Article
Hematology
John Chapin, Alan R. Cohen, Ellis J. Neufeld, Elliott Vichinsky, Patricia J. Giardina, Jeanne Boudreaux, Binh C. Le, Kristy Kenney, Sean Trimble, Alexis A. Thompson
Summary: Thalassaemia is a genetic disorder causing anaemia and dependence on transfusions, with recent clinical advancements leading to reduced mortality and potential future gene therapies. Research revealed older patients with more iron-related comorbidities and complications, while younger patients had excess liver and heart iron and were on combination chelation therapy.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Review
Medicine, General & Internal
Antonis Kattamis, Janet L. Kwiatkowski, Yesim Aydinok
Summary: Thalassaemia is a genetic disorder that affects globin chain synthesis and has a worldwide distribution. The unbalanced production of globin chains leads to ineffective erythropoiesis, increased haemolysis, and disrupted iron homeostasis. The clinical phenotype varies from mild to severe, with treatment options including transfusion, iron chelation, allogeneic haematopoietic stem cell transplantation, and gene therapy.
Review
Multidisciplinary Sciences
Yesim Aydinok
Summary: Combination chelation therapy is a viable option for transfusion-dependent thalassemia patients who have not achieved iron balance with monotherapy or need intensified treatment for rapid iron reduction. It allows for a more personalized approach, improving tolerability, adherence, and quality of life. Real-life data highlights the importance of compliance in achieving meaningful reduction in iron overload compared to monotherapy.
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES
(2023)
Article
Toxicology
Ruiying Cheng, Rajitha Gadde, Yingfang Fan, Neha Kulkarni, Nachiket Shevale, Kai Bao, Hak Soo Choi, Swati Betharia, Jonghan Kim
Summary: N,N'-bis(2-mercaptoethyl)isophthalamide (NBMI) is a lipophilic metal chelator and antioxidant used for mercury poisoning. It has shown potential in binding to other metals like lead and iron. In this study, it was found that NBMI selectively binds to ferrous iron, protecting neuroblastoma cells from high iron-mediated cytotoxicity and reducing reactive oxygen species. Furthermore, NBMI effectively reduced iron accumulation in the brain and liver of iron-loaded mice, with a superior safety profile compared to the commonly used iron chelator, DFP. These findings highlight the neuroprotective and therapeutic potential of NBMI for neurodegenerative diseases associated with brain iron accumulation.
ARCHIVES OF TOXICOLOGY
(2022)
Review
Nutrition & Dietetics
William Kardasis, Ethan R. Naquin, Richa Garg, Tejas Arun, Jyotsna S. Gopianand, Eshani Karmakar, Jaya P. Gnana-Prakasam
Summary: Iron is a crucial micronutrient for athletes, but impaired iron levels can hinder athletic performance. Prolonged exercise can affect iron absorption, utilization, and storage, increasing the risk of iron deficiency. On the other hand, iron overload may initially enhance performance but can have detrimental health consequences in the long term. Proper management of iron intake is crucial for improving athletic performance and overall well-being.
Review
Cardiac & Cardiovascular Systems
Shizhen Li, Xiangyu Zhang
Summary: Iron plays essential roles in cardiovascular diseases, with iron deficiency having detrimental effects in heart failure and pulmonary arterial hypertension patients. Ferroptosis, an iron-dependent cell death, has been reported in cardiomyopathy, atherosclerotic cardiovascular disease, and myocardial ischemia/reperfusion injury. Iron chelation therapy may be a potential strategy to address iron overload-related disorders in CVD.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2021)
Review
Medicine, General & Internal
Toni Valkovic, Marija Stanic Damic
Summary: Iron plays an important role in the survival and reproduction of fungal pathogens, and there is evidence linking invasive fungal infections to iron or iron overload. Understanding the association between iron and iron overload with susceptibility to invasive fungal infections is crucial, especially for patients with hematological malignancies.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Gerard M. Healy, Stephan A. R. Kannengiesser, Osvaldo Espin-Garcia, Richard Ward, Kevin H. M. Kuo, Kartik S. Jhaveri
Summary: Inline R-2*-LIC provides robust and comparable quantification of LIC compared to FerriScan, without the need for offline post-processing.
