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Background and Aim: Thalassemia major is a common genetic anemia disorder that causes dental problems such as dental caries and malocclusion. In this study, we investigated the effect of malocclusion severity on quality of life of patients with thalassemia major. Materials and Methods: In this descriptive-analytical cross-sectional study, 37 patients with thalassemia major participated. Patients' oral health quality of life was assessed based on orthognathic quality of life questionnaire (OQLQ) and malocclusion severity was measured by index of orthodontic treatment need (IOTN). Data was analyzed by Spss26 and One Way Anova with significance level less than 0.05. Results: In this study, it was found that quality of life associated with malocclusion was poor in 43.2% of patients with thalassemia major. Regarding the severity of malocclusion, 94.6% of people were in grade 3 to 5 and the highest prevalence was related to grade 3 (45.9%). There was no significant relationship between quality of life and malocclusion severity or gender (P>0.05). Conclusion: Approximately half of patients with thalassemia major had poor quality of life associated with malocclusion, and nearly all patients showed moderate to severe orthodontic treatment need. With increasing severity of malocclusion, no significant change was observed in quality of life. There was also no significant relationship between quality of life and gender and age. [ABSTRACT FROM AUTHOR]

Introduction: This study aims to investigate the necessity of simultaneous cholecystectomy in thalassemia major patients undergoing splenectomy, in light of reports regarding the prevalence of gallstones in these patients and the overall incidence of the disease in Mazandaran. Methods: This study was conducted on all 247 patients with beta-thalassemia major, who underwent splenectomy surgery during 2010-2020 for any indication in medical centers affiliated to Babol University of Medical Sciences (without a history of this procedure). Their percentage was determined and a decision was made regarding simultaneous surgery of cholecystectomy and splenectomy. Sampling was done by census method and all cases were reviewed and patients were followed up by phone and the necessary information was extracted and recorded in the checklist. Data were described using SPSS 22 software and analyzed at a significance level of 0.05. Results: A total of 247 patients with an average age of 20.13 ± 3.98 years (minimum age 10 and maximum age 29) were included in the study. The frequency of simultaneous cholecystectomy was reported in 33 cases (13.4%). Out of 214 patients who did not need simultaneous cholecystectomy (57.9%), 183 patients underwent cholecystectomy after splenectomy (85.5%).In 28 people (11.3 percent) had normal ultrasound and 3 people (1.3 percent) died. The prevalence of gallstones was reported as 87.4 percent. It is The average time for cholecystectomy compared to splenectomy in 183 patients who underwent cholecystectomy was 13.41±6.94 months (minimum time interval 3 and maximum 28 months) and in the examination of ultrasound findings, 219 patients (88.7%) had stones. They had a gall bladder and normal ultrasound was reported in 28 people (11.3%). Conclusion: The study results indicate a high prevalence of gallstones in patients with thalassemia major. Therefore, it suggests that simultaneous cholecystectomy and splenectomy should be considered, even in asymptomatic cases. [ABSTRACT FROM AUTHOR]

Background and purpose: Thalassemia is a common disorder in Iran and the world. The progress in the field of diagnosis and treatment of thalassemia made these people able to live for a longer period. In these patients, frequent blood transfusions are associated with numerous complications such as depression, anxiety, stress, etc. These problems are associated with a decrease in the quality of life. Therefore, this study was carried out to investigate and compare the quality of life in beta-thalassemia major patients with and without blood transfusion. Materials and methods: In this study, which was cross-sectional and descriptive-analytical in nature, a total of 170 patients with thalassemia major, aged 18-60, who were referred to the Thalassemia Center of Bo Ali Sina Hospital in Sari during the summer of 2014, were examined. The study was conducted with the ethical code IR.MAZUMS.REC.1402.18665 of Mazandaran University of Medical Sciences. According to the inclusion criteria, education level, and age range of the studied population, 116 patients out of 170 thalassemia major patients were studied. Considering that the number of thalassemia major patients with and without blood transfusions referring to the thalassemia center of Bo Ali Sina Hospital in Sari is almost equal, out of 116 patients with inclusion criteria, 58 patients are treated with periodic blood transfusions and 58 patients are under control without the need for transfusions. Blood was studied. Thalassemia patients are treated with blood transfusion every 15-30 days for injection and patients who do not need blood transfusion every 1-3 months to the thalassemia center for control. SF 36 standard questionnaire was used to check the quality of life. The reliability and validity of the Persian questionnaire were reported with Cronbach's alpha coefficient of 0.77 to 0.90 and vitality scale of 0.65. To analyze the variables, frequency indices, percentages, independent t-test, and chi-square test were used. Results: Among the thalassemia major patients studied, 52 (44.8%) were male and 64 (55.1%) were female. The average age in the group with and without blood transfusion was 34.75±8.35 and 39.33±8.18 years, respectively. There was no statistically significant difference in the average age between the two groups (P=0.128). There was no significant difference in the marital status, occupation, and level of education between the patients with and without thalassemia major injection. Among the investigated clinical complications, diabetes was significantly higher in the blood transfusion group (P=0.001). From the average coefficients of the eight indices of the SF 36 test, the subscale of general health (GH) was significantly better in patients with blood transfusion with a mean and standard deviation of 55.79±19.20 than in the non-injection group, 44.05±21.59 (P=0.002). Conclusion: It is suggested that in patients with thalassemia, especially thalassemia major, with or without blood transfusion, like other periodical control measures and medical treatment, evaluation of general health and quality of life should be carried out at regular intervals, to improve the quality of life of these patients, timely psychological or psychiatric interventions to be done at the required time.

