Article
Biochemistry & Molecular Biology
Ying Liu, Carine Fillebeen, Anik Forest, Amy Botta, Thibault V. Varin, Andre Marette, Yan Burelle, Christine Des Rosiers, Kostas Pantopoulos, Gary Sweeney
Summary: This study using the Th3/+ mouse model found that altered lipid metabolism and gut microbiota may be related to the development of cardiomyopathy in thalassemia. These findings are important for understanding the early biomarkers and therapeutic targets for the development of cardiomyopathy in thalassemia.
Article
Biochemistry & Molecular Biology
Patricia Reboucas, Carine Fillebeen, Amy Botta, Riley Cleverdon, Alexandra P. Steele, Vincent Richard, Rene P. Zahedi, Christoph H. Borchers, Yan Burelle, Thomas J. Hawke, Kostas Pantopoulos, Gary Sweeney
Summary: Using global proteomics, we found significant impairment in mitochondrial oxidative phosphorylation in the skeletal muscle of thalassemia mice, along with a shift in muscle fiber types and increased capillary density. These alterations led to metabolic dysfunction and reduced glucose handling capacity.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Engineering, Electrical & Electronic
Ahmed Karam Eldaly, Ayman M. Khalifa
Summary: This article proposes a general framework for estimating iron overload in the liver using magnetic resonance imaging (MRI), considering various liver models. The iron overload estimation is formulated as a minimization problem with regularization functions assigned to unknown model parameters. An alternating direction method of multipliers (ADMM) is applied for parameter estimation. Three models were tested and compared using synthetic datasets, showing good correlation with ground truth T2* measurements. The proposed approaches were also evaluated using MRI scans of patients, demonstrating better performance than existing methods.
DIGITAL SIGNAL PROCESSING
(2023)
Article
Biochemistry & Molecular Biology
Hanan Kamel M. Saad, Alawiyah Awang Abd Rahman, Azly Sumanty Ab Ghani, Wan Rohani Wan Taib, Imilia Ismail, Muhammad Farid Johan, Abdullah Saleh Al-Wajeeh, Hamid Ali Nagi Al-Jamal
Summary: Iron homeostasis is regulated by hepcidin, which is influenced by factors such as iron stores, hypoxia, inflammation, and erythropoiesis. Beta-thalassemia patients exhibit iron deficiency, iron overload, and ineffective erythropoiesis. Recent research has identified new diagnostic markers and therapeutic targets for beta-thalassemia.
Article
Physiology
Kornvipa Settakorn, Sarawut Kongkarnka, Anchan Chompupoung, Saovaros Svasti, Suthat Fucharoen, John B. Porter, Somdet Srichairatanakool, Pimpisid Koonyosying
Summary: This study found that green tea extract rich in epigallocatechin-3-gallate has positive effects in alleviating ineffective erythropoiesis, iron dysregulation, and oxidative stress in beta-thalassemic mice.
FRONTIERS IN PHYSIOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Davide Schiroli, Lucia Merolle, Eleonora Quartieri, Roberta Chicchi, Tommaso Fasano, Tiziana De Luca, Giuseppe Molinari, Stefano Pulcini, Thelma A. Pertinhez, Erminia Di Bartolomeo, Rino Biguzzi, Roberto Baricchi, Chiara Marraccini
Summary: This study compared two methods for preparing optimal P-RBCs products for beta-thalassemia major patients, finding that method 1 resulted in higher RBC count and Hb content, while method 2 produced P-RBCs with lower levels of K+, iron, and storage lesions markers. Further clinical studies are needed to determine the impact of these differences on transfusion-related complications and the quality of life of beta TM patients.
Article
Medicine, General & Internal
Reem Aldwaik, Tamara Abu Mohor, Israa Idyabi, Salam Warasna, Shatha Abdeen, Bashar Karmi, Rania Abu Seir
Summary: Management of beta-thalassemia in the West Bank presents challenges due to the lack of available therapies, leading to recurrent transfusions. Characteristics of patients include anemia, iron overload, and abnormal liver function tests. Iron chelation medication choice is associated with serum ferritin levels, highlighting the need for personalized assessment and follow-up protocols.
