... underwent splenectomy. It was determined that significantly more patients with hemoglobin H (HbH) disease, a form of α-thalassemia, have undergone splenectomy than β-thalassemia patients (20% vs. 7%). Further, HbH disease patients were diagnosed at a significantly older age than β-thalassemia patients...

...Thunwarat Suriyun; Woratree Kaewsakulthong; Pinyaphat Khamphikham; Sukanya Chumchuen; Suradej Hongeng; Suthat Fucharoen; Orapan Sripichai Introduction: β-Thalassemia/hemoglobin E represents one-half of all the clinically severe β-thalassemias worldwide. Despite similar genetic backgrounds, patients...

...Wenguang Jia; Weidong Wang; Hengying Zhu; Ping Chen β-Thalassemia is a genetic disease characterized by the defective synthesis of the hemoglobin tetramer β-globin chains. So far, a number of mutations have been identified and associated with this genetic disease. A high incidence of thalassemia...

...Konstantinos Skandalis; Christoforos Vlachos; Xanthi Pliakou; Georgios Gaitanis; Eleni Kapsali; Ioannis D. Bassukas Disturbed iron homeostasis characterizes β-thalassemia and increases its morbidity. Our aim was to retrospectively associate β-thalassemia disease characteristics with treatment...

... deferiprone (DFP) combined with 40 mg/kg/dose s.c. desferrioxamine (DFO) twice weekly were given to transfusion-dependent β-thalassemia children. Results: Enrolled patients (9 with β-thalassemia major and 33 with β-thalassemia hemoglobin E), ranging from 3 to 18 years in age, were divided into 3 groups; group...

...Ali Taher; Abdullah Al Jefri; Mohsen Saleh Elalfy; Kusai Al Zir; Shahina Daar; Diana Rofail; Jean François Baladi; Dany Habr; Ulrike Kriemler-Krahn; Amal El-Beshlawy Patient-reported outcomes of once-daily oral deferasirox (Exjade®) in iron-overloaded patients with β-thalassemia not achieving...

...C. Camaschella; M.A. Ciocca-Vasino; A. Guerrasio; G. Balegno; E. Barberis; D. Delponte; G. Saglio Hematological data, biosynthetic studies and γ-chain structure of three heterozygotes for HPFH Greek type and of two heterozygotes for both HPFH and β- thalassemia are reported. In the HPFH...

...R. Galanello; M.A. Melis; P. Muroni; A. Cao Hb A 2 was assayed by means of DE-52 microchromatography in hemolysates from 285 normal subjects and 223 β -thalassemia heterozygotes. No overlap was found between both groups. Comparable results were observed analyzing whole blood samples collected...

...U. Testa; A. Dubart; N. Hinard; F. Galacteros; W. Vainchenker; P. Rouyer-Fessard; Y. Beuzard; J. Rosa Hemoglobin synthesis in a 34-year-old man of Laotian ancestry with Hb E/β°- thalassemia was studied. Hemoglobin electrophoresis exhibited only Hb F and Hb E. Reticulocytes and bone marrow cells...

... heterozygotes for Hb Hasharon and β -Thalassemia from 43 apparently unrelated families living in the province of Rovigo (northern Italy). Biosynthetic studies are also reported. The data strongly suggest the presence of an α -thalassemia-2 determinant closely linked to the α Hasharon -chain locus. Selective...

..., instructions or products referred to in the content or advertisements. Hemolysis Polyamines Superoxide dismutase β -Thalassemia Acta haemat. 63: 226-229 (1980) Effect of Polyamines on Autohemolysis: Studies on Normal and Thalassémie Children A. Vanella, V. Rizza, S. Li Void, R. Pinturo, S...

... between the proportion of Hb F and Hb A 2 was found over an 8-year period, as well as in populations of red blood cells fractionated by density gradient. She exhibited two genetic defects, the Swiss type of heterocellular hereditary persistence of fetal hemoglobin and a β -thalassemia trait, which were...

...) deficiency, β-thalassemia (β-thal) heterozygotes and in a boy carrying both mutations. The results obtained confirmed a reduced concentration of NADPH in G6PD deficiency and showed that with the combination of both diseases, the red blood cell contained practically undetectable levels of NADPH. Assays...

..., instructions or products referred to in the content or advertisements. Human globin genes Polymorphic Taq I site β-Thalassemia Original Papers Actahaemat. 72: 217-220(1984) © 1984 S. Karger AG. Basel 0001-5792/84/0724-0217 $ 2.75/0 Non-Random Association of the Polymorphic Taq I Restriction Site...

...) was reached, thus interpreting its increase and the absence of Hb A, as an association with β °-thalassemia, producing a mild hemolytic anemia. Evidence was obtained that Hb Hofu is a mild unstable hemoglobin variant. 21 06 1984 11 09 1984 25 2 2009 Actahaemat. 73: 51-54(1985) © 1985 S...

... advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements. Hepatitis B β-Thalassemia Acta Haematol...

... cells by repeated blood transfusions. 25 07 1988 13 08 1989 20 2 2009 Complement Immune status Immunoglobulins Lymphocytes Phagocytes β-Thalassemia Acta Haematol 1990;83:76-81 © 1990 S. Karger AG. Basel 0001 -5792/90/0832-0076 $ 2.75/0 Immunologie Evaluation of Children...

...Ada Goldfarb; D. Grisaru; Z. Gimmon; E. Okon; P. Lebensart; E.A. Rachmilewitz The records of 48 patients with homozygous β -thalassemia were reviewed for evidence of cholelithiasis by abdominal ultrasonography or plain abdominal X-ray. The presence of cholelithiasis was reported in 25 patients (52...

.... Erythrocyte Pyrimidine 5′-nucleotidase β-Thalassemia Thiol enzyme Acta Haemat 1989;82:69-74 © 1989 S. Karger AG, Basel 0001 -5792/89/0822-0069 $ 2.75/0 Pyrimidine 5 '-Nucleotidase Acquired Deficiency in (3-Thalassemia: Involvement of Enzyme-SH Groups in the Inactivation Process O. Davidd, M.G. Votaa...

...Joseph Michaeli; Moshe Mittelman; Dan Grisaru; Eliezer A. Rachmilewitz Thromboembolic complications in β-thalassemia major have seldom been reported and their association with risk factors such as left ventricular failure and postsplenectomy thrombocytosis has remained speculative. In this report...