Background and purpose: Thalassemia is a common disorder in Iran and the world. The progress in the field of diagnosis and treatment of thalassemia made these people able to live for a longer period. In these patients, frequent blood transfusions are associated with numerous complications such as depression, anxiety, stress, etc. These problems are associated with a decrease in the quality of life. Therefore, this study was carried out to investigate and compare the quality of life in beta-thalassemia major patients with and without blood transfusion. Materials and methods: In this study, which was cross-sectional and descriptive-analytical in nature, a total of 170 patients with thalassemia major, aged 18-60, who were referred to the Thalassemia Center of Bo Ali Sina Hospital in Sari during the summer of 2014, were examined. The study was conducted with the ethical code IR.MAZUMS.REC.1402.18665 of Mazandaran University of Medical Sciences. According to the inclusion criteria, education level, and age range of the studied population, 116 patients out of 170 thalassemia major patients were studied. Considering that the number of thalassemia major patients with and without blood transfusions referring to the thalassemia center of Bo Ali Sina Hospital in Sari is almost equal, out of 116 patients with inclusion criteria, 58 patients are treated with periodic blood transfusions and 58 patients are under control without the need for transfusions. Blood was studied. Thalassemia patients are treated with blood transfusion every 15-30 days for injection and patients who do not need blood transfusion every 1-3 months to the thalassemia center for control. SF 36 standard questionnaire was used to check the quality of life. The reliability and validity of the Persian questionnaire were reported with Cronbach's alpha coefficient of 0.77 to 0.90 and vitality scale of 0.65. To analyze the variables, frequency indices, percentages, independent t-test, and chi-square test were used. Results: Among the thalassemia major patients studied, 52 (44.8%) were male and 64 (55.1%) were female. The average age in the group with and without blood transfusion was 34.75±8.35 and 39.33±8.18 years, respectively. There was no statistically significant difference in the average age between the two groups (P=0.128). There was no significant difference in the marital status, occupation, and level of education between the patients with and without thalassemia major injection. Among the investigated clinical complications, diabetes was significantly higher in the blood transfusion group (P=0.001). From the average coefficients of the eight indices of the SF 36 test, the subscale of general health (GH) was significantly better in patients with blood transfusion with a mean and standard deviation of 55.79±19.20 than in the non-injection group, 44.05±21.59 (P=0.002). Conclusion: It is suggested that in patients with thalassemia, especially thalassemia major, with or without blood transfusion, like other periodical control measures and medical treatment, evaluation of general health and quality of life should be carried out at regular intervals, to improve the quality of life of these patients, timely psychological or psychiatric interventions to be done at the required time. [ABSTRACT FROM AUTHOR]

Background and Objectives Thalassemia is the most common genetic disease associated with the body's recurrent blood transfusions and iron overload. Iron chelators can reduce the adverse effects of iron overload through various mechanisms. However, concerns have recently been raised about their negative impact on renal function. The current study, therefore, examined the effect of different iron chelators on renal function in patients with thalassemia major. Subjects and Methods This cross-sectional descriptive study included primary thalassemia patients (5 - 25 years) referring to Ahwaz Thalassemia Center for regular blood transfusion and regular treatment with iron chelators. They were divided into 3 groups (deferiprone 60-80 mg/kg/d, deferasirox 15-35 mg/kg/d and deferoxamine 11-48 mg/kg/d). Blood and 24-hour urine samples were collected to determine glomerular filtration rate (GFR) and biochemical factors. Results No significant difference was observed between the studied groups in terms of GFR, urine albumin, serum creatinine, serum cystatin C, serum and urine phosphorus, and urine sodium (p > 0.05), but serum sodium and urine β2microglobulin (β2M) were significantly different between the studied groups (p < 0.05). Serum sodium was significantly higher in the deferiprone group compared to the control (P=0.004). Urine β2M was significantly higher in the deferoxamine and deferasirox groups in comparison with the control group (P=0.041, P=0.013). Finally, serum sodium was significantly higher in the deferiprone and deferasirox groups than that in the deferoxamine group (P=0.001, P=0.021). Conclusion Urine beta-2 microglobulin increased in patients receiving deferoxamine and deferasirox compared to the control group, which might indicate primary kidney damage. [ABSTRACT FROM AUTHOR]

Background and Aim: Serum ferritin in patients with thalassemia major is an indicator of iron overload, which is the main cause of damage to various organs of the body in patients with thalassemia major. The aim of this study was to investigate the relationship between serum ferritin level and renal function in thalassemia major patients. Materials and Methods: This descriptive and cross-sectional study was performed on patients with thalassemia major in Kurdistan province (west region of Iran) in 2019. All patients with thalassemia major were evaluated in terms of having regular blood transfusions for at least 1 year. The obtained data were entered into SPSS 22 software and statistically analyzed after measuring serum ferritin, creatinine and calculating glomerular filtration rate (GFR). Results: In this study, 122 patients were evaluated according to the inclusion and exclusion criteria. 54.1% were male and 45.9% were female. The mean age of patients was 20.28 ± 8.14 years, the mean hemoglobin was 8.95 ± 1.17gr/dl, the mean ferritin was 2812 ± 2134 ng/ml, the mean creatinine was 0.65 ± 0.17 mg/dl and the mean GFR (ml/min) was 122.19 ± 24.13. No significant correlation was found between GFR and serum ferritin (P> 0.05). Conclusion: Due to the lack of correlation between serum ferritin and GFR, it can be concluded that iron overload has no role in possible glomerular damage in thalassemia major patients.