FRONTIERS IN MEDICINE
(2021)
Article
Medicine, Research & Experimental
Pakawit Lerksaipheng, Kittiphong Paiboonsukwong, Pimtip Sanvarinda, Rataya Leuchapudiporn, Ken-Ichi Yamada, Noppawan Phumala Morales
Summary: This study suggests that vascular complications in patients with beta-thalassemia are associated with oxidative modification of lipoproteins. The modification of lipid components may alter lipid peroxidation rate and products. Core lipids and cholesteryl esters levels determine the rate and total production of L center dot. Overall, lipoproteins in beta-thalassemia patients are more susceptible to lipid peroxidation.
BIOMEDICINE & PHARMACOTHERAPY
(2022)
Article
Hematology
Nashwa El-Khazragy, Safa Matbouly, Demiana H. Hanna, Nievin Ahmed Mahran, Sally Abdallah Mostafa, Bassam A. Abdelrehim, Yasmeen K. Farouk, Soha Abuelela
Summary: The study identifies an association between three iron-related miRNAs and tissue iron overload in transfusion-dependent beta-thalassemia major patients. Results suggest that circulating miR-let-7d and miR-200b are downregulated, while miR-122 is upregulated in TDT patients. Aberrant expression of miRNAs is significantly associated with increased iron accumulation in hepatic and cardiac tissues, making them potential predictive biomarkers for tissue iron excess.
ANNALS OF HEMATOLOGY
(2021)
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
Hematology
Suneerat Hatairaktham, Patarabutr Masaratana, Chattree Hantaweepant, Chatchawan Srisawat, Vorapan Sirivatanauksorn, Noppadol Siritanaratkul, Narumol Panichkul, Ruchaneekorn W. Kalpravidh
Summary: The study demonstrates that curcuminoids have significant therapeutic effects on non-transfused beta-thalassemia/Hb E patients, including reducing oxidative stress, hypercoagulability, and inflammation markers levels, especially in the 1000 mg/day dosage group. Furthermore, the baseline ferritin levels also impact the efficacy of curcuminoids supplementation.
ANNALS OF HEMATOLOGY
(2021)
Article
Hematology
Antonella Meloni, Luigi Barbuto, Laura Pistoia, Vincenzo Positano, Stefania Renne, Giuseppe Peritore, Priscilla Fina, Anna Spasiano, Massimo Allo, Giuseppe Messina, Tommaso Casini, Antonella Massa, Luigia Romano, Alessia Pepe, Filippo Cademartiri
Summary: Renal iron accumulation is not common in sickle/beta-thalassemia patients, with a significantly lower frequency compared to homozygous SCD patients. Kidney T2* values are similar between sickle/beta-thalassemia and TM patients, but significantly lower in regularly transfused homozygous SCD patients. Age, gender, splenectomy, and transfusion or chelation therapy are not associated with global renal T2* values, but there is a significant inverse correlation with serum lactate dehydrogenase and indirect bilirubin.
ANNALS OF HEMATOLOGY
(2022)
Article
Medicine, Research & Experimental
Sirawit Sriwichaiin, Parameth Thiennimitr, Chanisa Thonusin, Phinitphong Sarichai, Songphon Buddhasiri, Sirinart Kumfu, Wichwara Nawara, Weerayuth Kittichotirat, Suthat Fucharoen, Nipon Chattipakorn, Siriporn C. Chattipakorn
Summary: This study investigated the changes in gut microbiota and metabolites in iron-overload thalassemia, as well as the effect of an iron chelator on gut dysbiosis and inflammation. The results showed that iron-overload led to gut dysbiosis, inflammation, and disturbance of metabolites, and treatment with the iron chelator deferiprone alleviated these conditions more effectively in wild-type mice than in thalassemic mice. The study suggests that deferiprone may be a potential therapeutic drug for iron-related gut diseases.
Article
Radiology, Nuclear Medicine & Medical Imaging
Jingwen Huang, Jun Shen, Qihua Yang, Ziliang Cheng, Xiaodong Chen, Taihui Yu, Jinglian Zhong, Yun Su, Hua Guo, Biling Liang
Summary: Pediatric TM patients can have homogeneous iron siderosis and heterogeneous fat infiltration in the pancreas as measured by gradient-echo imaging, both of which are risk factors for diabetes.
