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BACKGROUND Meningeal melanocytomas are rare benign pigmented tumors of meningeal melanocytes. Nevus of Ota is a melanocytic pigmentation of the sclera and the skin adjacent to the eye. We report a rare case of parietal convexity meningeal melanocytoma associated with ipsilateral mucocutaneous melanosis and review the literature. METHODS A 17-year-old man(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 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)
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)
Hydroxyurea (HU) is a well-known chemotherapeutic agent that has been used largely for the treatment of various myeloproliferative conditions over the past 20 years. In beta-thalassemia, the role for HU is much less clear and remains controversial. This study was undertaken to describe the hematologic and clinical responses of thalassemia intermedia(More)
BACKGROUND AND OBJECTIVES Hydroxyurea (HU) is known to increase gamma-globin chain expression in postnatal life. The efficacy of HU treatment in thalassemia patients is still unclear. The aim of this study was to monitor treatment of a large cohort of patients with beta-thalassemia major in order to establish the response to HU and the associated elements.(More)
Chronic hypercoagulable state expressed clinically by thromboembolic events has been described in thalassemia. One of the affected organs is the brain where symptomatic and asymptomatic damage has been reported. The present report describes seven cases who presented with the signs of cerebrovascular accident (CVA), five ischemic and two with hemorrhage. Two(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)
The preliminary results of a pilot study are reported, intended as an initiation of a research plan, focused on the prevention of beta-thalassemia in Iran. The aims of this study are: (i) to improve the knowledge of the molecular background of beta-thalassemia intermedia in Southern Iran; (ii) to verify the role of the -158 (G)gamma (C-->T) (Xmn I)(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)