Background and Aims: The chronic blood disorders could have negative effects on various fields of patient’s quality of life. The aim of this study was to evaluate the relationship between the quality of life and oral health in major thalassemia and hemophilia patients. Materials and Methods: This cross- sectional study was performed in 1398 on 56 hemophilia patients and 35 thalassemia major patients over 15 years of age. All the subjects were examined clinically to determine DMFT and CPITN, then the subjects completed OHIP-14, xerostomia, SF36 questionnaire. Data was analyzed with T-Test, ANOVA and Chi-square using Stata14. Results: TThe mean age of hemophilia and beta-thalassemia patients was 32.3 and 26.2, the mean DMFT index was 8.75 and 7.6, and CPITN index was 2.02 and 2.26, respectively. Analysis of the components of the DMFT index showed a predominance of number of decayed teeth over filled teeth in both groups. According to the CPITN index, 19.6% and 20% of hemophilia and thalassemia patients had gingival healthy, respectively. The mean quality of life total scores in patients with hemophilia and thalassemia was 62.11 and 58.07, respectively. The mean DMFT had a significant negative relationship with the quality of life total scores in both groups (P

Background: Major thalassemia has a significant impact on the personal performance and life of the affected person and leads to reduced quality of life (QoL) of the patient. One of the ways to improve quality of life is to use the Continuous Care Model (CCM). Aim: This study aimed to determine the impact of CCM on the QoL of patients with thalassemia major. Method: A clinical trial was performed with 60 thalassemia patients referring to Aliasghar Hospital in Zahedan, Iran. Eligible candidates were selected by convenience sampling method and randomly assigned to the intervention and control groups (n=30 each). Data collection tools included a demographic form, the quality of life questionnaire the brief version of the World Health Organization's QoL, and a self-control checklist. The intervention was performed in six training sessions three times a week in the presence of the patient and a family member; however, the control group received no intervention. Data analysis was performed in SPSS software (version 21). Results: The mean scores of QoL and its dimensions showed no significant difference between the intervention and control groups before the intervention (P>0.05); nevertheless, a significant difference was observed one and three months after the implementation of the model regarding (P

Introduction: Chronic diseases, including Thalassemia, have a profound effect on the functioning and overall life of the individual, especially at an early age. Given the low quality of life of adolescents with thalassemia and the importance of their function in society, this study aimed to determine the impact of applying life skills on quality of life in adolescents in Ahwaz in 2018. Methods: This quasi-experimental study was performed on 84 adolescents with thalassemia major referred to Thalassemia Clinic of Baqa'i Hospital, Ahwaz city, Iran. The study was conducted from 2018 to 2020. Adolescents were randomly divided into intervention and control groups. Both groups completed the demographic information questionnaire and the Kidney Screening Questionnaire for Quality of Life of the Teenager 52. Then the experimental group received the educational content (holding a class, Q&A and booklet) for 6 sessions of 2-hour. The control group did not receive any training. Data were analyzed by SPSS 22 and Chi-square, independent t-test, and paired t-test. Results: The results showed that the difference between the mean of quality of life in the two groups of control and intervention was not significant (P

Background and Aims : Thalassemia is a common hereditary disease which is one of the health problems in Iran due to its high prevalence. The aim of this study was to evaluate the dental health and treatment needs of patients with major thalassemia in Bandar Abbas. Materials and Methods: This descriptive cross-sectional study was performed on 80 thalassemic patients referred to Bandar Abbas health centers. The control group consisted of 80 healthy individuals who were selected by age and sex similar to the patient group. The age range of participants was between 4 and 22 years old with an average of 12.36 ± 14.8 years. The demographic data were recorded in the questionnaire and then the caries and dental needs were determined by clinical examination. Data were analyzed by descriptive and analytical statistics using the SPSS-21 software. Results: The mean DMFT of permanent teeth in thalassemia and control group was 5.04 ± 5.07 and 2.9 ± 3.57 respectively, which according to Mann-Whitney test, there was a significant difference between the two groups (P value = 0.015). This rate in deciduous teeth for thalassemia and control groups was 5.59 ±3.36 and 4.05± 2.99, respectively (P value = 0.044). In thalassemia patients, the need for restoration (P value = 0.027) and root canal treatment (P value = 0.013) were higher. There was also a significant difference between DMFT and frequency of tooth brushing in both groups of thalassemia and control group (<0.0001). Conclusion: Due to the higher level of DMFT in patients with thalassemia major, health education, preventive measures and dental treatments are necessary for these patients. [ABSTRACT FROM AUTHOR]

Background and purpose: Patients with thalassemia major need more dental care because of their special health conditions. The purpose of this study was to evaluate dental health and therapeutic needs of these patients in Sari, Iran 2018. Materials and methods: A descriptive cross-sectional study was done using a researcher-made form and direct observation in 144 cases attending Sari Thalassemia Center. The subjects included thalassemia patients (41 males and 34 females) and 69 individuals as the control group. The needs for dental treatment and prevalence of dental problems were recorded using Decayed, Missing, and Filled Teeth (DMFT) index. Data analysis was done in SPSS V24. Results: There were significant differences between the two groups in DMFT index (P< 0.001), decayed teeth (P= 0.004), and missing teeth (P=0.001). In fact, the values except the mean number of filled teeth were higher in thalassemia patients (P=0.150). Pulp therapy was the most common treatment needed in thalassemia patients while dental filling was more needed in control group. Conclusion: High prevalence of dental caries in patients with thalassemia major highlights the need for effective preventive measures, appropriate health trainings, and dental treatments in this group.

Background and Aim: Thalassemia is a chronic disease which is extremely expensive, complex and debilitating. The management skill of thalassemia patients should be enhanced to minimize the risk of disease complications. The main purpose of this study was to develop personal electronic health records for thalassemia major patients. Materials and Methods: This is a developmental applied study which was conducted to develop a personal electronic health record for thalassemia major. First, a questionnaire was prepared to determine the data elements and was filled by Hematology and Oncology professionals in the country (110 persons). Then, based on the results of needs analysis, the system was designed using PHP programming language and MySQL database and was evaluated by 50 thalassemia patients who referred to the Thalassemia Clinic of Bu Ali Sina Hospital of Mazandaran University of Medical of Sciences during the second half of the month of Aban. Finally, a standard questionnaire of usability and user satisfaction assessment was distributed among them. Results: Usability evaluation of the system showed that patients evaluated the system at a good level with a mean rating of 7.91 (out of 9 points). Conclusion: The web-based systems can be used to help thalassemia patients to control injection and reduce the complications of the disease and to promote health.

