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beta-thalassemia/phosphatase
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The red cell acid phosphatase polymorphism in Greek b-thalassemia patients.
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Red cell acid phosphatase polymorphism was studied by starch gel electrophoresis in 70 b-thalassemia patients and in 310 healthy Greeks. Our results gave the following gene frequencies; b-thalassemia patients: pa 0.321, pb 0.643, pc 0.036; healthy Greeks: pa 0.302, p b 0.653, pc 0.045. No
Comparative Evaluation of BMI, Dental Age, Salivary Alkaline Phosphatase Levels, and Oral Health Status in Children with β Thalassemia Major.
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Kinetic alterations of the red cell membrane phosphatase in alpha- and beta-thalassemia.
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We studied the red cell membrane neutral phosphatase, which is part of the Na+K+ ATPase, in several types of oxidative hemolytic anemias. We used an artificial substrate, the p-nitrophenylphosphate. In controls and in patients heterozygous for various unstable hemoglobins (Hb Hope, Hb Köln, or Hb
Red cell acid phosphatase activity in carriers of beta-thalassemia trait and glucose-6-phosphate dehydrogenase deficiency.
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Association of Serum Vitamin D Level with its Receptor Gene Polymorphism BSML in Beta Thalassemia Major Patients from East India.
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Background Vitamin D deficiency is commonly identified in beta thalassemia major patients, related to iron accumulation.Vitamin D mediates its action upon binding to vitamin D receptor (VDR), a classical nuclear receptor. Several single nucleotide gene polymorphisms has been identified in VDR gene
Bone minerals in beta-thalassemia minor.
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Homozygous beta-thalassemia is a severe hereditary disorder associated with osteopenia. Recently it was suggested that thalassemia minor may be a risk factor for osteoporosis. The purpose of the present study was to investigate this suggestion. Bone mineral status was assessed in 22 premenopausal
Short stature in beta-thalassemia minor subjects.
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BACKGROUND
Numerous disturbances in growth and development have been observed and demonstrated in homozygote beta-thalassemia patients. However, short stature in thalassemia minor subjects, who have a minor defect in hemoglobin chain synthesis, has not yet been studied.
METHODS
In this
[A molecular abnormality of the erythrocyte membrane in beta-thalassemia].
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We present a new abnormality of the red cell membrane observed in the course of a study on beta-thalassemia. In a child suffering from a beta 0-thalassemia major, the neutral membrane bound phosphatase, instead of having Michaelis-Menten kinetics, displayed a biphasic kinetics consistent with
Cytochemical evaluation of neutrophil components in beta thalassemia hemoglobin E.
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The mean +/- S.D. of LAP score in neutrophil of the normal control, non splenectomised patients and splenectomised patients were 116 +/- 78, 94 +/- 48 and 37 +/- 23 Kaplow units, respectively. The significant reduction in the leukocyte alkaline phosphatase level (P less than 0.05) was found in beta
Recombinant erythropoietin trial in children with transfusion-dependent homozygous beta-thalassemia.
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Augmentation of gamma-gene synthesis by using recombinant human erythropoietin (r-Hu-EPO) represents a new approach to the therapy of beta-thalassemia. A prospective study was conducted in 26 transfusion-dependent beta-thalassemia major patients. r-Hu-EPO (Eprex/Cilag, Switzerland) was given to the
Hematological and biochemical status of Beta-thalassemia major patients in Bangladesh: A comparative analysis.
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BACKGROUND
Thalassemia is one of the most common hereditary disorders and Beta-thalassemia major is its severe form. The present study is concerned with the analysis of liver function, thyroid function and estimation of critical serum ions as well as hematological characteristics in beta-thalassemia
Hematological and biochemical evaluation of β-thalassemia major (βTM) patients in Gaza Strip: A cross-sectional study.
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A high frequency of Gilbert syndrome (UGT1A1*28/*28) and associated hyperbilirubinemia but not cholelithiasis in adolescent and adult north Indian patients with transfusion-dependent β-thalassemia
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Hyperbilirubinemia and pigment gallstones are frequent complications in transfusion-dependent β-thalassemia (TDβT) patients. Bilirubin production and clearance are determined by genetic as well as environmental variables like ineffective erythropoiesis, hemolysis, infection-induced hepatic injury,
Effects of deferoximine on chondrocyte alkaline phosphatase activity: proxidant role of deferoximine in thalassemia.
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The homozygous form of beta-thalassemia, the most common single gene disorder, is treated by red cell transfusion therapy. Following transfusion, the chelator, deferoximine, is administered to patients to remove excess iron. However, when this drug is given to young children, metaphyseal dysplasia
CTD small phosphatase like 2 (CTDSPL2) can increase ε- and γ-globin gene expression in K562 cells and CD34+ cells derived from umbilical cord blood.
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BACKGROUND
A potential strategy for treatment of sickle cell disease (SCD) and β-thalassemia in adults is reactivation of the ε- and γ-globin genes in the adult. We aimed to identify trans-activators of ε- and γ-globin expression and provide new candidate targets for effective treatment of sickle
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