Background. Iron deficiency is a major health problem worldwide and especially in developing countries. Irondeficiency anemia has adverse effects on the development of children.Objective.
The results showed that kidney dysfunction in thalassemia increases with increasing age, duration, and levels of blood transfusion and hypercalciuria. It is therefore recommended that the presence of severe renal dysfunction in thalassemic patients should be investigated using sensitive and specific tests, mainly NAG, to prevent progress towards the complications.
Although alpha-thalassemia (alpha-thal) is the most common hereditary hemoglobin (Hb) disorder in Iran, no comprehensive data are so far available on the prevalence of the disease in the province of Khuzestan in Southwest Iran. This study investigates the spectrum of alpha-thal mutations in this region. One hundred and twenty-one subjects from Khuzestan Province, Iran, were initially tested for the three most common Iranian alpha-thal mutations (- alpha3.7, -alpha4.2, and --MED) by gap-polymerase chain reaction (gap-PCR). Reverse hybridization test strips and DNA sequencing were used to identify additional alpha-globin mutations. A total of 131 mutated alpha-globin alleles were identified in these patients. Of the 13 mutations that were detected in Khuzestan Province, Iran, the - alpha3.7 single gene deletion was the most frequently identified variant, representing 62.6% of the total; we also observed significant numbers of individuals with compound heterozygous mutations. On the basis of our results, we strongly recommend screening for the most common mutations to improve the molecular diagnosis of anemia in this region.
The results showed that molecular analysis must be used for accurate diagnosis of double heterozygotes in couples presumed to be discordant for alpha- and beta-thalassemia on hematologic testing. The accuracy of this technique was evaluated blindly, and the results were 100% (40 of 40) concordant with the genotypes previously characterized by Southern blotting or Gap-PCR.
Background:Although it is life-saving, blood transfusion therapy has resulted in risk for transfusion-transmitted infections (TTIs) in the majority of sickle cell anemia being patients. Objectives: The current study aimed to determine the prevalence of HBV, HCV and different genotypes of HCV among sickle cell anemia (SCA) patients in Ahvaz city, South-western Iran. Materials and Methods: A cross-sectional study was conducted on 56 SCA patients who referred to the Hemoglobinopathy and Thalassemia research centre during January 2009 to February 2010. Patients` sera were tested for HBsAg and anti-HCV using ELISA and confirmed by PCR (HBV) and RT-PCR (HCV). HCV genotypes were determined with HCV genotype specific primers using HCV genotyping kit.
Results:The overall prevalence rate of HBsAg and anti-HCV were 1.8% (95% CI: 0-5.26) and 12.5% (95% CI: 3.8 -21.1), respectively. Five of the anti-HCV patients (71.4%) were also HCV RNA positive. The predominant HCV genotype in the patients was 1a (60%), though genotype 1b was found in 40% of the subjects. Conclusions: It seems that stringent donor selection procedures reduce HCV infection in multi-transfused patients, but there is still serious risk for these patients.
Extramedullary hematopoiesis (EMH) is a rare disorder, characterized by the appearance of hematopoietic elements outside of the bone marrow, which occurs in patients with chronic myeloproliferative disorders or congenital hemolytic anemias. We report a case with thalassemia intermedia, EMH in spinal cord and a paravertebral mass. We report the case of a 20 year old female who initially presented with back pain and leg weakness was found to have paravertebral mass in the spinal cord.
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