Background and Aims: The chronic blood disorders could have negative effects on various fields of patient’s quality of life. The aim of this study was to evaluate the relationship between the quality of life and oral health in major thalassemia and hemophilia patients. Materials and Methods: This cross- sectional study was performed in 1398 on 56 hemophilia patients and 35 thalassemia major patients over 15 years of age. All the subjects were examined clinically to determine DMFT and CPITN, then the subjects completed OHIP-14, xerostomia, SF36 questionnaire. Data was analyzed with T-Test, ANOVA and Chi-square using Stata14. Results: The mean age of hemophilia and beta-thalassemia patients was 32.3 and 26.2, the mean DMFT index was 8.75 and 7.6, and CPITN index was 2.02 and 2.26, respectively. Analysis of the components of the DMFT index showed a predominance of number of decayed teeth over filled teeth in both groups. According to the CPITN index, 19.6% and 20% of hemophilia and thalassemia patients had gingival healthy, respectively. The mean quality of life total scores in patients with hemophilia and thalassemia was 62.11 and 58.07, respectively. The mean DMFT had a significant negative relationship with the quality of life total scores in both groups (P<0.05). There was a significant relationship between different domains of quality of life and CPITN and OHIP-14 in thalassemia patients (P<0.05). Also patients with dry mouth had lower quality of life. Conclusion: Hemophilia and thalassemia patients indicated undesirable oral health. It is necessary to improve multidimensional strategies in various fields of health care in patients. [ABSTRACT FROM AUTHOR]

Background and Objectives Mothers of children with thalassemia as primary caregivers face problems with treatment and care issues. These problems as consequences of child’s illness often lead to inappropriate adaptive behaviors. The aim of this study was to disclose the relationship between caregiver burden and adaptive behaviors in mothers of children with thalassemia. Materials and Methods In this cross-sectional study, 70 mothers of children with thalassemia major who had referred to the Thalassemia Clinic of Ali Asghar Hospital, Zahedan, in 2016 were studied. Data were gathered using a demographic questionnaire, the Coping Health Inventory for Parents (CHIP), and the Caregiver Burden Scale (CBS). The results were analyzed in SPSS 22 using descriptive statistics, Pearson correlation coefficient, and stepwise multiple regression. Results The mean scores of mothers’ care burden and adaptive behaviors were 56.92 ± 12.84 and 57.07 ± 19.84, respectively. It was observed that while care burden has a significant inverse relationship with the total score of adaptive behaviors (r = -0.41, p < 0.001), it displays a direct significant relationship with mother's education (r = 0.26 p = 0.05). The results of multiple regression analysis suggested that the two components of adaptive behaviors and mother’s education could predict 18% of changes in mothers’ care burden. Conclusions According to the results, it is recommended to utilize appropriate educational and supportive interventions for improving the mothers' adaptive behaviors and continuous evaluation of these interventions is necessary. [ABSTRACT FROM AUTHOR]

Background & Aim: Improving the mental health of mothers of children with thalassemia major who experience great caregiving burden, requires an efficient supportive training to be developed and implemented. Therefore, this study aimed to determine the impact of a group supportive training on caregiving burden in the mothers of children with thalassemia major. Methods & Materials: A quasi-experimental study (IRCT2016122731612N1) was conducted on 80 mothers of children with thalassemia, referred to Aliasghar hospital in Zahedan in summer 2016. The mothers were selected through convenience sampling method and divided into two intervention and control groups of 40 each. The intervention group received four sessions of supportive training during four weeks. Data were collected by the Caregiver Burden Inventory. SPSS version 21 and independent t test, paired t-test and the analysis of covariance were used to analyze the data. Results: The mean score of caregiving burden before training in the intervention and control groups were respectively, 86.60±13.55 and 92.37±8.54 and declined to 59.62±9.90 and 89.57±5.83 after the intervention. Furthermore, The caregiving burden score in five dimensions including temporal, developmental, physical, social and emotional was significantly lower in the intervention group than in the control group (P

Pulmonary arterial hypertension (PAH) is a progressive disease with high morbidity and mortality rates. Research has shown that PAH has a prevalence rate of 10-79% in thalassemia major patients. This cross-sectional study was carried out in 2014 to determine the prevalence and risk factors of PAH in all thalassemia major patients of over 18 years of age in Ilam, Iran. A cardiologist measured systolic pulmonary artery pressure (SPAP) by using Doppler echocardiography. SPAP of higher than 25 mm Hg was defined as PAH criterion. The obtained data was analyzed using SPSS, version 17. Of the 36 studied patients, 17 (47.2%) were male and 19 (52.8%) were female. The mean age of the patients was 26.0±5.6 years. The prevalence of PAH in the thalassemia major patients was estimated to be 47.2% and the mean SPAP was determined to be 26.2±14.6 mm Hg. Among the examined PAH risk factors, only the history of pulmonary disease (0.02) and transfusion (0.03) was found to be significant.

Background and purpose: Patients with thalassemia major need more dental care because of their special health conditions. The purpose of this study was to evaluate dental health and therapeutic needs of these patients in Sari, Iran 2018. Materials and methods: A descriptive cross-sectional study was done using a researcher-made form and direct observation in 144 cases attending Sari Thalassemia Center. The subjects included thalassemia patients (41 males and 34 females) and 69 individuals as the control group. The needs for dental treatment and prevalence of dental problems were recorded using Decayed, Missing, and Filled Teeth (DMFT) index. Data analysis was done in SPSS V24. Results: There were significant differences between the two groups in DMFT index (P< 0.001), decayed teeth (P= 0.004), and missing teeth (P=0.001). In fact, the values except the mean number of filled teeth were higher in thalassemia patients (P=0.150). Pulp therapy was the most common treatment needed in thalassemia patients while dental filling was more needed in control group. Conclusion: High prevalence of dental caries in patients with thalassemia major highlights the need for effective preventive measures, appropriate health trainings, and dental treatments in this group. [ABSTRACT FROM AUTHOR]

Background and Objectives: A small sample size can influence the results of statistical analysis. A reduction in the sample size may happen due to different reasons, such as loss of information, i.e. existing missing value in some variables. This study aimed to apply bootstrap and jackknife resampling methods in survival analysis of thalassemia major patients. Methods: In this historical cohort study, the data of 296 patients with thalassemia major who were visited at Zafar Clinic, Tehran, from 1994 to 2013 were used. Parametric survival models were used to analyze the data. The log – normal survival model was selected as the best model and then the bootstrap and jackknife resampling algorithms were used for this model. Data analysis was carried out with the STATA 12.0 software. Results: The results of the resampling methods showed that standard errors decreased and confidence intervals were shortened. In addition, the result of the bootstrap and jackknife resampling methods showed that age group and the relationship of the parents (P0.900). Conclusion: Comparison of the confidence intervals suggests that the jackknife resampling method can be used when the sample size is small.

