Approximately 5.1% of the US population has diabetes mellitus, and hemoglobin (Hb) A1c levels are routinely measured to monitor long-term glycemic control in these patients. Many laboratories use ion exchange chromatography for such measurements, and the presence of hemoglobin variants and hemoglobinopathies often results in abnormal peaks on the chromatogram. The goal of this study was to eval...

Plasmodium falciparum malaria kills over 500,000 children every year and has been a scourge of humans for millennia. Owing to the co-evolution of humans and P. falciparum parasites, the human genome is imprinted with polymorphisms that not only confer innate resistance to falciparum malaria, but also cause hemoglobinopathies. These genetic traits--including hemoglobin S (HbS), hemoglobin C (HbC...

objective: the use of fetal hemoglobin (hbf) inducer drugs is considered as a novel approach in treatment of β-hemoglobinopathies, especially β- thalassemia and sickle cell disease. hbf inducers including hydroxyurea, histone deacetylase (hdac) inhibitor agents such as sodium butyrate, azacitidine, decitabine and new immunomodulator drugs like pomalidomide, lenalidomide and thalidomide can redu...

BACKGROUND Hemoglobinopathies are among the most common inherited diseases around the world. They have become much more common recently in northern and central Europe, including Germany, due to immigration. METHOD Selective review of the literature with consideration of national guidelines. RESULTS The hemoglobinopathies encompass all genetic diseases of hemoglobin. They fall into two main ...

PURPOSE Our aim was to identify couples at risk of having a homozygous or compound heterozygous child with a severe hemoglobinopathy by antenatal screening and prenatal diagnosis in Surat, South Gujarat. METHOD Pregnant women were screened for hemoglobinopathies by means of red cell indices, the solubility test, cellulose acetate electrophoresis tests, and confirmation by HPLC. Husbands of th...

OBJECTIVE To determine the geographical distribution of hemoglobinopathies in the State of Pernambuco, to characterize the children with these diseases and to describe factors associated with their follow-up at the referral center during the period from 2003 to 2010. METHODS A retrospective, cross-sectional, descriptive study was carried out of 275 medical records from a total of 302 children...

Hemoglobinopathies constitute a heterogeneous group of hereditary hemoglobin disorders characterized by either reduced (thalassemias) or defective (sickle cell disease) globin chain synthesis that results in chronic hemolytic anemia. They represent the most common monogenetic disorders in humans, and although traditionally confined to specific geographic areas and populations (the Mediterranean...

I am honored to invite you to enjoy reading this special issue of The Scientific World Journal. One review article is “Ineffective erythropoiesis in β-thalassemia.”The authors concluded that this ineffective erythropoiesis could be the conjunction of several mechanisms of which the final consequence is the arrest of maturation and increased apoptosis of erythroblasts during their terminal diffe...

214 Turkey is settled at a crossroads of the earth’s continents, so migrations throughout the world, especially between Asia, Europe and Africa, have affected Turkey in numerous ways, including in the evolution of hemoglobin disorders. In the middle of the 1940s, many thalassemic patients and abnormal hemoglobins began to be diagnosed at Istanbul University, and the pioneering works of Prof. Ak...

Abstract Background Iron overload is the main transfusion related side effects in patients with transfusion dependent hemoglobinopathies. Severe iron deposition in tissues leads to organ dysfunction. Many organs can be affected such as heart, liver, and endocrine organs. Cardiac failure and liver fibrosis are the consequent of Iron overload in transfusion dependent hemoglobinopathy. Magne...