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https://www.readbyqxmd.com/read/28320041/prevention-of-iron-overload-and-long-term-maintenance-of-normal-iron-stores-in-thalassaemia-major-patients-using-deferiprone-or-deferiprone-deferoxamine-combination
#1
Annita Kolnagou, Christina N Kontoghiorghe, George J Kontoghiorghes
Decrease in mortality and morbidity is observed in thalassaemia major patients with reduced iron load in comparison to heavy iron loaded patients. Effective and complete treatment of transfusional iron overload can be achieved by chelation protocols that can eliminate excess iron and maintain normal iron stores (NIS). The maintenance of NIS, i. e., serum ferritin (350 μg/L >), MRI T2* cardiac (>20 ms) and liver (>6.3 ms) relaxation time levels was monitored in 16 thalassaemia major patients (32-53 years, 12 splenectomized, 10 male, erythrocyte transfusions 120-323 ml/kg/year) for about 90 patient years...
March 20, 2017: Drug Research
https://www.readbyqxmd.com/read/28318192/-abdominal-pain-as-a-presentation-by-lead-poisoning-case-report
#2
Daniel M Mottiera, Elda Cargnel
Acute lead poisoning is not a common pathology seen in the pediatrician's office. Lead poisoning symptoms can be digestive or neurological, and they can be confused with other pathologies. That is the reason why it should be considered and, in case of doubt, complementary studies to confirm lead poisoning should be requested. This is the case of a nine-year-old child that comes to the office with a strong abdominal pain and vomiting, and after a close physical examination and a detailed anamnesis, a suspicious diagnosis of "acute" lead poisoning is obtained...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28301910/soluble-fms-like-tyrosine-kinase-1-as-a-link-between-angiogenesis-and-endothelial-dysfunction-in-pediatric-patients-with-%C3%AE-thalassemia-intermedia
#3
Azza Abdel Gawad Tantawy, Amira Abdel Moneam Adly, Eman Abdel Rahman Ismail, Omneya Ibrahim Youssef, Mohamed ElSayed Ali
Endothelial damage has been implicated in the pathogenesis of vascular complications in β-thalassemia intermedia (β-TI). Soluble fms-like tyrosine kinase 1 (sFLT-1) is a member of the vascular endothelial growth factor receptor (VEGFR) family. Soluble fms-like tyrosine kinase 1 is an antiangiogenic protein that induces endothelial dysfunction by adhering to and inhibiting VEGF and placenta growth factor. The aim of this study was to assess the level of sFLT-1 in 35 children and adolescents with β-TI, correlating it with markers of hemolysis and iron overload as well as cardiopulmonary complications...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28300275/iron-chelation-therapy-in-low-risk-myelodysplastic-syndrome
#4
REVIEW
Sally B Killick
Anaemia is the commonest cytopenia seen in patients with myelodysplastic syndrome (MDS), and the majority of patients will require transfusion support at some point. Blood transfusions are rich in iron, which leads to the accumulation of body iron over time. It is accepted that this ultimately causes end organ damage and may impact on both morbidity and mortality. In addition, recent data has increased our interest in the subject with regard to the potential impact on stem cell transplant outcome and an anti-leukaemic effect of iron chelation therapy...
March 16, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28296163/long-term-safety-and-efficacy-of-deferasirox-in-young-pediatric-patients-with-transfusional-hemosiderosis-results-from-a-5-year-observational-study-entrust
#5
Elliott Vichinsky, Amal El-Beshlawy, Azzam Al Zoebie, Annie Kamdem, Suzanne Koussa, Thirachit Chotsampancharoen, Andreas Bruederle, Geralyn Gilotti, Jackie Han, Mohsen Elalfy
BACKGROUND: Children with red blood cell disorders may receive regular transfusions from an early age and consequently accumulate iron. Adequate iron chelation therapy can prevent organ damage and delayed growth/development. Deferasirox is indicated for treatment of pediatric patients with chronic iron overload due to transfusional hemosiderosis; however, fewer than 10% of patients in the registration studies were aged 2 to less than 6 years. PROCEDURE: Deferasirox, a once-daily oral iron chelator, was evaluated in young pediatric patients with transfusional hemosiderosis during the observational 5-year ENTRUST study...
