سال:1388 | دوره: | شماره: |تعداد مقالات:9

Despite low number of patients with coagulation disorders this field becomes one of the most lucrative marketing fields for pharmaceutical industry worldwide. Governments both in countries with developed economies and some developing countries such as Iran spends substantial amounts of their health care budget on providing very expensive medicines for managing patients with coagulation disorders.

Background: Expansion of bone marrow cavity and decreased cortical and trabecular bone tissues and osteoporosis are resulted from beta-thalassemia. The aim of this study was to assess bone mineral density (BMD) in patients with b thalassemia major and intermedia, and to determine their biochemical and hormonal profiles that may affect BMD.Materials and Methods: In a cross sectional study from October 2004 to April 2006, 305 patients (273 thalassemia major [137 males and 136 females] and 32 thalassemia intermedia [13 males and 19 females]) were evaluated for BMD. Dual x-ray absorptiometry was performed at 3 sites: spine (L2-L4), femoral neck, and radius. Z score< -2.5 was considered as osteoporosis, and between -1 and -2.5 as osteopenia. Z-scores were calculated according to bone density values based on age and sex. Patients were grouped according to age 3-6, 6-10, 10-13, 13-16, and over 16 years old. The stage of puberty was determined according to Tanner staging and its progression was followed. Biochemical and hormonal profiles of patients were recorded.Results: In thalassemia major, mean age was 14±6.5 years, and mean BMD Z-score of spine, radius and hip were -2.3±0.9, -2.8±1.2, and -1.9±1.4, respectively. Mean age of patients with thalassemia intermedia was 13.4±6.2 years, and the mean Z-score of spine, radius, and hip were -2.1±0.9, -2.0±1.3, and -2.3±1.3, respectively. Hypogonadism was detected in 36% of thalassemia major and 35% of thalassemia intermedia; but hypothyroidism, diabetes mellitus, and hypoparathyroidism were detected only in thalassemia major with frequency of 2.8%, 1.8%, and 1.2%, respectively. BMD in spine and radius were significantly lower in patients with hypogonadism than in patients with normal puberty (P=0.039 and P=0.015, respectively). Height Standard Deviation Score (HSDS) was not significantly different in groups of osteoporosis and normal. Osteoporosis was seen in all age groups, and was more common in males than females at spine and radius bones (P<0.001). It was less common in patients with hypothyroidism, hypoparathyroidism, and diabetes mellitus. BMD Z-Score had significant correlation with serum ferritin only in radius area (P=0.04), and it had no significant correlation with serum Ca, P, Mg and Zn.Conclusion: Our results showed that the patients with thalassemia major and intermedia had low BMD. Patients with hypogonadism and males had lower BMD. Young children also had low bone mass, so early attention is essential.

Background: Hepatitis C virus (HCV) infection is the most common transfusion transmitted disease in poly-transfused patients worldwide. In this study we aimed to evaluate the effects of pegylated interferon alfa-2a (PEG-IFN A-2a) in reducing serum ALT and eradicating serum hepatitis C virus (HCV) RNA in HCV infected polytransfused thalassemic patients.Materials and Methods: A cohort of 51 HCV-RNA positive thalassemic patients was enrolled to our study and received 180 mg PEG-IFN A-2a once-weekly for 48 weeks. The primary end point was sustained virological response (SVR). The secondary outcome was normalization of ALT. Patient safety was assured by monthly, and if needed, weekly laboratory assessment and visits.Results: Of 52 patients, 42 participants completed the treatment schedule. A sustained virological response (SVR) was attained in 22/51 (43%) cases. Among non-responders or relapsers to previous HCV antiviral therapy, 9/27 (33%) attained an SVR. Five patients died during treatment and 3 subjects discontinued the therapy because of adverse effects. Adverse events were generally mild, and laboratory abnormalities were rare.Conclusion: A course of 48-week PEG-IFN A-2a monotherapy is effective in eradicating HCV-RNA during treatment. But about one third of thalassemic patients would relapse within 6 months of treatment schedule completion, in whom combination therapy is needed.

Background: Treatment of cancer patients using conventional chemotherapy causes serious side effects and, at best, merely extends the patient’s lifespan by a few years. The potential of alternative therapies may therefore be of great benefit in cancer control. The effect of Trigonella Foenum Graecum seed extract has been previously reported on some neoplastic cells. Here, its effect is evaluated on human acute myeloblastic leukemia cell lines.Materials and Methods: The cell line KG-1 was treated with various concentrations of Fenugreek seeds extract with various durations. Cellular enumeration, viability test, staining and light microscopy, and apoptosis induction were evaluated.Results: Results showed significant cytotoxic effect of Fenugreek seeds extract against this cell line which resulted in growth inhibition, cell death and morphological changes. Apoptosis induction was not considerable. Fenugreek seeds extract did not change the count and morphology of normal lymphocytes.Conclusion: Applying herbal medicines could be an effective and safe treatment for leukemia. To our knowledge, this is the first study that suggests significant chemotherapeutic effects of Fenugreek seeds against these cell lines.

