in the non-invasive detection of iron overload in various organs for thalassemia major patients. This study aims to determine the iron load in the heart and liver of patients with thalassemia major using T2* MRI and to evaluate its correlation with serum ferritin level and iron chelation therapy. METHODS: This cross-sectional study included 162 subjects diagnosed with thalassemia major, who were classified into acceptable, mild, moderate, or severe cardiac and hepatic iron overload following their T2* MRI results, respectively, and these were correlated to their serum ferritin levels and iron chelation therapy...
February 20, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28217776/iron-acquisition-in-fungal-pathogens-of-humans
#4
Gaurav Bairwa, Won Hee Jung, James W Kronstad
The devastating infections that fungal pathogens cause in humans are underappreciated relative to viral, bacterial and parasitic diseases. In recent years, the contributions to virulence of reductive iron uptake, siderophore-mediated uptake and heme acquisition have been identified in the best studied and most life-threatening fungal pathogens: Candida albicans, Cryptococcus neoformans and Aspergillus fumigatus. In particular, exciting new work illustrates the importance of iron acquisition from heme and hemoglobin in the virulence of pathogenic yeasts...
February 20, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/28215151/iron-chelators-dictate-immune-cells-inflammatory-ability-potential-adjuvant-therapy-for-ibd
#5
Marcello Chieppa, Vanessa Galleggiante, Grazia Serino, Monica Massaro, Angelo Santino
BACKGROUND: The importance of hemoproteins for life lies largely in their iron-mediated chemical properties. In the human body, there are about 4 g of iron, a precious resource preserved by sophisticated recycling mechanisms. Iron is also important for pathogen growth, so it is not surprising that immune cells developed mechanisms to reduce iron availability in cases of inflammation. In healthy conditions, macrophages degrade hemoproteins and export iron, while if inflammation develops, they retain cytoplasmic iron to reduce extracellular iron concentrations...
February 15, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28212618/clinical-features-and-outcome-in-patients-with-osseomuscular-type-of-wilson-s-disease
#6
Hao Yu, Juan-Juan Xie, Yu-Chao Chen, Qin-Yun Dong, Yi Dong, Wang Ni, Zhi-Ying Wu
BACKGROUND: Wilson's disease with osseomuscular type is a rare condition, which often lacks typical hepatic and neurological symptoms and causes misdiagnoses easily. During the past 10 years, eight Chinese patients of osseomuscular type of Wilson's disease were identified in our clinic. METHODS: Clinical information was gathered from medical records and follow-ups. The genetic testing was performed in each patient. Serum ceruloplasmin, Kayser-Fleischer rings, liver function, brain magnetic resonance imaging and abdominal ultrasonography were also evaluated...
February 17, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28211259/liver-transplantation-from-a-deceased-donor-with-%C3%AE-thalassemia-intermedia-is-not-contraindicated-a-case-report
#7
Ersin Gumus, Osman Abbasoglu, Cahit Tanyel, Fatma Gumruk, Hasan Ozen, Aysel Yuce
The use of extended criteria donors who might have previously been deemed unsuitable is an option to increase the organ supply for transplantation. This report presents a pediatric case of a successful liver transplantation from a donor with β-thalassemia intermedia. A patient, 6-year-old female, with a diagnosis of cryptogenic liver cirrhosis underwent deceased donor liver transplantation from a thalassemic donor. Extreme hyperferritinemia was detected shortly after transplantation. The most probable cause of hyperferritinemia was iron overload secondary to transplantation of a hemosiderotic liver...
February 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28209778/role-for-dithiolopyrrolones-in-disrupting-bacterial-metal-homeostasis
#8
Andrew N Chan, Anthony L Shiver, Walter J Wever, Sayyeda Zeenat A Razvi, Matthew F Traxler, Bo Li
Natural products harbor unique and complex structures that provide valuable antibiotic scaffolds. With an increase in antibiotic resistance, natural products once again hold promise for new antimicrobial therapies, especially those with unique scaffolds that have been overlooked due to a lack of understanding of how they function. Dithiolopyrrolones (DTPs) are an underexplored class of disulfide-containing natural products, which exhibit potent antimicrobial activities against multidrug-resistant pathogens...
February 16, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28205399/synthesis-characterization-and-relaxation-studies-of-gd-do3a-conjugate-of-chlorambucil-as-a-potential-theranostic-agent
#9
Jasleen Kaur, Yoanna Tsvetkova, Karim Arroub, Sabri Sahnoun, Fabian Kiessling, Sanjay Mathur
DO3A-based macrocycles serve as attractive templates from which clinically useful theranostic agents can be obtained after coupling with molecular targeted therapeutic drugs. In this study, we describe the chemical synthesis, relaxation, and cytotoxicity studies of a new DO3A conjugate of chlorambucil (CHL) as a magnetic resonance imaging (MRI) theranostic agent. A convenient route of synthesis is reported, which allowed conjugation of the macrocyclic ligand (DO3A) to the chemotherapeutic drug (CHL) via tyrosine for the preparation of an attractive chelate-drug ensemble (DO3A-TR-CHL)...