BACKGROUND AND OBJECTIVE: Hypoparathyroidism is one the common complications of endocrine in patients with thalassemia major. Several studies have been conducted to report the prevalence of hypoparathyroidism in patients with thalassemia major in Iran. However, there is no comprehensive assessment of it. Therefore, the present study was conducted to investigate the prevalence of Hypoparathyroidism in patients with thalassemia major in Iran through systematic review and meta-analysis. METHOD: The present study was conducted based on PRISMA checklist for systematic review and meta-analysis studies. We searched national and international online databases such as Magiran, Iranmedex, SID, Medlib, Scopus, PubMed, Science Direct, Cochrane, Web of Science, Springer, Online library Wiley and Google Scholar for certain standard keywords without time limit until 2016. FINDING: In 19 studies, 3219 patients with thalassemia major were examined. Prevalence of hypoparathyroidism in patients with thalassemia major in Iran was calculated to be 10% (CI-95%: 7-10). Moreover, the percentage for various regions of Iran was different; 14% in the west, 18% in the east, 10% in the north, 12% in the south and 7% in the center of Iran. Prevalence of Hypoparathyroidism in men and women was estimated to be 9 and 14, respectively. CONCLUSIONS: Prevalence of hypoparathyroidism in patients with thalassemia major in Iran is high. Therefore, new policy and planning are necessary to minimize the complications of endocrine glands including Hypoparathyroidism in patients with thalassemia major in Iran

Introduction: Although many studies have been conducted on thalassemia from different aspects, none of ‎them have yet identified the preferences of parents of patients with thalassemia major in ‎receiving health information. Parents of these patients need appropriate and timely ‎information to manage and resolve the problems of their children and family. This study ‎aimed to identify the preferences of these parents in obtaining information to manage their ‎children’s health. Methods: This was an applied, descriptive, and cross-sectional research. The study was conducted ‎through interviews with the parents of patients with thalassemia major who were admitted to ‎the pediatric ward of a hospital affiliated to Semnan University of Medical Sciences, Iran, in ‎‎2014. The data collection tool was a valid and reliable researcher-made questionnaire (Cronbach’s alpha = 0.87). The data were analyzed using descriptive statistics and the ‎Friedman test. Results: According to the results, 39.6% of the parents reported obtaining information about mental ‎health problems as their first priority, while 56.6% of the parents reported obtaining ‎information about the side effects of Desferal as their first priority. In addition, 56.6% of the ‎parents preferred that the information be given to the patients’ father. Conclusion: The results indicated the type of information parents prefer to obtain first. Informational ‎support can help the parents better manage their children’s illness. Transference of valuable ‎knowledge through several information sources to the parents can lead to improvement of ‎health in the society and parents feeling that they are socially supported.

Introduction: Impaired glucose tolerance (IGT) is one of the most common endocrine complications in major thalassemia patients. IGT prevalence in patients with thalassemia major has been differently in different studies in Iran, about which no total estimate can be proposed. Hence, this meta analysis study aimed to investigate IGT prevalence in major thalassemia patients in Iran. Methods: This study was a systematic review based on electronic databases including Magiran, Iranmedex, SID, Medlib, IranDoc, Scopus, PubMed, Sciencedirect, Cochrane, Embase, Web of Science, Springer, Online Library Wiley as well as Google Scholar search engine without any time limitations up to 2015. All the potentially relevant papers were reviewed independently by two investigators using the standard key words. Then, all the reviewed studies that had inclusion criterion were evaluated. In order to statistically analyze the study data, random effect model was used via Stata software (Ver.11.1). Results: 1346 subjects were examined in 13 studies. IGT prevalence in patients with thalassemia major was estimated 9.6 %( CI: 95%, 6.6-12.5) in Iran. The IGT prevalence in male and female major thalassemia patients were 6.5 %( CI: 95%, 1.6-11.3) and 10.2 %( CI: 95% , 6.1-14.3), respectively. Conclusion: IGT Prevalence was demonstrated to be really high in patients with thalassemia major. As a result, implementing screening programs seems to be necessary in regard with early diagnosis of endocrine disorders especially IGT in these patients. Furthermore, it is recommended that annual glucose tolerance test be taken within such patients.

Background and Objective: Deferoxamin is the current “gold standard” chelator in comparison with new chelators. Combined therapy of Deferiprone and deferoxamin reduces the cardiac iron overload in patients with major talassemia. This study was done to evaluate the effect of defriprone-deferoxamine on heart function in patients with major thalassemia. Methods: In this historical cohort study, 8 patients with major beta thalassemia treated by subcutaneous deferoxamine were randomly selected and LVEF (the rate of blood that exited from heart in each beat) and serum ferritin were measeared. The patients were treated by deferiprone (50-100 mg/kg/day) compained with dferoxamine (30-50 mg/kg as 3 times in a week). In the end of each year, LVEF and serum ferritin of patients were measured. Results: The ferritin level changed from 3243.12 in the first year to 2672.75 mg/kg at the end of third year. The mean of LVEF changed from 71.12% to 64.62 %. The correlation of serum ferritin and LVEF only at the end of third year was significant (P