EUROPEAN RADIOLOGY
(2021)
Article
Pharmacology & Pharmacy
Mehrdad Alikhani, Mahdi Aalikhani, Masoumeh Khalili
Summary: This study investigated the effects of several natural compounds on iron-overload in the heart and found that hesperidin, vanillin, and ferulic acid were the most effective antioxidant compounds, improving the activity of antioxidant enzymes and reducing iron deposition.
EUROPEAN JOURNAL OF PHARMACOLOGY
(2022)
Review
Chemistry, Analytical
Zahra Tariq, Muhammad Imran Qadeer, Iram Anjum, Christophe Hano, Sumaira Anjum
Summary: Thalassemia is a monogenic autosomal recessive disorder caused by mutations, which affect hemoglobin production. Conventional therapeutic methods such as transfusion and iron chelation therapy have limitations, leading to the demand for advanced therapies. Nanotechnology-based applications have emerged as simple, convenient, and cost-effective methods for theragnostics. This review summarizes the potential of nanotechnology in developing theragnostic approaches for diagnosing thalassemia-causing gene mutations and employing nano-based therapeutic procedures.
Review
Immunology
Sarah Weber, Anastasia Parmon, Nina Kurrle, Frank Schnuetgen, Hubert Serve
Summary: MDS and AML patients often experience systemic iron overload issues, iron is closely associated with the pathogenesis and potential treatment strategies of the diseases, imbalances in iron homeostasis may lead to various cell death.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Chemistry, Multidisciplinary
Srinivas Abbina, Usama Abbasi, Arshdeep Gill, Heather Leitch, Jayachandran N. Kizhakkedathu
Summary: Liver dysfunction and failure are major causes of premature death in patients with iron-related pathologies. This study developed a new class of liver targeted nanochelators, which can rapidly reduce iron burden in patients with iron overload diseases, with favorable pharmacokinetics and biodistribution.
JOURNAL OF CONTROLLED RELEASE
(2022)
Review
Pharmacology & Pharmacy
Laura Grech, Karen Borg, Joseph Borg
Summary: Beta-thalassaemia, a significant haemoglobinopathy, is currently managed with treatments such as repeated transfusions and iron-chelating therapy, while novel approaches like gene therapy and gene editing are being developed to address the underlying causes of the disease. These new therapies aim to correct the imbalance of globin chains, treat ineffective erythropoiesis, and improve iron overload associated with beta-thalassaemia.
BRITISH JOURNAL OF CLINICAL PHARMACOLOGY
(2022)
Review
Chemistry, Medicinal
George J. Kontoghiorghes
Summary: The iron chelating orphan drug deferiprone (L1), which has been used daily by patients at high doses for over 30 years with no serious toxicity, has unique properties that have played a major role in transforming thalassaemia from a fatal to a chronic disease. These properties include oral effectiveness, highly effective iron removal from organs, rapid absorption and clearance from the body, and the ability to cross the blood-brain barrier. Additionally, L1 exhibits specific protein interactions, antioxidant effects, and the ability to inhibit iron-containing proteins associated with various pathological conditions.
Article
Agriculture, Dairy & Animal Science
Kathleen E. Sullivan, Shana R. Lavin, Shannon Livingston, Mitchell Knutson, Eduardo V. Valdes, Lori K. Warren
Summary: Iron overload disorder is prevalent in black rhinoceroses, and the use of an oral iron chelator showed potential for treatment in horses. The study found that the chelator increased urinary iron output in horses without any adverse effects.
JOURNAL OF ANIMAL PHYSIOLOGY AND ANIMAL NUTRITION
(2022)