March 10, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28295188/how-we-manage-iron-overload-in-sickle-cell-patients
#6
REVIEW
Thomas D Coates, John C Wood
Blood transfusion plays a prominent role in the management of patients with sickle cell disease (SCD), but causes significant iron overload. As transfusions are used to treat the severe complications of SCD, it remains difficult to distinguish whether organ damage is a consequence of iron overload or is due to the complications treated by transfusion. Better management has resulted in increased survival, but prolonged exposure to iron puts SCD patients at greater risk for iron-related complications that should be treated...
March 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28293409/myelodysplastic-syndromes-and-iron-chelation-therapy
#7
REVIEW
Emanuele Angelucci, Silvana Anna Maria Urru, Federica Pilo, Alberto Piperno
Over recent decades we have been fortunate to witness the advent of new technologies and of an expanded knowledge and application of chelation therapies to the benefit of patients with iron overload. However, extrapolation of learnings from thalassemia to the myelodysplastic syndromes (MDS) has resulted in a fragmented and uncoordinated clinical evidence base. We're therefore forced to change our understanding of MDS, looking with other eyes to observational studies that inform us about the relationship between iron and tissue damage in these subjects...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28290970/severe-systemic-lead-toxicity-resulting-from-extra-articular-retained-shrapnel-presenting-as-jaundice-and-hepatitis-a-case-report-and-review-of-the-literature
#8
Ian A Grasso, Matthew R Blattner, Tyler Short, John W Downs
INTRODUCTION: Despite greater than 60,000 nonfatal firearm injuries per year in the United States, retained shrapnel is a relatively rare cause of systemic lead toxicity with less than 100 cases reported in the medical literature since 1867. While intra-articular retained shrapnel as a cause of lead toxicity is well-described, extra-articular fragments are less well known to cause symptomatic disease. CASE REPORT: A 31-year-old man initially presented with abdominal pain, constipation, jaundice, and elevated liver transaminases approximately 3 weeks after suffering a left lower extremity injury during athletic activity...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28288539/ischemia-modified-albumin-as-a-marker-of-vascular-dysfunction-and-subclinical-atherosclerosis-in-%C3%AE-thalassemia-major
#9
Amira Abdel Moneam Adly, Nayera Hazaa Khalil ElSherif, Eman Abdel Rahman Ismail, Yosra Abdelzaher Ibrahim, Gamal Niazi, Sara Hamed Elmetwally
BACKGROUND: Ischemia-modified albumin (IMA) is an altered type of serum albumin that forms under conditions of oxidative stress and an independent predictor of major adverse cardiovascular events. OBJECTIVES: To measure the levels of IMA in 45 children and adolescents with β-thalassemia major (β-TM) compared with 30 healthy controls and assess its relation to lipid peroxidation, vascular complications and subclinical atherosclerosis. METHODS: β-TM patients without symptoms of heart disease were studied focusing on transfusion history, chelation therapy, serum ferritin, malondialdehyde (MDA) and IMA levels...
March 13, 2017: Redox Report: Communications in Free Radical Research
https://www.readbyqxmd.com/read/28284907/minocycline-protects-rescues-and-prevents-knockdown-transgenic-parkin-drosophila-against-paraquat-iron-toxicity-implications-for-autosomic-recessive-juvenile-parkinsonism
#10
Hector Flavio Ortega-Arellano, Marlene Jimenez-Del-Rio, Carlos Velez-Pardo
Autosomal recessive Juvenile Parkinsonism (AR-JP) is a chronic, progressive neurodegenerative disorder caused by mutation in the PARKIN gene, and invariably associated with dopaminergic (DAergic) neuronal loss and brain iron accumulation. Since current medical therapy is symptomatic and lacks significant disease-modifying effects, other treatment approaches are urgently needed it. In the present work, we investigate the role of minocycline (MC) in paraquat (PQ)/iron-induced neurotoxicity in the Drosophila TH>parkin-RNAi/+ (w[*]; UAS-parkin-RNAi; TH-GAL4) fly and have shown the following: (i) MC increased life span and restored the locomotor activity of knockdown (KD) transgenic parkin flies in comparison with the control (vehicle) group; (ii) MC at low (0...
