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Volume 64, Issue 10 (10-2006)
Background: Major thalassemia is relatively common in Iran and in looking of their need to recurrent transfusion their high risk for acquisition of HCV is revealed. These patients also suffer form liver hemosiderosis that accelerate disease excursion to cirrhosis and hepatocellular carcinoma. In this study combined therapy with interferon and amantadin has been evaluated.
Methods: This prospective clinical trail has been done on thalasemic patients that had been contaminated by HCV and had laboratory signs of hepatitis. During years of 81-82, 26 patients admitted at Children Mmedical Center with major thalassemia and HCV hepatitis. Inclusion criteria were positive HCV-RNA, high ALT and histologic evidence of hepatitis in liver biopsy and exclusion criteria were history of incomplete treatment any contraindication of IFN or amantadin-emergence of drugs adverse reactions, to intending of these criteria 10 case of them had situation for treatment and follow-up.
Results: In 10 cases, treatment with IFN - 2b in doses 3 mu for every square meter of body surface three times in week subcutaneously and cap. Amantadin in doses 100 mg po B.d for 6 months was done and after it, 8 cases were negative for HCV-RNA (8%) and in 6 patients, ALT turned to normal (60%) and in 2 other case ALT decrease to lower than 50% of pretreatment value. None of them showed drug adverse reactions and response to therapy was better in lower ages. No relation between response to therapy and liver hemosiderosis, inflammation and sexuality was found.
Conclusion: Combined therapy with IFN and Amantadin is effective in HCV treatment and for lower recurrence, treatment period longer than 24 wk, such as 48 wk is recommended
Volume 74, Issue 3 (6-2016)
Hemoglobinopathies are most common inherited disorders in the world; approximately 7 percent of the worldwide population and 5-6 percent of population of Iran are carriers. The hemoglobin disorders inherit as autosomal recessive and are very common in the Mediterranean area and much of the Asia and Africa. The control of this inherited disorders need to genetic counseling and accurate screening by more advanced and more accurate methods. This study explains features of current Iran hemoglobin disorders, nominates the accessible methods for screening them and introduces the capillary zone electrophoresis as a rapid and more accurate method. The required data were extracted of various articles and then for good explanation, current Iran hemoglobinopathies properties were showed in the tables and electropherograms of important hemoglobin disorders in Iran population were provided for help to interpretation results of blood tests by capillary zone electrophoresis method. Hemoglobin disorders are including thalassemias and hemoglobin variants; Disruption in the production and malfunction of globin chains cause types of hemoglobin disorders. We cannot introduce one of clinical laboratory tests as critical and basic method for screening and distinguishing types of inherited hemoglobin disorders as alone. For distinguishing the types of them must be prepared enough information and data of the hemoglobin disorders and for more accurate analysis must be used simultaneously different methods as gel electrophoresis, high performance liquid chromatography, isoelectric focusing, capillary zone electrophoresis or molecular tests. The capillary electrophoresis is an accurate and rapid method for screening types of the hemoglobin disorders. Other side this method cannot analyze all of them, so must be used biochemical, biophysical and molecular methods for confirmation the results. This review showed we can use the capillary electrophoresis and HPLC as two complementary methods for hemoglobinopathies screening. We can analyze by the methods more hemoglobin disorders and decrease more laboratory errors. Moreover, we must have patient history, hematological indices, information and data of types of hemoglobinopathies. The patient history and complete blood count results as red blood cell count, mean corpuscular volume, mean corpuscular hemoglobin and mean corpuscular hemoglobin concentration can be useful and helpful in screening the hemoglobin disorders and then distinguishing all of hemoglobin disorders.
Volume 74, Issue 5 (8-2016)
Background: Human leukocyte antigen E is a member of non-classical HLA class I. Interaction between HLA-E molecule on the target cells and inhibitory CD94/NKG2A receptor on the cell surface of natural killer (NK) cells has an important role in the regulation of immune system against pathogens; therefore different cell surface expression of HLA-E molecule plays an important role in host resistance against viral infections as well as host response to treatment. Considering this fact, we analyzed the frequency of different HLA-E genotypes (HLA-E*01010101, HLA-E*01030103, HLA-E*01010103) in major thalassemic patients who underwent frequent transfusion therapy and are thus more susceptible to infectious diseases.
Methods: This study was a cross-sectional study of 104 major thalassemic patients who referred to Tehran Thalassemia Clinic between the years 2015 to 2016. Blood DNA was extracted and proliferated by sequence-specific primer polymerase chain reaction (SSP PCR). The PCR product was subjected to electrophoresis on 1.5 percent agarose gel then DNA fragment bands on the gel were detected by exposing to UV light. Furthermore, PCR products were also subjected to sequencing analysis for further confirmation.