Background: Hydroxyurea (HU) is a well known chemotherapeutic agent that has been used largely for various myeloproliferative diseases over the past 20 years. In b-thalassemia, the effect of HU is much less clear and remains controversial. This study was undertaken to describe the hematologic and clinical responses of thalassemia major and intermediate patients to HU treatment during 2 years.Materials and Methods: Seventy one major and twenty transfusion-dependent intermediate thalassemia participants were selected among 150 b-thalassemia patients. All patients underwent laboratory tests, and the state of energy, social activity, tolerance, and mood were recorded in the beginning of the study. Echocardiography was carried out before and during treatment with HU. All patients were treated with HU; the initial dose was 10-15mg/kg/day given once a day.Results: All the patients tolerated HU well and showed a dramatic response to the drug. Nine of 20 intermediate and 8 of 71 major patients became completely transfusion free. In 6 intermediate and 15 major patients, transfusion interval prolonged more than 50%. After treatment, 95% of intermediate and 81% of major patients described an increase in social activity. HU therapy was also associated with a marked decrease in serum ferritin level in major thalassemia patients.Conclusion: HU may be administered in thalassemia major and intermediate patients to minimize or obviate the need for regular transfusion and concomitant iron overload. HU therapy appears to be safe and effective when administrated in thalassemia patients.

Background: Malaria remains the world’s most devastating human parasitic infection. Our goal was to assess the capacity of increasing concentrations of five antimalarial drugs (FansidarTM, HalfanTM, Quinine, CoartemTM and Chloroquine phosphate) to elicit the generation of methemoglobin in three human erythrocyte genotypes (Hb AA, Hb AS and Hb SS).Materials and Methods: Spectrophotometric method was used for determination of plasma methaemoglobin concentration in the presence of 0.2 g%, 0.4 g%, 0.6 g% and 0.8 g% (w/v) of the five antimalarial drugs.Results: The five antimalarial drugs showed a concentration dependent variability to cause the elevation of plasma methemoglobin concentration in the three genotypes. Specifically, CoartemTM, exhibited the highest propensity to elevate plasma methemoglobin concentration. However, the other four antimalarial drugs showed a statistically significant (P<0.05) but minimal effect to cause elevation of plasma methemoglobin concentration. For instance, with Hb AS blood sample and at drug concentration of 0.8g%, methaemoglobin concentrations (percentage) 0f 3.03±1.82, 2.65±0.45, 6.41±1.21, and 3.02±0.98 were obtained for halfan, quinine, coartem and chloroquine phosphate, respectively. The control value was 2.17±1.82% of methemoglobin.Conclusion: The oxidative potentials of these four antimalarial drugs and their metabolites in the red cells did not overwhelm the erythrocyte methemoglobin reducing capacity that could elicit the presentation in vitro toxic methemoglobinemia.

Background: The "Iron Supplementation Project" for anemic toddlers in Iran has not been subject to scientific studies yet. Since daily consumption of iron drops by children could bring about physical and mental problems, the aim of this study was to determine whether weekly doses of iron drops would also improve the iron status of children significantly.Materials and Methods: We determined the iron status of 12 to 21-month-old anemic toddlers receiving iron drops (ferrous sulfate) daily referred to health care centers in Shiraz. One hundred and four children were divided into two groups; one group receiving iron drops (1 mg/kg) daily as before, and the other group receiving iron drops weekly (3 mg/kg).Results: After three months children showed a significant increase in hemoglobin (Hb), mean corpuscular hemoglobin (MCH), and mean cellular hemoglobin concentration (MCHC) levels. Although weekly supplementation led to a significant increase in Hb levels, daily supplementation had a significantly greater effect than weekly supplementation on the levels of mean corpuscular volume (MCV) and MCHC.Conclusion: We generally conclude that weekly iron supplementation is not suitable for anemic children.

Methyltetrahydrofolate reductase (MTHFR) enzyme is responsible for conversion of 5, 10- methyl tetrahydrofolate to 5-tetrahydrofolate for folic acid (folate) synthesis. One of the most common MTHFR mutations is a nucleotide transition from C-to-T at position 677 that results in amino acid alteration from alanine to valine.

Lymphoma is a hematologic malignancy characterized by nodal or extra nodal malignant infiltration by monoclonal lymphoid cells (B lineage cells in 70% of the cases). Its etiology is not precisely known. Prolonged life expectancy has resulted in increase of the disease incidence. Marginal zone lymphoma is a low-grade small B-cell lymphoma incorporated in the World Health Organization classification. Low-grade lymphomas usually demonstrate a characteristic natural history with multiple remissions and relapses.