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Despite recent advances in understanding the pathophysiologic mechanisms behind the thalassemia intermedia (TI) phenotype, data on the effects of treatment are deficient. To provide such data, we evaluated 584 TI patients for the associations between patient and disease characteristics, treatment received, and the rate of complications. The most common(More)
BACKGROUND beta-thalassaemia is a preventable disease. Iran has about 20,000 homozygote beta-thalassaemia patients and 3,750,000 carriers. OBJECTIVE To assess the 10-year results of the screening programme, which has been operating in Southern Iran since 1995. METHODS All couples wanting to marry were required to be checked for beta-thalassaemia by(More)
Sickle cell anemia in Iran is accompanied by a high level of HbF and mild clinical presentation. Here we report haplotypes of the beta gene cluster found in 81 randomly selected sickle cell patients, including 47 sickle cell anemia (SS), 17 sickle cell trait (AS), and 17 sickle/thalassemia (S/thal) from southwest Iran. We found all five common typical(More)
Despite remarkable advances in understanding cerebrovascular disease attributed to sickle cell anemia, data from other hemoglobinopathies have only recently started to emerge. Several brain magnetic resonance imaging studies confirm a high prevalence of silent ischemic lesions in patients with β-thalassemia intermedia, especially in splenectomized adults(More)
Factor XIII (FXIII) deficiency is a very rare severe autosomal bleeding disorder with a frequency of 1:2,000,000 in the general population and only a few patients have been genetically characterized so far. We report a phenotype-genotype characterization of 10 unrelated Iranian patients. Two FXIII (transglutaminase) activity assays showed no FXIII activity,(More)
BACKGROUND Iron-deficiency anemia (IDA) is a common clinical problem throughout the world and an enormous public health risk in developing and even in industrialized countries. Traditionally, several methods other than serum ferritin were used to assess IDA. Our main objective was to obtain the prevalence of IDA by serum ferritin in 6 month to 5-year-old(More)
We present the molecular analysis of HFE gene in 400 Southwest Iranian individuals. We have studied 43 newborn, selected for the presence of HbBart's at birth, 203 normal adult and 154 transfused patients affected with beta-thalassemia. Mutation analysis consisted of amplification and direct sequencing using two different pairs of forward and reverse(More)
To prevent pregnancy loss in women with severe FXIII deficiency, prophylactic replacement therapy with a source of FXIII throughout pregnancy is essential. The aim of this study was to evaluate the bleeding score and rate of successful deliveries in FXIII-deficient pregnant Iranian women receiving regular prophylaxis. Seventeen FXIII-deficient women 18-35(More)
Prenatal diagnosis (PND) is an important issue in the comprehensive care of haemophiliacs. As a consequence of technological progress made in the field of PND, the early detection of an affected fetus provides the expectant couple with a chance to terminate pregnancy. This study was undertaken to assess the attitudes of two different haemophilic populations(More)
A comprehensive survey concerning the Shiraz Hemophilia Society and the associated haemophilia treatment centre was undertaken in April 2002 to collect data on demographics, signs and symptoms in the southern Iranian population with haemophilia and allied disorders. The total number of patients with coagulation disorders was 367. Haemophilia A (factor [F](More)