The thalassemias are a heterogeneous group of disorders characterized by accumulation either of unmatched alpha or beta globin chains. These in turn cause the intramedullary and peripheral hemolysis that leads to varying anemia. A partial explanation for the hemolysis came our of our studies on material properties that showed that beta-thalassemia (beta-thal) intermedia ghosts were very rigid b...

Abstract   Introduction: Hemoglobin Constant Spring (Hb CS),  a abnormal Hb characterized by elongated α-globin chain resulting from mutations of the termination codon in the α2 - globin gene , is the most common nondelitional  α-thalassemic mutation and is an important cause of HbH like disease in Southeast Asia. Case Report: A 9- years-old female with immune hemolytic anemia and splenomegally...

BACKGROUND AND OBJECTIVE After the first National Census of transfusion-dependent thalassemics (1984), in 1992 the Italian Association of Pediatric Hematology and Oncology and the National Health Institute organized its 3rd edition. Here, results concerning Latium are presented and discussed. DESIGN AND METHODS Data for Latium, as in the rest of Italy, were collected by a single reference cen...

One hundred seven adult patients with thalassemia aged from 17 through 35 years and transplanted from HLA-identical siblings between November 1988 and September 1996 were evaluated on December 31, 1997. The outcome experience of 20 consecutive patients transplanted between November 13, 1988 and January 10, 1991 and reported in September 1992 is updated after 5 additional years. The experience o...

Dengue hemorrhagic fever (DHF) causing by dengue viral infection is endemic in Thailand and Southeast Asian countries where thalassemias are prevalent. Thalassemic patients are also at risk to acquire dengue viral infections and to develop DHF. However, they can have different clinical manifestations and complications as well as more severity than general population requiring special awareness ...

OBJECTIVE Thalassemia is one of the most common genetic disorders worldwide. Cap +1 mutation which causes 'silent beta thalassemia' is present around all ethnic groups of Pakistan. This study was designed to detect the frequency of Cap+1 mutation in Pakistani Population. METHODS Molecular genetic for Cap+1 beta thalassemic mutation was done by extracting DNA from whole blood by using Genomic ...

Abstract Background Beta-thalassemia is a common hereditary hemoglobinopathy, which is a reason of microcytic hypochromic anemia. Patients with major thalassemia require multiple blood transfusions. This study evaluated growth in thalassemic patient and relationship with ferritin level. Materials and Methods This is a cross sectional study on seventy patients (36 boys, 34girls) with transfu...

INDIAN PEDIATRICS 1072 VOLUME 41OCTOBER 17, 2004 In developing countries including Pakistan, due to lack of resources for universal and effective screening of blood donors for antibodies to hepatitis C virus (anti-HCV), blood transfusion is still a major source of HCV transmission. Therefore, in addition to other potential recipients of blood transfusion, the thalassemic patients on a long-term...

Preclinical and clinical studies demonstrate the feasibility of treating β-thalassemia and Sickle Cell Disease (SCD) by lentiviral-mediated transfer of the human β-globin gene. However, previous studies have not addressed whether the ability of lentiviral vectors to increase hemoglobin synthesis might vary in different patients.We generated lentiviral vectors carrying the human β-globin gene wi...

Thalassaemia consists one of the most common chronic and genetic hematological disorder, globally. Moreover, is a serious life-limiting and potentially life-threatening disease that causes substantial disruption in all dimensions of life. As more effective management and even cure of thalassemia become possible, attention is drawn to the evaluation of depression that frequently ollows suffering...