February 2017: Chemical Biology & Drug Design
https://www.readbyqxmd.com/read/28203323/the-effects-of-nutrition-exercise-and-a-praying-program-on-reducing-iron-overload-in-patients-with-beta-thalassemia-major-a-randomized-clinical-trial
#10
Zahra Molazem, Roghaye Noormohammadi, Roya Dokouhaki, Maryam Zakerinia, Zahra Bagheri
BACKGROUND: Excessive iron accumulation in the visceral organs creates problems for patients with beta-thalassemia major. Despite chelation therapy, mortality rate from the complications of this disease is still quite high. OBJECTIVES: This study aimed to investigate the effectiveness of nutrition, exercise, and a praying program at reducing iron overload in patients with beta-thalassemia major. PATIENTS AND METHODS: This randomized clinical trial assessed the effect of the designed care program on iron overload...
October 2016: Iranian Journal of Pediatrics
https://www.readbyqxmd.com/read/28189044/preparation-and-evaluation-of-aptes-peg-coated-iron-oxide-nanoparticles-conjugated-to-rhenium-188-labeled-rituximab
#11
Bakhtiar Azadbakht, Hossein Afarideh, Mohammad Ghannadi-Maragheh, Ali Bahrami-Samani, Mehdi Asgari
Radioimmuno-conjugated (Rhenium-188 labeled Rituximab), 3-aminopropyltriethoxysilane (APTES)-polyethylene glycol (PEG) coated iron oxide nanoparticles were synthesized and then characterized. Therapeutic effect and targeting efficacy of complex were evaluated in CD20 express B cell lines and tumor bearing Balb/c mice respectively. To reach these purposes, superparamagnetic iron oxide nanoparticles (SPIONs) were synthesized using coprecipitation method and then their surface was treated with APTES for increasing retention time of SPIONs in blood circulation and amine group creation...
May 6, 2016: Nuclear Medicine and Biology
https://www.readbyqxmd.com/read/28186720/cuprizone-intoxication-induces-cell-intrinsic-alterations-in-oligodendrocyte-metabolism-independent-of-copper-chelation
#12
Alexandra Taraboletti, Tia Louise Walker, Robin Avila, He Huang, Joel Caporoso, Erendra Manandhar, Thomas C Leeper, David A Modarelli, Satish Medicetty, Leah P Shriver
Cuprizone intoxication is a common animal model used to test myelin regenerative therapies to treat diseases such as multiple sclerosis. Mice fed this copper chelator develop reversible, region-specific oligodendrocyte loss and demyelination. While the cellular changes influencing the demyelinating process have been explored in this model, there is no consensus on the biochemical mechanisms of toxicity in oligodendrocytes and if this damage arises from the chelation of copper in vivo. Here we have identified an oligodendroglial cell line that displays sensitivity to cuprizone toxicity and performed global metabolomic profiling to determine biochemical pathways altered by this treatment...
February 10, 2017: Biochemistry
https://www.readbyqxmd.com/read/28185035/anthracycline-chemotherapy-and-cardiotoxicity
#13
John V McGowan, Robin Chung, Angshuman Maulik, Izabela Piotrowska, J Malcolm Walker, Derek M Yellon
Anthracycline chemotherapy maintains a prominent role in treating many forms of cancer. Cardiotoxic side effects limit their dosing and improved cancer outcomes expose the cancer survivor to increased cardiovascular morbidity and mortality. The basic mechanisms of cardiotoxicity may involve direct pathways for reactive oxygen species generation and topoisomerase 2 as well as other indirect pathways. Cardioprotective treatments are few and those that have been examined include renin angiotensin system blockade, beta blockers, or the iron chelator dexrazoxane...
February 9, 2017: Cardiovascular Drugs and Therapy
https://www.readbyqxmd.com/read/28169355/enhancement-of-5-aminolevulinic-acid-based-fluorescence-detection-of-side-population-defined-glioma-stem-cells-by-iron-chelation
#14
Wenqian Wang, Kouichi Tabu, Yuichiro Hagiya, Yuta Sugiyama, Yasuhiro Kokubu, Yoshitaka Murota, Shun-Ichiro Ogura, Tetsuya Taga
Cancer stem cells (CSCs) are dominantly responsible for tumor progression and chemo/radio-resistance, resulting in tumor recurrence. 5-aminolevulinic acid (ALA) is metabolized to fluorescent protoporphyrin IX (PpIX) specifically in tumor cells, and therefore clinically used as a reagent for photodynamic diagnosis (PDD) and therapy (PDT) of cancers including gliomas. However, it remains to be clarified whether this method could be effective for CSC detection. Here, using flow cytometry-based analysis, we show that side population (SP)-defined C6 glioma CSCs (GSCs) displayed much less 5-ALA-derived PpIX fluorescence than non-GSCs...