March 8, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28281897/patients-with-acute-myeloid-leukemia-can-be-subclassified-based-on-the-constitutive-cytokine-release-of-the-leukemic-cells-the-possible-clinical-relevance-and-the-importance-of-cellular-iron-metabolism
#11
Annette K Brenner, Tor Henrik Anderson Tvedt, Ina Nepstad, Kristin P Rye, Karen M Hagen, Håkon Reikvam, Øystein Bruserud
OBJECTIVE: Acute myeloid leukaemia (AML) is a heterogeneous malignancy; we studied how the constitutive cytokine release by the AML cells varies among patients. METHODS: We investigated the constitutive release of 28 mediators during in vitro culture for 79 consecutive patients. RESULTS: Constitutive cytokine release profiles differed among patients, and hierarchical clustering identified three subsets with high, intermediate and low release, respectively...
April 2017: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/28281098/effect-of-metal-chelators-on-the-aggregation-of-beta-amyloid-peptides-in-the-presence-of-copper-and-iron
#12
Foozhan Tahmasebinia, Saeed Emadi
Amyloid β (Aβ) fibrils and amorphous aggregates are found in the brain of patients with Alzheimer's disease (AD), and are implicated in the etiology of AD. The metal imbalance is also among leading causes of AD, owing to the fact that Aβ aggregation takes place in the synaptic cleft where Aβ, Cu(II) and Fe(III) are found in abnormally high concentrations. Aβ40 and Aβ42 are the main components of plaques found in afflicted brains. Coordination of Cu(II) and Fe(III) ions to Aβ peptides have been linked to Aβ aggregation and production of reactive oxygen species, two key events in the development of AD pathology...
April 2017: Biometals: An International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine
https://www.readbyqxmd.com/read/28280824/one-pot-method-to-prepare-a-theranostic-nanosystem-with-magnetic-resonance-imaging-function-and-anticancer-activity-through-multiple-mechanisms
#13
Enyi Chen, Xin Chen, Xiuxue Yuan, Shaohua Wei, Lin Zhou, Jiahong Zhou, Jian Shen
A facile and robust one-pot approach to prepare a theranostic nanoplatform, based on chelation between Gd(3+) and hypericin photosensitizer (PS) and their controlled in situ hydrolysis precipitation, was developed. In this strategy, PS drugs and Gd(3+) were directly used as building blocks to construct theranostic nanoparticles, resulting in a greatly increased active substance-loading efficiency and ensuring their theranostic effect. The resulting nanoparticles have multifunction capabilities for nuclear magnetic resonance (NMR) imaging and anticancer activity through adenosine triphosphate (ATP) deprivation and heavy atom effect (HAE)-improved photodynamic therapy (PDT) mechanisms...
March 10, 2017: Dalton Transactions: An International Journal of Inorganic Chemistry
https://www.readbyqxmd.com/read/28274224/effect-of-microbial-siderophores-on-mammalian-non-malignant-and-malignant-cell-lines
#14
Karuna Gokarn, Vishwas Sarangdhar, Ramprasad B Pal
BACKGROUND: Iron is a vital nutrient for all cells, and malignant cells have a higher requirement for the metal due to their rapid multiplication. Bacterial siderophores can be used to reduce free ferric ion concentration from the cellular environment. METHODS: In the present study, we have evaluated effect of three siderophores - exochelin-MS, mycobactin S and deferoxamine B on the proliferation of mammalian cell lines using MTT assay. RESULTS: These siderophores caused a significant decrease in the viability of malignant cells, without significantly affecting non-malignant cells...
March 9, 2017: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/28267454/from-bench-to-bed-new-gastrin-releasing-peptide-receptor-directed-radioligands-and-their-use-in-prostate-cancer
#15
REVIEW
Theodosia Maina, Berthold A Nock
Gastrin-releasing peptide receptors (GRPRs) are overexpressed in prostate and breast cancer, and are therefore attractive molecular targets for diagnosis and therapy with radiolabeled GRPR-directed peptide probes. The amphibian tetradecapeptide bombesin or the mammalian gastrin-releasing peptide and neuromedin C have been modified with a variety of chelators. As a result, labeling with radiometals attractive for SPECT or PET imaging and for radionuclide therapy has led to the development of peptide radioligands suitable for in vivo targeting of prostate cancer...
