objective: this study was designed to investigate rbc indices and hba2 levels in parents of major beta-thalassemia patients to detect possible silent beta- thalassemia carriers and examine its potential impact on the premarital genetic counseling. materials and methods: this cross sectional study was performed at children medical center from 2004 to 2006. after genetic counseling and getting in...

background beta-thalassemia is the most common hematology disease in human and leptin is one of the hormone that produce by adiposities cells. the purpose of this study was to investigate the relationship between serum leptin level and thyroid hormones in children with major beta-thalassemia. materials and methods this descriptive-cross sectional study was performed on 90 children aged 6-16 yea...

BACKGROUND We aimed to investigate the frequency of celiac disease in children with β-thalassemia major (B-TM) in Shiraz, southern Iran. MATERIALS AND METHODS In this study, the prevalence of celiac disease in children with B-TM was evaluated. Children with B-TM were screened for celiac disease by ant-tissue transglutaminase (anti-tTG) IgA antibody, IgA level and anti-tTG IgG. A total of 1500...

Hemoglobin E/β-thalassemia (HbE/β-thalassemia) is the most important type of thalassemia in northeastern Thailand. Serious complications of the disease are associated with iron overload and the consequences of oxidative damage to various organs, especially the cardiovascular system. Endothelial dysfunction is an important predictor for the long-term outcome of the disease. In this study, 19 pat...

By PARK S. GERALD AND Louis K. DIAMOND T HIS REPORT details our experiences with a new hemoglobinopathy which we have named the “Lepore trait.” This condition was discovered during a recent survey of the relatives of thalassemia major children.1 The mother of a child with clinical thalassemia major was found to possess a new abnormal hemoglobin (which we have named the Lepore hemoglobin) demons...

The globally widespread single-gene disorders β-thalassemia and sickle cell anemia (SCA) can only be cured by allogeneic hematopoietic stem cell transplantation (HSCT). HSCT treatment of thalassemia has substantially improved over the last two decades, with advancements in preventive strategies, control of transplant-related complications, and preparative regimens. A risk class-based transplant...

OBJECTIVES The term beta-thalassemia includes all those hereditary disturbances of the hemoglobin (Hb), transferred trough a recessive autosomal mechanism, due to a reduced or else defective synthesis of beta globin sequences. The aim of this paper is to highlight as sometimes the only biochemical diagnosis is not exhaustive and a molecular diagnostic widening is necessary to detect the genetic...

Abstract Background Beta-thalassemia major patients are at increased risk of complications including endocrinopathies and bone disease due to iron overload. So, this study aimed assess the growth parameters, serum levels 25-OH-vitamin D, calcium, phosphorous in children with beta-thalassemia major. This was a case-control that included 55 compared 30 sex- age-matched healthy served as control g...

Coexistence of thalassemia, hemoglobinopathies and malaria has interested geneticists over many decades. The present study represents such a population from the eastern Indian state of Orissa. Children and their siblings (n=38) were genotyped for β-thalassemia mutations and genotype-phenotype correlation was determined. The major genotype was IVS 1.5 mutation: 26% homozygous (n=10) and 37% (n=1...

OBJECTIVE To compare the intelligence quotient (IQ) of patients with thalassemia major (TM) to that of normal children. METHODS We conducted the study in April and May 2002 on 294 homozygote beta-thalassemia patients, (157 male and 137 female, mean age of 13.2 years; range, 9-18 years). These 294 patients were randomly selected from the 984 TM patients who routinely refer to Shiraz Cooley's M...