QUANTITATIVE IMAGING IN MEDICINE AND SURGERY
(2021)
Article
Hematology
G. Piatti, M. Giuditta, D. Consonni, E. Cassinerio, M. D. Cappellini
Summary: β-Thalassemia patients often have reduced exercise capacity due to muscular deconditioning and decreased cardiac inotropism. Regular physical activity may be beneficial in improving endurance and quality of life.
ANNALS OF HEMATOLOGY
(2022)
Review
Obstetrics & Gynecology
Faith A. Miller, Adalina Sacco, Anna L. David, Ashley K. Boyle
Summary: Interventions targeting inflammation show therapeutic potential for infection/inflammation-induced preterm births in animal models. However, the heterogeneity in methodology and lack of standardization remain challenges in preclinical studies.
REPRODUCTIVE SCIENCES
(2023)
Review
Obstetrics & Gynecology
Adalina Sacco, Emily F. Cornish, Neil Marlow, Anna L. David, Dino A. Giussani
Summary: According to a systematic review of human clinical literature, antenatal corticosteroids (ACS) exposure does not have long-term effects on cardiovascular function in human offspring. However, due to the limited number, heterogeneity, and mixed quality of studies, the effects on cardiac structure and other measures of cardiac function remain unclear. Further research is needed to assess central cardiac function in human offspring exposed to ACS.
BJOG-AN INTERNATIONAL JOURNAL OF OBSTETRICS AND GYNAECOLOGY
(2023)
Review
Hematology
Jed Ashman, Nyasha Mutsonziwa, Marco Romano, Shahram Kordasti, Giovanna Lombardi, Panicos Shangaris
Summary: Challenges in haematopoietic stem cell transplantation, such as low bone marrow engraftment, graft versus host disease, and the need for long-term immunosuppression, could be addressed using tissue-resident T regulatory cells (Tregs), specifically bone marrow Tregs. Controlling adverse immune response and reducing risks associated with long-term immunosuppression is crucial in clinical practice. Tregs have shown promise as a safe and effective therapy, and their potential in haematopoietic stem cell transplantation is being explored.
Article
Obstetrics & Gynecology
Megan Jeffery, Sarah Tai-MacArthur, Panicos Shangaris, Martin Duggan, Julia Spencer, Srividhya Sankaran
Summary: Foetal urological abnormalities are a complex issue affecting pregnant women, and most women choose to terminate the pregnancy after severe abnormalities are detected by prenatal diagnosis. The number of cases in England and Wales has been increasing steadily, but declined in 2019.
REPRODUCTIVE SCIENCES
(2023)
Editorial Material
Hematology
Panicos Shangaris, Sara El Hoss
Article
Engineering, Biomedical
Sophia Bano, Francisco Vasconcelos, Anna L. David, Jan Deprest, Danail Stoyanov
Summary: Fetoscopic video mosaicking can create an expanded field-of-view image of the fetoscopic intraoperative environment to support surgeons during twin-to-twin transfusion syndrome treatment. However, existing methods relying on vessel presence have limitations. We propose a vessel-guided hybrid fetoscopic mosaicking framework that combines placental vessel-based registration and deep learning-based dense matching to improve overall performance. Experimental results demonstrate the robustness of the proposed framework over state-of-the-art methods, even in vessel-free and low-textured fetoscopic views.
COMPUTER METHODS IN BIOMECHANICS AND BIOMEDICAL ENGINEERING-IMAGING AND VISUALIZATION
(2023)
Article
Medicine, General & Internal
Tania Jeyaseelan, Panicos Shangaris, Athina Efthymiou, Linzi Martin, Lisa Story, Surabhi Nanda, Neelam Gupta, Mudher Al-Adnani, Andreas Marnerides, Kypros H. H. Nicolaides, Srividhya Sankaran
Summary: This study describes a rare complication in dichorionic twin pregnancy called twin anemia polycythemia sequence, which was managed using intrauterine blood transfusion and resulted in a prolonged pregnancy of nearly two weeks.