Background and Objectives Estimation of the frequency and causes of immunization to red blood cells in transfusion dependent thalassemia (TDT) patients can help us know the limitations of current practice and plan specific measures to reduce it. This study aimed to evaluate the frequency and causes of immunization in TDT patients. Materials and Methods This case control study was conducted on 732 (TDT) patients between 20 November 2015 to November 2016 in Dastgheib Hospital, Shiraz, Iran. Direct coombs test, antibody screening, and antibody identification were performed for every TDT patient. Then, the frequency and type of antibodies were surveyed. Clinical and laboratory data of immunized and nonimmunized patients were compared. Results All of the case group had positive results in direct or indirect coombs tests. Indirect coombs test (ICT) was positive in 59 patients (63.4%) while direct coombs tests (DCT) was positive in 41 (44%) of immunized patients. In addition, 7 patients (7.52%) showed positive results for both DCT and IAT. The most frequent antibodies were against Kell (48.6%), D (22.7%) and E (18.2%). Our results reflected that lower age of starting transfusion and splenectomy had an impact on higher immunization rate. Conclusions Antbodies against Rh and Kell system were the most frequent antibodies. It seems that extended RBC matching at least for ABO/Rh/Kell antigens must be performed for thalassemia patients with its effect on RBC immunization rate to be then surveyed. [ABSTRACT FROM AUTHOR]

Background and Objectives Immune abnormalities are the fourth leading cause of death in thalassemia major patients. These patients are affected by stimulating chronic immunosuppression and immune deficiency following repeated blood transfusions and iron overload. Considering the role of chemokine network in immunosuppression, we examined the role of CCL-5 chemokine and its receptor CCR-5 in β-Thalassemia major patients. Materials and Methods In the present case/control study, 45 β-thalassemia patients having referred to Kerman Special Health Center and 45 healthy subjects as controls participated. For the expression of CCR-5 and CCL5 receptor expression, flow cytometry and ELISA were used, respectively. After the tests, T-test and SPSS software version 22 were used for analysis and p < 0.05 was considered as a significant difference. Results Our results indicated that both CCR5 and CCL-5 were induced in β-thalassemia patients compared to the control (p < 0.05). Conclusions This study showed the increase of CCL5/CCR5-receptor chemokine-centered axis in patients with thalassemia major compared with healthy subjects and similarly the very increase in patients with spleen compared with those without. Due to the fact that a study on this chemokine network has not been done so far, it is necessary to repeat this study with more samples and to carefully examine the role of the spleen. [ABSTRACT FROM AUTHOR]

Background and Aim: Thalassemia is a chronic disease which is extremely expensive, complex and debilitating. The management skill of thalassemia patients should be enhanced to minimize the risk of disease complications. The main purpose of this study was to develop personal electronic health records for thalassemia major patients. Materials and Methods: This is a developmental applied study which was conducted to develop a personal electronic health record for thalassemia major. First, a questionnaire was prepared to determine the data elements and was filled by Hematology and Oncology professionals in the country (110 persons). Then, based on the results of needs analysis, the system was designed using PHP programming language and MySQL database and was evaluated by 50 thalassemia patients who referred to the Thalassemia Clinic of Bu Ali Sina Hospital of Mazandaran University of Medical of Sciences during the second half of the month of Aban. Finally, a standard questionnaire of usability and user satisfaction assessment was distributed among them. Results: Usability evaluation of the system showed that patients evaluated the system at a good level with a mean rating of 7.91 (out of 9 points). Conclusion: The web-based systems can be used to help thalassemia patients to control injection and reduce the complications of the disease and to promote health. [ABSTRACT FROM AUTHOR]

ABSTRACTBACKGROUND AND OBJECTIVE: In transfusion dependent thalassemic patients, if their transfusion requirement increases over 20 ml/Kg of whole blood, splenectomy is indicated. Portal vein thrombosis (PVT) is one of life threatening complications of splenectomy. Follow up of splenectomized patients in order to the earliest diagnosis and treatment of PVT can improve the survival of them. The aim of this study was to assess the frequency of PVT in splenectomised thalassaemia patients.METHODS: This cross sectional study was performed on 63 splenectomized thalassemic patients who referred to Yahyanejad hospital of Babol for follow up during 8 years (from June 2001 to June 2009). Patients were evaluated for age, gender, type of operation, splenectomy indication, spleen size, operation duration, clinical manifestation, PVT frequency, and time interval of splenectomy and occurrence of PVT, Color Doppler Sonography findings, PVT treatment and follow-up.FINDINGS: PVT was identified in 6 (9.5%) patients (4 females and 2 males)(95% CI: 2.1-17). The mean time of patient’s follow up was 5.26±2.7 years. Average of surgery duration was 2.72±0.8 hours. The average of time interval of splenectomy and occurrence of PVT was 229±176 days. All of the patients treated by low molecular weight heparin during hospitalization and discharged by Warfarin for 4 months. Two patients received propranolol at the time of discharge, too.CONCLUSION: The results of this study show that early diagnosis of PVT by following up the patients who splenectomised and treatment of PVT with anticoagulant can reduce the adverse of this complication.

BACKGROUND AND OBJECTIVE: Thalassemia is the most common hereditary disease. Hypersplenism and splenomegaly occur in thalassemic patients due to the hereditary defect in globin chain of hemoglobin molecule and extramedullary hematopoiesis that results in more destruction in red blood cells. Therefore, these patients need to splenectomy to reduce recurrent blood transfusion and its complications. The aim of this study was to compare the need of transfusion and adverse effects before and after splenectomy in beta-thalassemic patients referred to Yahyanejad hospital (Babol, Iran).METHODS: This cross sectional study was performed on 63 splenectomized thalassemic patients who referred to Yahyanejad hospital in Babol during 8 years. Demographic information, kind of thalassemia, clinical symptoms, spleen size, number of transfusions, and platelet and red blood cell count were evaluated before and after splenectomy in these patients.FINDINGS: In this study, 37 (58.7%) out of the 63 cases had thalassemia major and 26 cases (41.2%) had thalassemia intermedia. Eleven, 21, 9 and 17 cases had abdominal pain, anorexia, fever and gall stone, respectively. These patients had undergone cholecystectomy simultaneously. Six cases had portal vein thrombosis and 5 patients had infectious complications in long time post splenectomy. Mean operation time and mean hospital stay was 2.70±0.73 and 6.26±2.14. Mean white blood cell count, hemoglobin level, platelets and red blood cells (RBCs) count were raised significantly after splenectomy (p

