Thalassemias are a group of inherited disorders that are characterized by decreased production of the alpha or beta globin chains. The amount of the produced normal hemoglobin within the red blood cells correlates with the severity of the symptoms. Fetuses with alpha-thalassemia major usually die. Children with beta-thalassemia major are usually dependent on repeated blood transfusions. Splenec...

Thalassemia is the most common single gene disorder around the world (1, 2). This disease is a type of chronic, microcytic and inherited anemia associated with defect in hemoglobin synthesis and reduction in life span of red blood cells (3). Thalassemia is distributed in Mediterranean, Middle East, Arabian Peninsula, Turkey, Iran, India, Burma and Southeast Asia (1). There are two types of thal...

Hb E/b thalassemia is the most common b thalassemia syndrome in Asia-Pacific due to a high prevalent of Hb E and b thalassemia genes. Management of this condition can be cumbersome due to its clinical heterogeneity and various disease severity ranging from severe end in which patients are transfusion dependent thalassemia (TD) similar to that of b thalassemia major (TM) to moderate and mild sev...

Background: Thalassemia is an inherited disorder of hemoglobin (Hb) synthesis. Treatment thalassemia has not yet reached the level cure. Globally, management symptomatic in form lifelong blood transfusions. Data on children with major Indonesia been widely published. However, various studies clearly showed growth and development disorders behavior that greatly affected quality life thalassemia....

The clinical presentation of β-thalassemia intermedia phenotypes are influenced by many factors. The persistence of fetal hemoglobin and several polymorphisms located in the promoters of γ- and β-globin genes are some of them. The aim of this study was to evaluate the combined effect of the -158 Gγ (C→T) polymorphism and of the (AT)x(T)y configuration, as well as their eventual association with...

BACKGROUND Alpha-thalassemia is a common hereditary disease in Taiwan. Affected patients always carry a heavy burden of morbidity and early death. Prenatal diagnosis has reduced the disease burden on families and the health care system. This study evaluated a new non-radioactive Southern blotting hybridization method for prenatal diagnosis of this disease. METHODS Seventy two chorionic villi ...

OBJECTIVE To review the reproductive health knowledge, attitudes, and behaviors of persons with homozygous beta-thalassemia. DESIGN Case-control study. SETTING Patients treated at a tertiary hospital and community-based healthy controls. PARTICIPANT(S) One hundred and thirty persons, 16 years of age or older, who had homozygous beta-thalassemia and were attending hospital for regular bloo...

The ratio of hematocrit (Hct) to hemoglobin (Hb) in the people with normal red blood cell (RBC) morphology is generally three to one. We studied Hct/Hb ratios among patients with alpha-thalassemias (Hb H, H-CS, AEBart, AEBart-CS, EFBart and EFBart-CS diseases) diagnosed by high performance liquid chromatography, and compared them with normal subjects and with patients having anemia due to chron...

INTRODUCTION Thalassemia is a chronic blood disease, which imposes adverse effects on patients and their families. Parents of such patients, given that they had the thalassemia trait, hold themselves responsible for their children's disease in addition to other difficulties, bear the burden of guilt and hopelessness and worry about the health and future of their children. This study aimed to ex...

BACKGROUND Thalassemia syndromes are highly prevalent in Southeast Asia. In Thailand, high performance liquid chromatography (HPLC) is the most common technique routinely performed in diagnosis of thalassemia and hemoglobinopathies, while isoelectric focusing (IEF) is rarely employed. We compared the diagnostic utility of IEF and HPLC in neonatal screening for thalassemia and non-sickling hemog...