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Publication Metadata only An overview of current treatment strategies for β-thalassemia(2014-01-01) Maria Domenica Cappellini; Vip Viprakasit; Ali T. Taher; Universita degli Studi di Milano; Mahidol University; American University of BeirutIntroduction: At least 40,000 people per year worldwide are born with β-thalassemia. Patients with β-thalassemia major are reliant on regular red blood cell transfusions for survival from a young age. For those with β-thalassemia intermedia... or hemoglobin (Hb) E/β-thalassemia, symptoms range from mild clinical presentation to a more severe phenotype and patients are not necessarily transfusion-dependent.Areas covered: Here, β-thalassemia treatment strategies including transfusion, splenectomy, fetalPublication Metadata only α/β-Globin mRNA ratio determination by multiplex quantitative real-time reverse transcription-polymerase chain reaction as an indicator of globin gene function(2007-12-01) Chulaporn Chaisue; Suttiphan Kitcharoen; Prapon Wilairat; Arunee Jetsrisuparb; Goonnapa Fucharoen; Supan Fucharoen; Khon Kaen University; Mahidol UniversityObjectives: Imbalance in α/β-globin chains is an important determinant of thalassemia disease severity. This study examined the relationship between α/β-globin mRNA ratio and disease severity in various thalassemia genotypes. Design and methods: α...- and β-globin mRNA contents of red blood cells of 75 α- and 32 β-thalassemia subjects (5 with β0-thalassemia/Hb E) and 14 normal controls were measured using multiplex quantitative real-time reverse transcription-polymerase chain reaction (qRT-PCR). The αPublication Metadata only Clinical and hematologic aspects of hemoglobin E β-thalassemia(2000-03-02) Suthat Fucharoen; Pranee Winichagoon; Mahidol UniversityHemoglobin E β-thalassemia is an important cause of childhood chronic disease in Southeast Asia. It is characterized by the presence of hemoglobin E and F, and the amount of hemoglobin E ranges from 35% to 75%. The patients are generally classified... as having thalassemia intermedia because they have inherited a β-thalassemia allele and hemoglobin E, which acts as a mild β+-thalassemia. However, a remarkable variability in the clinical expression, ranging from a mild form of thalassemia intermediaPublication Metadata only Problems in determining thalassemia carrier status in a program for prevention and control of severe thalassemia syndromes: A lesson from Thailand(2013-08-01) Vip Viprakasit; Chanin Limwongse; Sathein Sukpanichnant; Pornpimol Ruangvutilert; Chompunut Kanjanakorn; Waraporn Glomglao; Monchan Sirikong; Witayakarn Utto; Voravarn S. Tanphaichitr; Mahidol UniversityBackground: Prevention and control of severe β thalassemia by carrier detection and identification of couples at risk in developed countries is one of the most successful stories in modern medicine. Similar programs in developing countries... especially Southeast Asia, are more problematic because both α and β thalassemias are highly prevalent. In Thailand, there are limited data on whether we could determine, based on hematological phenotypes, the mutation severity and/or coinheritance of αPublication Metadata only Concomitant inheritance of α‐thalassemia in β°‐thalassemia/hb e disease(1985-01-01) Pranee Winichagoon; Suthat Fucharoen; David Weatherall; Prawase Wasi; Faculty of Medicine, Thammasat University; John Radcliffe Hospital; Mahidol UniversityConcomitant inheritance of α‐thalassemia in patients with β°‐thalassemia/hemoglobin (Hb) E disease was detected by restriction endonuclease DNA mapping. Among 42 patients with β°‐thalassemia/Hb E disease, seven were found to have an α‐thalassemia‐2... detectable α‐thalassemia had hemoglobin levels both higher and lower than 7.4 g/dI. The latter attended the clinic regularly, the former did occasionally. These findings suggest that concomitant inheritance of α‐thalassemia can alleviate the severity of βPublication Metadata only Iron metabolism in heterozygotes for hemoglobin E (HbE), α-thalassemia 1, or β-thalassemia and in compound heterozygotes for HbE/β-thalassemia(2008-10-01) Michael B. Zimmermann; Suthat Fucharoen; Pattanee Winichagoon; Pornpan Sirankapracha; Christophe Zeder; Sueppong Gowachirapant; Kunchit Judprasong; Toshihiko Tanno; Jeffery L. Miller; Richard F. Hurrell; ETH Zurich; Mahidol University; The Institute of Science and Technology for Research and Development, Mahidol University; National Institute of Diabetes and Digestive and Kidney Diseasesiron absorption or utilization (or both) in women heterozygous for β-thalassemia, α-thalassemia 1, orhemoglobinE (HbE) differed from that in control subjects and compound HbE/β-thalassemia heterozygotes. Design: In Thai women (n = 103), red blood cell... utilization was measured by the infusion of (58Fe) Fe citrate. Results: Iron utilization was ≈15% lower in α-thalassemia 1 or β-thalassemia heterozygotes than in controls. When corrected for differences in serum ferritin, absorption was significantly higherPublication Metadata only Molecular analysis of β-thalassemia in South Vietnam(2002-10-01) M. L. Saovaros Svasti; Tran Minh Hieu; Thongperm Munkongdee; Pranee Winichagoon; Tran Van Be; Tran Van Binh; Suthat Fucharoen; The Institute of Science and Technology for Research and Development, Mahidol University; Blood Transfusion and Hematology HospitalIn Vietnam, the carrier rate for β-thalassemia varies from 1.5% to 25% depending on the ethnic groups of the population. The molecular basis of β-thalassemia in South Vietnam was studied in 50 unrelated β-thalassemia patients. Of these, 31 had β...-thalassemia/Hb E, 18 were homozygous for β-thalassemia, and 1 carried the β-thalassemia trait. The majority of the patients were Kinh, four were Chinese, and two were Kinh-Chinese. All had severe anemia and received blood transfusions regularly, every 1-3Item Metadata only Up-regulation of microRNA 101-3p during erythropoiesis in β-thalassemia/HbE(2023-11-01) Phannasil P.; Sukhuma C.; Nauphar D.; Nuamsee K.; Svasti S.; Mahidol UniversityIneffective erythropoiesis is the main cause of anemia in β-thalassemia. The crucial hallmark of ineffective erythropoiesis is the high proliferation of erythroblast. microRNA (miR/miRNA) involves several biological processes, including cell... proliferation and erythropoiesis. miR-101 was widely studied and associated with proliferation in several types of cancer. However, the miR-101-3p has not been studied in β-thalassemia/HbE. Therefore, this study aims to investigate the expression of miR-101-3pPublication Metadata only Implication of globin gene expression, hemoglobin F and hemoglobin E levels on β-thalassemia/Hb E disease severity(2014-01-01) Suwimol Siriworadechkul; Sumalee Jindadamrongwech; Suporn Chuncharunee; Saranya Aupparakkitanon; Mahidol University© 2014 by the Association of Clinical Scientists, Inc. One of the factors affecting the degree of severity in β-thalassemia disease is the presence of unmatched a-hemoglobin chains. Thus, the expression levels of globin genes in reticulocytes of β...-thalassemia subjects were measured using quantitative RT-PCR, demonstrating that a/β globin mRNA ratio, as well as levels of ?-globin mRNA and Hb F, increased with progressing degree of β globin synthesis defect. The levels of γ-globin mRNA and Hb F couldPublication Metadata only Molecular basis of β-thalassemia in the Maldives(1998-01-01) H. Furuumi; N. Firdous; T. Inoue; H. Ohta; P. Winichagoon; S. Fucharoen; Y. Fukumaki; Kyushu University; Society for Health Education; Mahidol UniversityWe have systematically analyzed β-thalassemia genes using polymerase chain reaction-related techniques, dot-blot hybridization with oligonucleotide probes, allele specific-polymerase chain reaction, and sequencing of amplified DNA fragments from 41... unrelated patients, including 37 β-thalassemia homozygotes, three with β-thalassemia/Hb E, and one with β-thalassemia/Hb S. Four different β-thalassemia mutations were detected in 78 alleles. These are the IVS-I-5 (G→C), codon 30 (AGG→ACG) [also indicated