Introduction: Thalassemia major is one of the most common chronic blood disorders that is characterized by reduced or lack of production of one or more globin chains. Treatment with transfusion programs, chelating therapy has considerably prolonged survival in thalassemic patients. Studies have demonstrated that chelation therapy regularly can prevent from disorders in growth of thalassemic children. This study intended to determine the effect of defroxamine injection on physical growth in children with Thalassemia major. Methods: This is a descriptive-analytical investigation in which 500 children with thalassemia major were enrolled by using systematic random sampling. The patients were the ones who attended to Shiraz transfusion centers and were divided into two groups of defroxamine group who had defroxamine injection before and the control group in which the patients did not receive any defroxamine injection. Data were collected by measuring the height, weight, arm and head circumference and interviewing with parents of children. SPSS software was utilized to analyze the data through inferred statistic tests(chi-square and variance analysis). Results: The results indicated that 62.8 percent of patients have regular defroxamine injection. There was a significant difference between two groups in regard to the mean height, weight and arm circumference(P=0. 001) and head circumference(P=0.02). Conclusion: The study findings demonstrated that chelation therapy will be effective in promoting the physical growth of children with Thalassemia major specially during the first years of life. It is recommended that patients receive regular blood transfusion with chelation therapy.

Background: Frequent transfusion of blood and blood products to patients with thalassemia major and hemophilia and under hemodialysis patients may cause infections such as hepatitis infection in them. This study aimed to determine the prevalence of hepatitis C and associated risk factors in these patients in Isfahan, the second big province in Iran. Methods: In a descriptive study, all the patients with hemophilia and thalassemia and under hemodialysis in Isfahan province were enrolled. A questionnaire, including demographic and risk factors of hepatitis C was completed through a structured interview with closed questions by a trained interviewer for each patient and HCV-Ab test results were extracted from patient records. Finding: In this study, 60of 570 patients with thalassemia major (10.5%), 232 of 350 patients with hemophilia A and B (66%) and 17 of 800 under hemodialysis patients (2.1%) were positive for hepatitis C. Based on Multivariate Logistic Regression model, no independent risk factor was found. Conclusion: Prevalence of hepatitis C in patients with recurrent blood transfusion in Isfahan is high. Since no independent risk factor for hepatitis C disease was found in these three groups, it can be concluded that multitransfusion is the only predictor for hepatitis C.

مقدمه: مطالعات زیادی در زمینه تأثیر تالاسمی ماژور بر استخوان‏های ناحیه کرانیوفاشیال انجام شده است اما تأثیر این بیماری بر تکامل دندان‏ها کمتر مورد مطالعه قرار گرفته است. هدف از این تحقیق بررسی میزان تکامل دندان‏ها و همچنین شیوع آنومالی های دندانی در بیماران بتاتالاسمی ماژور و مقاسیه آن با گروه شاهد بود. مواد و روش ها: در این مطالعه مورد و شاهد که مسائل اخلاقی آن مورد تایید و تصویب کمیته اخلاق معاونت پژوهشی دانشگاه علوم پزشکی شیراز رسیده است، رادیوگرافی های پانورامیک 120 بیمار تالاسمی ماژور بررسی شد. بیماران شامل 68 پسر و 52 دختر با میانگین سنی 66/2±98/12 سال، در محدودهسنی 19-8 سال بودند و 120 نفرگروه شاهد از نظر سن و جنس، با گروه مورد یکسان‏سازی شدند. رادیوگرافی ها از نظر وقوع مواردآنومالی و همچنین تعیین سن دندانی بررسی شدند. آزمون دقیق فیشر برای مقایسه شیوع آنومالی های دندانی در دو گروه مورد و شاهد استفاده شد. آزمون Paired t-test، سن دندانی و سن تقویمی را در هر گروه مقایسه نمود. سن دندانی گروه مورد و شاهد نیز با استفاده از Independent t-test با یکدیگر مقایسه شدند. یافته ها: شیوع میکرودونشیا، دندان‏های با ریشه کوتاه و میخی شکل، تارودونتیسم و کمبود دندانی در بیماران تالاسمی بیشتر از گروه شاهد بود. بین میانگین سن دندانی و سن تقویمی بیماران تالاسمی و همچنین بین میانگین سن دندانی بیماران تالاسمی و افراد گروه شاهد، اختلاف آماری قابل ملاحظه‏ای مشاهده نشد. نتیجه گیری: بر اساس مطالعه حاضر آنومالی های دندانی در بیماران تالاسمی در مقایسه با افراد نرمال شایع تر است. اما سیر تکاملی دندان‏ها و زمان رویش آنها تحت تاثیر بیماری تالاسمی قرار نمی گیرد.

Background and Objective: Thalassemia major is a genetic disorder. Blood transfusion is critical for survival in these patients. Over the course of the past two and three decade’s hyper transfusion therapy in these patients has increased significant improvement in life expectancy and quality of life. Unfortunately this type of therapy increased the frequency of complication due to iron overloud. The aim of this study was to evaluate the prevalencey of diabetes, impaired fasting glucose and impaired glucose tolerance in patients with thalassemia major, with 10-27 years of age in Tabriz. Materials and Methods: This descriptive study was done on 56 patients between 10-27 years of age with thalassemia major. The demographic informaiton theraputic regiment, the age of first trasfussion. The level of blood transfusion, the history and dosage of familial history of diabetes, Fe, TIBC, ferritin levels were assessed and recorded. For each patient glucose tolerance test, blood glucose level are performed. Results: In this study prevalence of diabetes mellitus, impaired fasting glucose and impaired glucose tolerance test were found in 8.9%, 28.6% and 7.1% of patients respectively. Conclusion: This study showed that despite recent therapy with Desferal in the management of beta-thalassemia major, the risk of secondary endocrine dysfunction remains high. Prevalence of diabetes mellitus, impaired fasting glucose and impaired glucose tolerance test are greater than general population. Endocrine evaluation in patients with thalassemia major must be carried out regularly especially in those patients over the age of 10 years.