Results: From 104 patients in this study, 49 (47.1%) were man and 55 (52.9%) were women. These patients were in the age range of 16 to 43 years (mean+SD; 31.03±4.7 year). The frequency of HLA-E*01010103 genotype (64.4 percent) was significantly (P= 0.001) higher than the genotypes of HLA-E*01010101 (15.4%) and HLA-E*01030103 (20.2%) whereas there was no difference between the frequency of HLA-E*0103 allele (52.4%) and HLA-E*0101 (47.6%).
Conclusion: This is the first study that examined the HLA-E polymorphisms in Iranian thalassemic patients referred to Tehran Thalassemia Clinic. This study has shown that the frequency of HLA-E*01010103 genotype was significantly higher than other genotypes of HLA-E whereas there was no difference between the frequency of HLA-E*0103 allele and HLA-E*0101 allele. Whether different frequencies of HLA-E genotype may affect thalassemic patients’ susceptibility to blood-borne infections will be of interest for future studies.
Volume 75, Issue 10 (1-2018)
Methods: In this case-control study, thalassemia patients who had visited in special clinic of Vali-e-Asr hospital of Birjand University Medical Sciences during January of 2013 to May of 2014 compared with normal subjects. They matched by age and sex. After explaining the aims of the project, the written consent was received from both groups. Clinical examinations including history taking and auscultation of heart and lungs were performed by Pediatrician. Moreover, using the standard methods, the measurement of height, weight and blood pressure were performed. In following, electrocardiography (ECG) was taken in both groups. Also, both groups underwent transthoracic echocardiography by one cardiologist.
Results: 42 thalassemia patients and 42 healthy subjects was compared in this study. Dyspnea was the only different symptom in the two groups. (23.8% in case and 4.8% in control P=0.01) In physical examination respiratory rate (RR) is only higher in patients with thalassemia that controls, but heart rate (HR), abnormal heart and lung sounds were not different significantly between two groups. In ECG, PR interval and QTc interval in patients with thalassemia was longer than healthy subject. In echocardiography, ejection fraction was lower but left ventricular end-diastolic diameter (LVEDD), left ventricular end-systolic diameter (LVESD) and size of the right ventricle and pulmonary artery pressure (PAP) was significantly higher than those in the control group.
Conclusion: According the result of this study, dyspnea was higher in patients with thalassemia from normal subjects and PR interval and QT interval was longer in thalassemic patients. As well as the size of the left ventricle, size of right ventricle, pulmonary artery pressure were higher than normal population. Also, LV ejection fraction was lower in thalassemia than normal population.
Volume 81, Issue 2 (5-2023)
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Thalassemia is an autosomal recessive hereditary disease that occurs due to a decrease in the synthesis of Please recheck. In beta thalassemia, defects in β-globin synthesis lead to an imbalance of β- and α-globin chains and the accumulation of α4 chains in the erythroid precursor which leads to ineffective erythropoiesis, shortened red blood cell survival, and finally clinical symptoms such as delayed sexual and physical maturation, endocrine dysfunction, cardiomyopathy, liver disease, bone deformities and hepatosplenomegaly. Current treatments such as transfusion, iron chelating agents and allogeneic stem cell hematopoietic transplantation have limitations in their use, including iron overload, lack of a human leukocyte antigen (HLA) matched compatible donor, and graft versus host disease (GVHD). Gene therapy is a new therapeutic option for beta thalassemia patients that induces the continuous expression of beta globin chains in the patient’s hematopoietic stem cells. The idea of gene therapy was first proposed in the early 1970s, and the ultimate goal of this treatment method is to express the defective gene in the target cell in a way that can reduce the symptoms of the disease or eliminate them (symptoms) altogether. There are two general methods for gene therapy: the integrating vector, in which the desired gene is inserted into the genome of the target cell and its lifelong expression follows, is the non-integrating method, in which the vector doesn’t integrate into the genome of the target cell and the cytoplasmic form enables gene expression. The first beta thalassemia gene therapy was performed in France in 2006, and in this clinical trial, the first patient with the E/β0 thalassemia was treated at the age of 18. Gene therapy for beta-thalassemia has been approved by the food and drug administration in 2022 for patients aged 12 years and older who have a non β0/β0 phenotype. It seems that this therapeutic option is the definitive treatment method for blood transfusion-dependent beta-thalassemia patients. However, this treatment method still has limitations: high cost, sensitivity of lentiviral vector production, and the possibility of integration of the vector near the proto-oncogene and its activation are some of them.
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