JOURNAL OF MEDICAL CASE REPORTS
(2023)
Review
Pediatrics
Daniel Cromb, Megan Hall, Lisa Story, Panicos Shangaris, Mudher Al-Adnani, Mary A. Rutherford, Grenville F. Fox, Neelam Gupta
Summary: Placental examination is of great importance as it provides valuable clinical information related to fetal development, neonatal morbidity and mortality, and future health outcomes. The examination can identify intrinsic or secondary placental lesions that are directly associated with adverse neonatal outcomes or indicate an unfavorable intrauterine environment. This article describes the clinical value of placental examination, common pathological findings, how to interpret key findings, as well as the medico-legal and financial implications and current and future clinical considerations.
ARCHIVES OF DISEASE IN CHILDHOOD-FETAL AND NEONATAL EDITION
(2023)
Review
Endocrinology & Metabolism
Simon N. Waddington, William H. Peranteau, Ahad A. Rahim, Ashley K. Boyle, Manju A. Kurian, Paul Gissen, Jerry K. Y. Chan, Anna L. David
Summary: Fetal gene therapy was proposed in the late 1990s when gene therapy was at its peak of inflated expectations. Over the years, gene therapy matured and became a clinical and market reality. The trajectory of treatment for genetic diseases is now focused on earlier intervention, with increasing ability to diagnose genetic diseases in utero. Recent clinical trials have pointed towards the potential for fetal gene therapy. This review summarizes the history of fetal gene therapy, advancements in fetal surgery and diagnosis, and the scope of preclinical gene therapy for inherited metabolic diseases.
JOURNAL OF INHERITED METABOLIC DISEASE
(2023)
Article
Developmental Biology
Nada Mufti, Joanna Chappell, Patrick O'Brien, George Attilakos, Hassna Irzan, Magda Sokolska, Priya Narayanan, Trevor Gaunt, Paul D. Humphries, Premal Patel, Elspeth Whitby, Eric Jauniaux, J. Ciaran Hutchinson, Neil J. Sebire, David Atkinson, Giles Kendall, Sebastien Ourselin, Tom Vercauteren, Anna L. David, Andrew Melbourne
Summary: The study investigates the use of super-resolution reconstruction (SRR) MRI to improve placental assessment and predict adverse maternal outcomes. The results show that SRR imaging and paired imaging can enhance the detection of pathological MRI markers, aiding in surgical planning.
Article
Obstetrics & Gynecology
Isabelle Schiff, Panicos Shangaris, Mary Grinsted, Srividhya Sankaran
Summary: This study analyzes the trends in feticide in singleton pregnancy in England and Wales between 2012 and 2020. The prevalence of feticide fluctuated, but there was an overall decrease over the years. Intracardiac injection of potassium chloride was the most common method used for feticide. Most feticides took place at 23 weeks, followed by 22 weeks and between 20 and 21 weeks.
REPRODUCTIVE SCIENCES
(2023)
Meeting Abstract
Obstetrics & Gynecology
Hania Arain, Tina Patel, Nicoleta Mureanu, Athina Efthymiou, Giovanna Lombardi, Timothy Tree, Kypros H. Nicolaides, Panicos Shangaris
REPRODUCTIVE SCIENCES
(2023)
Meeting Abstract
Developmental Biology
Kimberley Botting-Lawford, Ruth Horler, Anthony White, Sheila Mizani, Gabrielle Crombie, Hakim-Moulay Dehbi, Owen Vaughan, Anna L. David
Article
Engineering, Biomedical
Efthymios Maneas, Andreas Hauptmann, Erwin J. Alles, Wenfeng Xia, Sacha Noimark, Anna L. David, Simon Arridge, Adrien E. Desjardins
Summary: A post-processing framework based on a convolutional neural network (CNN) is proposed to improve the spatial resolution of ultrasonic tracking images. The application of CNNs shows promising potential in enhancing the resolution of tracking images.
INTERNATIONAL JOURNAL OF COMPUTER ASSISTED RADIOLOGY AND SURGERY
(2023)
Letter
Obstetrics & Gynecology
Jenny Carter, Naomi Carlisle, Anna David, Jane Sandall, Paul Seed, Andrew Shennan, Rachel Tribe, Helena Watson
ARCHIVES OF GYNECOLOGY AND OBSTETRICS
(2023)
