Comparative Analysis of Erythroferrone and Hepcidin as Emerging Biomarkers of Iron Homeostasis in Patients with β-Thalassemia Major: A Case-Control Study from Pakistan [0.03%]
巴基斯坦β-地中海贫血患者中铁稳态新兴生物标志物 eryroferrone 和 hepcidin 的比较分析:一项病例对照研究
Sadia Babar,Muhammad Younus Jamal Siddiqi,Muhammad Rizwan et al.
Sadia Babar et al.
Iron dysregulation in β-thalassemia major arises from a cycle of ineffective erythropoiesis, chronic transfusions, and suppressed hepcidin activity, leading to progressive iron overload and multi-organ toxicity. Erythroferrone (ERFE) plays...
Muhammad Saboor,Maryam Jasem Alblooshi,Alreem Adel Alkaabi et al.
Muhammad Saboor et al.
β-Thalassemia is a prevalent inherited disorder of β-globin chains. The clustered regularly interspaced short palindromic repeats (CRISPR) genome editing system has emerged as a potential curative strategy. We conducted a bibliometric ana...
Neurological Complications in Sickle Cell Disease: Clinical, Analytical and Radiological Correlations in a Multicentre Cohort [0.03%]
sickle细胞疾病中的神经并发症:多中心队列的临床、分析和影像学相关性
Ana Gómez-Martínez,Marta Moreno Carbonell,Sergio Pinzón Mariño et al.
Ana Gómez-Martínez et al.
Sickle cell disease is a structural hemoglobinopathy with high global prevalence, especially in Africa. Neurological complications affect a high percentage of patients. An observational, retrospective, multicenter study was conducted in Ara...
Matched Unrelated Donor (MUD) Transplants Show Promising Outcomes in Thalassemia Patients Aged ≤ 12 Years [0.03%]
匹配无关供者(MUD)移植在≤12岁地中海贫血患者中的临床效果良好
Mayank Soni,Reema Singh,Sujay Rainchwar et al.
Mayank Soni et al.
Matched unrelated donor (MUD) hematopoietic stem cell transplantation (HSCT) is a viable curative option for children with transfusion-dependent thalassemia major lacking a matched sibling donor. This study aimed to evaluate outcomes of MUD...
First Case of Hb City of Hope (HBB: c.208G > A) in Andalusia. A Molecular Update of β-Thalassemia in Southwestern Spain (Huelva Province) [0.03%]
西班牙安达卢西亚省第一例城市希望血红蛋白(HBB:c.208G>A)病例及其西南部(胡埃拉省)β-地中海贫血的分子更新报告
Candela L Hernández,Sergio Aguado,Luis J Sánchez-Martínez et al.
Candela L Hernández et al.
Beta-thalassemia, caused by mutations in the β-globin gene, is an important disease for both basic and translational research. This hemoglobinopathy is molecularly and clinically heterogeneous, and its high prevalence in certain geographic...
Pyruvate Kinase Activators for Sickle Cell Disease: An Exploratory Systematic Review and Meta-Analysis [0.03%]
丙酮酸激酶激活剂治疗镰状细胞病:探索性系统评价和meta分析
Henrique Guimaraes Barbosa Coelho,Andre Felipe Pastick de Hollanda Oliveira,Vítor Lourival De Sousa Silva et al.
Henrique Guimaraes Barbosa Coelho et al.
Pyruvate kinase (PK) activators enhance glycolytic flux, increasing ATP production and lowering red blood cell (RBC) 2,3-diphosphoglycerate (2,3-DPG), thereby improving oxygen affinity and potentially reducing hemoglobin S polymerization in...
Treatment Status and Quality-of-Life Assessment of Transfusion-Dependent Thalassemia Managed with Standard-of-Care or Hematopoietic Stem Cell Transplantation in Southern Jiangxi, China: A Cross-Sectional Study [0.03%]
中国江西南部输血依赖型地中海贫血患者标准治疗与造血干细胞移植的治疗状况及生存质量评估:横断面研究
Ping-Fang Liu,Shan Wen,Qi Xiao et al.
Ping-Fang Liu et al.
This study aimed to evaluate health-related quality of life (HRQoL) and its determinants in patients with transfusion-dependent thalassemia (TDT) to inform clinical decision-making and patient-centered outcomes. A cohort of 111 patients age...
Maya Mohan,Swathi V,Pooja Aggarwal et al.
Maya Mohan et al.
Pain is the defining factor of sickle cell disease (SCD), an inherited blood disorder. A cross-sectional study on the prevalence of the neuropathic component of pain in the Indian tribal population was conducted from August 2024 to June 202...
Duran Canatan,Emel Altunsoy
Duran Canatan
Thalassemia syndromes result from a significant reduction in the synthesis of globin chains (α, β, γ, δ). An imbalance between α- and non-α-globin chains underlies β-thalassemia. α-Globin tetramers accumulate and precipitate in eryt...
Vitamin B12 Deficiency Does Not Induce Homocysteine Increase in Transfusion-Dependent β-Thalassemia Major Patients Receiving Folic Acid Supplementation [0.03%]
叶酸补充的输血依赖性β-地中海贫血患者不会因维生素B12缺乏而导致同型半胱氨酸水平升高
Arash Arya,Saeid Jokar
Arash Arya
Vitamin B12 deficiency is a frequent but underdiagnosed complication in patients with transfusion-dependent β-thalassemia. Folic acid supplementation, commonly prescribed in this population, may mask hematological signs and alter homocyste...