February 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28168622/alpha-synuclein-and-iron-two-keys-unlocking-parkinson-s-disease
#15
REVIEW
Paul Lingor, Eleonora Carboni, Jan Christoph Koch
Current therapies for Parkinson's disease (PD) confer symptomatic relief and are particularly efficient in the treatment of motor symptoms in earlier disease stages. However, we are still unable to treat the causes of neurodegeneration by modification of the underlying mechanisms, which is partially due to their insufficient understanding. In this short review, we focus on two pivotal disease mechanisms: alpha-synuclein pathology and dysfunction of iron homeostasis as well as their intricate interaction. Both pathomechanisms have been extensively studied in the past and represent valid targets for disease-modifying pharmacological treatment approaches for PD...
February 6, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/28163981/current-advances-in-5-aminolevulinic-acid-mediated-photodynamic-therapy
#16
Connor Thunshelle, Rui Yin, Qiquan Chen, Michael R Hamblin
Kennedy and Pottier discovered that photodynamic therapy (PDT) could be carried out using a procedure consisting of topical application of the porphyrin-precursor, 5-aminolevulinic acid (ALA) to the skin, followed after some time by illumination with various light parameters in the 1980s. Since then, ALA-PDT has expanded enormously and now covers most aspects of dermatological disease. The purpose of this review is to discuss a range of ingenious strategies that investigators have devised for improving the overall outcome (higher efficiency and lower side effects) of ALA-PDT...
September 2016: Current Dermatology Reports
https://www.readbyqxmd.com/read/28161963/effects-of-heme-degradation-products-on-reactivation-of-latent-hiv-1
#17
P Shankaran, M Madlenakova, V Hajkova, D Jilich, I Svobodova, A Horinek, Y Fujikura, Z Melkova
Human immunodeficiency virus (HIV-1) infection can be currently controlled by combined antiretroviral therapy, but a sterilizing cure is not achievable as this therapy does not target persistent HIV-1 in latent reservoirs. Therefore, different latency reversal agents are intensively explored in various models. We have previously observed that heme arginate, a drug approved for human use, reveals a strong synergism with PKC inducers in reactivation of the latent provirus. Heme is physiologically decomposed by heme oxygenases into 3 degradation products: iron (Fe2+), carbon monoxide (CO) and biliverdin which is further converted to bilirubin by biliverdin reductase...
February 6, 2017: Acta Virologica
https://www.readbyqxmd.com/read/28159390/sickle-cell-disease
#18
REVIEW
Russell E Ware, Mariane de Montalembert, Léon Tshilolo, Miguel R Abboud
Sickle cell disease is a common and life-threatening haematological disorder that affects millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in small vessels, and this vaso-occlusion leads to distal tissue ischaemia and inflammation, with symptoms defining the acute painful sickle-cell crisis. Repeated sickling and ongoing haemolytic anaemia, even when subclinical, lead to parenchymal injury and chronic organ damage, causing substantial morbidity and early mortality. Currently available treatments are limited to transfusions and hydroxycarbamide, although stem cell transplantation might be a potentially curative therapy...
January 31, 2017: Lancet
https://www.readbyqxmd.com/read/28156470/patient-reported-distress-in-adults-with-myelodysplastic-syndromes-mds
#19
Carlos deCastro, Mary Ruth Pupa, Greg P Samsa, Amy Pickar Abernethy, Thomas William LeBlanc
: 80 Background: MDS are hematopoietic malignancies that are incurable in most patients. The National Comprehensive Cancer Network (NCCN) defines distress as an unpleasant emotional experience associated with the psychosocial complications of cancer that may interfere with quality of life. Distress is not well described in MDS and may be useful to screen for palliative care needs. METHODS: We conducted a retrospective review of distress in ambulatory adult MDS patients visiting our clinics from July 2013 to September 2015...
October 9, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28144156/update-on-the-clinical-management-of-wilson-s-disease
#20
REVIEW
Peter Hedera
Wilson's disease (WD), albeit relatively rare, is an important genetic metabolic disease because of highly effective therapies that can be lifesaving. It is a great imitator and requires a high index of suspicion for correct and timely diagnosis. Neurologic, psychiatric and hepatologic problems in WD are very nonspecific, and we discuss the most common clinical phenotypes. The diagnosis remains laboratory based, and here we review the most important challenges and pitfalls in laboratory evaluation of WD, including the emerging role of genetic testing in WD diagnosis...
2017: Application of Clinical Genetics
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