introduction: beta thalassemia is the most common inherited bloody disorder, affecting synthesis of the beta globin chain of hemoglobin. the type of β-thalassemia mutation affects on the β-globin chain synthesis that appears as β ° ، β + and β ++ -thalassemia. the presence of xmni polymorphic site at the 5 َ region of the g γ-globin gene affects on the rate of g γ chain synthesis and in some con...

OBJECTIVES The aim of this study was to investigate the geographic distribution of β-globin gene mutations in different ethnic groups in Yunnan province. METHODS From 2004 to 2014, 1,441 subjects with hemoglobin disorders, identified by PCR-reverse dot blot and DNA sequencing, were studied according to ethnicity and geographic origin. Haplotypes were examined among 41 unrelated thalassemia ch...

The clinical approach to thalassemia and hemoglobinopathies, specifically Sickle Cell Disease (SCD), based on transfusions, iron chelation and bone marrow transplantation has ameliorated their prognosis. Nevertheless, infections still may cause serious complications in these patients. The susceptibility to infections in thalassemia and SCD arises both from a large spectrum of immunological abno...

background with modern medical management, thalassemia major is now extending into adulthood and it is expected to have a negative impact on the quality of life (qol) of the patients. the aim of this study was to evaluate quality of life in patients with thalassemia major. materials and methods this is an analytic case control study. two hundred and fifty patients and 51 participants as control...

BACKGROUND Prevention of myocardial siderosis is a key step to reduce rate of mortality in thalassemic patients. Our objective was to study association between echocardiography parameters and serum ferritin level in patients with major thalassemia. MATERIALS AND METHODS Sixty-six patients with major thalassemia were studied in Amir Kabir hospital, Arak, Iran. Serum ferritin levels were measur...

Cardiac iron overload causes most deaths in beta-thalassemia major. The efficacy of deferasirox in reducing or preventing cardiac iron overload was assessed in 192 patients with beta-thalassemia in a 1-year prospective, multicenter study. The cardiac iron reduction arm (n = 114) included patients with magnetic resonance myocardial T2* from 5 to 20 ms (indicating cardiac siderosis), left ventric...

Few literatures have elaborated on the clinical characteristics of children with thalassemia from low-prevalence areas. A retrospective analysis was conducted on children genetically confirmed with thalassemia at Seoul National University Children's Hospital in Korea. Nine children (1α thalassemia trait, 6β thalassemia minor, 2β thalassemia intermedia) were diagnosed with thalassemia at median ...

In Ontario, Canada, beta-thalassemia is easily detected through measurement of hemoglobin A2, but most laboratories do not do exhaustive DNA investigations for alpha-thalassemia. Therefore, the prevalence of thalassemia in microcytic samples for hemoglobinopathy investigation in Ontario is unknown. To address this, we performed a prospective cohort study in which samples referred for hemoglobin...

background: the advances in treatment regimes for thalassemic patients have increased the survival among them therefore osteoporosis has emerged as an important cause of morbidity. the aim of this study was to determine the prevalence of osteoporosis and osteopenia in patients with thalassemia from zafar adult thalassemia clinic, tehran, iran. patients and methods: in this cross sectional inves...

OBJECTIVE Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The objective of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy. METHODS During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes melli...