April 2017: PET Clinics
https://www.readbyqxmd.com/read/28263719/mechanism-of-ascaridole-activation-in-leishmania
#16
Gerald Geroldinger, Matthias Tonner, Hubert Hettegger, Markus Bacher, Lianet Monzote, Martin Walter, Katrin Staniek, Thomas Rosenau, Lars Gille
Endoperoxides (EP) are an emerging class of drugs which have potential in antiparasitic therapy, but also in other fields. For malaria therapy the EP artemisinin (Art) and its derivatives are successfully used. We have shown in the past that the EP ascaridole (Asc) is useful for the treatment of cutaneous leishmaniasis in a mouse model. Biomimetic experiments suggested that these drugs need activation in the respective target pathogens to exert their function. In spite of this idea, direct activation of EP to radicals inside cells has never been demonstrated...
March 2, 2017: Biochemical Pharmacology
https://www.readbyqxmd.com/read/28247750/synergistic-cisplatin-doxorubicin-combination-chemotherapy-for-multidrug-resistant-cancer-via-polymeric-nanogels-targeting-delivery
#17
Haiqiu Wu, Haojie Jin, Cun Wang, Zihao Zhang, Haoyu Ruan, Luyan Sun, Chen Yang, Yongjing Li, Wenxin Qin, Changchun Wang
Combination chemotherapy has been proposed to achieve synergistic effect and minimize drug dose for cancer treatment in clinic application. In this article, the stimuli-responsive polymeric nanogels (<100 nm in size) based on poly(acrylic acid) were designed as codelivery system for doxorubicin and cisplatin to overcome drug resistance. By chelation, electrostatic interaction, and π-π stacking interactions, the nanogels could encapsulate doxorubicin and cisplatin with designed ratio and high capacity. Compared with free drugs, the nanogels could deliver more drugs into MCF-7/ADR cells...
March 22, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28245422/-underlying-mechanisms-of-iron-overloading-in-myelodysplastic-syndrome-review
#18
Qing-Qing Zheng, Chun-Kang Chang
Myelodysplastic Syndromes(MDS) comprise a heterogenous group of hematopoietic stem cell malignancies characterized by peripheral cytopenias and have a substantial risk of progression to acute myeloid leukemia(AML). MDS, without effective cure methods, is one of the common hematologic malignant tumors with great threaten to people's health. The phenomenon of iron overloading is common in MDS, which has a poor effect on overall survival and leukemic progression to MDS but get good prognosis by iron chelation therapy...
February 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28243431/combination-iron-chelation-therapy-with-deferiprone-and-deferasirox-in-iron-overloaded-patients-with-transfusion-dependent-%C3%AE-thalassemia-major
#19
Hossein Karami, Mehrnoush Kosaryan, Arash Hadian Amree, Hadi Darvishi-Khezri, Masoomeh Mousavi
There are few papers on the combination therapy of deferiprone (DFP) and deferasirox (DFX) in iron-overloaded patients with transfusion-dependent β-thalassemia major (β-TM). A total of 6 patients with β-TM (5 males and 1 female) with a mean age of 23.8±5.8 years (ranging from 17 to 31) used this treatment regimen. The mean doses of DFP and DFX were 53.9±22.2 and 29.3±6.8 mg/kg/day, respectively. The duration of treatment was 11.5±4.6 months. Their serum ferritin levels were measured to be 2800±1900 and 3400±1600 ng/mL before and after treatment, respectively (p<0...
January 11, 2017: Clinics and Practice
https://www.readbyqxmd.com/read/28242540/relation-between-chelation-and-clinical-outcomes-in-lower-risk-patients-with-myelodysplastic-syndromes-registry-analysis-at-5-years
#20
REVIEW
Roger M Lyons, Billie J Marek, Carole Paley, Jason Esposito, Katie McNamara, Paul D Richards, Nicholas DiBella, Guillermo Garcia-Manero
Prospective data are needed to ascertain the impact of iron chelation therapy in patients with myelodysplastic syndromes. The present 5-year prospective registry analysis was conducted to compare clinical outcomes between chelated and nonchelated patients with lower-risk myelodysplastic syndromes and transfusional iron overload. In an interim analysis at 24 months, we previously reported that chelation therapy was associated with longer median overall survival and a tendency toward longer leukemia-free survival and fewer cardiac events...
January 31, 2017: Leukemia Research
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