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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
Thalassemia poses clear systemic and oral health problems. Clinicians must be aware of various dental treatment needs in these patients. This study was undertaken to assess body mass index (BMI), dental age, salivary alkaline phosphatase levels, malocclusion, and treatment needs in 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
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
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
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
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
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
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
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
In Gaza Strip, Palestine, β-thalassemia is a major public health problem where more than 300 β-thalassemia major (βTM) patients are currently being managed at governmental hospitals. We set up to evaluate the hematological and biochemical aspects of our βTM patients at the Gaza 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,
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
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