Journal of School of Public Health and

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Background and Aim: Patients with hemophilia and thalassemia are at high risk for hepatitis C virus (HCV) infections. Unscreened blood and blood products are thought to be the most important risk factors in these two groups.

Materials and Methods: Blood samples were collected from 98 thalassemia patients and 74 hemophiliacs in Markazi province. The presence of anti-HCV antibody was tested by a third generation enzyme immunoassay. All ELISA-reactive samples were tested with the confirmatory third-generation recombinant immunoblot assay RIBA. We used a specially designed questionnaire to obtain data from patients and the software package SPSS 11.5 for statistical analysis.

Results: HCV antibody was detected in 5 thalassemia patients (5.1%) and 31 hemophiliacs (41.9%). Anti-HCV positivity in patients with thalassemia was associated with the total volume of the transfusions, splenectomy, and the duration of treatment. In hemophiliacs, seropositivity was significantly associated with the duration of transfusion treatment (P=0.01) and disease severity (P=0.00). The prevalence of HCV antibody in thalassemia subjects dropped from 8.1% to 0% after the implementation of anti-HCV screening in1996.

Conclusion: In Markazi province, the current policy of screening donors for anti-HCV and the strict safety controls on factor concentrates can effectively protect thalassemia and hemophilia patients from HCV infection.

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Background and Aim: Thalassemia is one of the most common worldwide single-gene diseases. On the molecular level, it is a heterogeneous disease. So far, beside large deletions, more than fifty point mutations have been identified for this disease around the world. Four to ten percent of Iranians are carriers of thalassemia-affected genes. In this study we determined the relationship between the value of MCV/MCH and the type and severity of mutations in alpha thalassemia carriers.

Methods and Materials: The major abnormality in alpha thalassemia carriers is decreased values of MCV and MCH. A total of 104 adult alpha thalassemia heterozygotes, 20-40 years old, including 33 persons from Khoozestan Province and 71 from Khorasan Province, were included in the study. Two hundred and eight chromosomes were isolated from peripheral blood leukocytes using standard procedures. DNA samples were amplified by Gap-PCR.

Results: Only one ° mutation (MED) was found in 2.9% of the population. The carriers of this mutation had virtually identical ranges of MCV and MCH. In contrast, + mutations were associated with significant differences in mean MCV and MCH values. The various mean MCV/MCH ratios of ° carriers were statistically significantly lower than those of + heterozygote. The results indicated that the degree of reduction in MCV and MCH is directly related to the severity of the mutations. Deviations, in cases, were associated with lower numbers of the carriers.

Conclusion: Only one ° mutation (MED) was found in 2.9% of the population. The carriers of this mutation had virtually identical ranges of MCV and MCH. In contrast, + mutations were associated with significant differences in mean MCV and MCH values. The various mean MCV/MCH ratios of ° carriers were statistically significantly lower than those of + heterozygote. The results indicated that the degree of reduction in MCV and MCH is directly related to the severity of the mutations. Deviations, in cases, were associated with lower numbers of the carriers.

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Background and Aim: Thalassemia, a heterogeneous disease, is one of the most common single-gene diseases worldwide. The aim of this study was to find associations between hematological indices and severity of beta-globin gene mutations in beta-thalassemia carriers.
Materials and Methods: In this cross-sectional study, 30 beta-goblin gene mutations (b+ and b○) in 1206 unrelated beta-thalassemia carriers were investigated. In addition, their hematological indices, including CBC and electrophoresis results, were determined. The association between genetic findings and hematological parameters (mean corpuscular volume (MCV) and mean hematocrit corpuscular hematocrit (MCH)) were determined using the SPSS software, the statistical test being the t-test.
Results: The results indicated that b+-thalassemia carriers had higher MCV and MCH means than b○-thalassemia carriers. These findings would certainly have practical implications in public health.
Conclusion: The results show a significant correlation between two hematological indices and certain types of mutations in beta-thalassemia carriers.

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Background and Aim: Thalassemia major (ThM) is one of the most common autosomal recessive genetic disorders in the world. Its prevention and control is a priority in the disease prevention programs in the Iranian health system. The ThM screening program (screening at the time of marriage) has been an important step in its prevention in Iran. This study aimed to evaluate this program.

Materials and Methods: This was a descriptive-analytical study in Babosar District consisting of two phases. The first phase was a retrospective descriptive study aiming at finding the background causes of the diseases in subjects under 18 years old (born after March 1997). In phase 2, then, in an interventional analytical study, screening was repeated along with laboratory tests before, or at the beginning of, pregnancy (n = 5500).

Results: Phase 1(cross-sectional descriptive): There were 25 cases of ThM under 18 years old, parents of 15 of whom (60%) had married before 1997 and 4 (16%) had children suffering from ThM (S2). The parents of the remaining 10 (40%) had married after 1997 (S1). Phase 2: Considering the findings of Phase1, an interventional program called thalassemia screening was started in September 2006 including 9750 pregnant or pre-pregnanct mothers. The program led to identifying 20 new beta-thalassemia-carrier couples, including 10 couples married before 1997 and 10 married after 1997 (without official marriage registration, laboratory errors and hemoglobinopathies). All the 20 couples were quickly referred to the Genetic Center, which resulted in prevention of the birth of 4 ThM neonates. 

Conclusion: The 9-year intervention (2006-2015) resulted in timely action, preventing birth of 4 thalassemia major babies. Considering incidence of the disease during the previous 9 years (1997-2006), it can be concluded that the before-after randomized clinical trial is an effective way to reduce thalassemia major incidence.