AbstractBackground and Purpose: Thalassemia is a hereditary quantitative hemoglubinopathy which is common in mediteranian area including IRAN. Homos zygotic thalassemia patients suffer from severe anemia and complication of the disease in many organs. Studies have shown different results about renal complication and disease. Thus, in this study we investigated renal function of thalassemia Major (TM) patients in comparison with control group.Materials and Methods: This was a historical cohort Study. The population who TM patients was were admitted to Boalisina hospital, Sari, and control group were brothers and sisters of the patients who were matched in gender and age. Serum and urine markers of renal function were measured and demographic and therapeutic data were gathered from medical records. Analysis of the data was performed using SPSS 11 with statistical test (t, chi square).Results: the Total of 84 (42 patients and, 42 controls) patients were studied. The Mean age of the patients was years. Dose of Deferral was 70±19 mg/kg. The results showed no significant statistical differences in levels of microglobulin, 24 urine protein, Excretion Fraction of Na and K between case and control group. There was significant differences in levels of serum BUN, creatinin, Potassium and urine potassium and creatinin between case and control group. Gender, level of Hb and serum Ferritin significantly affected the differences between two groups.Conclusion: In this study, evidences of renal tubular damage were not detected in TM patients. There was increase in levels of Bun, serum potassium, uric Acid, specially with sever anemia, high dose desferal and Iron over load.

Background and aims: Hypogonadism is one of the most common endocrine complications in patients with thalassemia major. A simple review of studies show different hypogonadism prevalence and has been reported between 31.3-88% in Iranian patients with thalassemia major. So, this meta-analysis study was conducted to determine the hypogonadism prevalence in Iranian patients with thalassemia major. Methods: This study was conducted based on PRISMA checklist for systematic review and meta-analysis studies. A comprehensive search by two researchers was conducted according to MESH keywords in databases such as Magiran, Iranmedex, SID, Medlib, IranDoc, Scopus, PubMed, Science Direct, Cochrane, Embase, Springer, Online Library Wiley and also search engine Google Scholar. Then, all articles were examined without any time limited until October 2015. Data were analyzed using random effects model and Stata ver. 11.1 software. Results: Sixteen studies involving 2938 patients with average age of 17.34 were identified to analyze in the meta-analysis. Hypogonadism prevalence in patients with thalassemia major in Iran has been estimated 42.3% (CI 95%: 30.7-53.8). Minimum and maximum prevalence in Iran were related to the center (41%) and east (45%), respectively. Hypogonadism prevalence in male and female patients was 49% and 45.2%, respectively. There was no significant relationship between hypogonadism prevalence and year of the study and sample size. Conclusion: The prevalence of hypogonadism in Iranian patients with thalassemia major is high and it is needed to perform a continuous and regular plan and follow up for these patients. [ABSTRACT FROM AUTHOR]

Background and purpose: Diabetes mellitus is one of the most common endocrine complications in thalassemic patients. Several studies investigated the prevalence of diabetes in Iranian patients with thalassemia major and reported different results but no overall estimate was calculated. Therefore, we conducted a meta-analysis to study the prevalence of diabetes in patients with thalassemia major in Iran. Materials and methods: This review study was conducted by PRISMA checklist. Electronic databases including Magiran, Iranmedex, SID, Medlib, IranDoc, Scopus, Pubmed, Science direct, Cochrane, Web of Science, Springer, Online Library Wiley and Google Scholar were searched in chronological order without time limit, in January 2016, using relevant keywords. Two researchers independently performed article selection and data extraction. All studies meeting our inclusion criteria were investigated. Data analysis was done using random effect model in Stata Ver.11.1. Results: Overall, 6380 patients were studied in 42 articles. Prevalence of diabetes in patients with thalassemia major in Iran was 9.5% (CI: 95%, 7.8-11.3). The minimum and maximum prevalence of diabetes were found in West (10.3%) and North of Iran (8.3%), respectively. The prevalence rate in male and female thalassemia major patients were 12.6% (CI: 95%, 6.1-19.1) and 10.8% (CI: 95%, 8.2-14.5), respectively. Conclusion : Prevalence of diabetes in Iranian patients with thalassemia major was found to be high. Therefore, routine blood tests should be done every six-month for early diagnosis of diabetes in this population. [ABSTRACT FROM AUTHOR]

Background: Subcutaneous deferoxamine is the first line for management of iron overload in thalassemia major. There is a wide deferoxamine adherence variation in patients with thalassemia major. In order to assess the effect of education on patients and their parents, a cognitive approach was used. Methods: In a randomized control trial, 78 consecutive patients with thalassemia major were randomly divided into educational (38 cases, 50% male and 50% female) and control (40 cases, 65% male and 35% female) groups. Oral and practical education sessions for deferoxamine injection were planned. Deferoxamine usage, pump function, side effects, knowledge levels of the patients and their parents, and ferritin serum levels were assessed before and after the trail. The knowledge was also evaluated promptly after educational sessions. Results: the mean age of experimental and control groups were 14.6 and 13.0 years, respectively. There was no significant difference in age, sex and weight (P>0.05) at the end of the study, there was no significant change in the level of knowledge in both groups however, the patients who used deferoxamine at least 5 times a week were increased in the experimental group (P0.001). Conclusion: A cognitive approach had relatively significant effect on deferoxamine adherence. However, educational sessions had not dramatic change in knowledge levels of the patients and their parents, but oral and practical education produced a significant change in decreasing local side effects of subcutaneous deferoxamine in thalassemia major.

Background & Objective: Major ?-Thalassemia is the most common form of anemia, which has a relatively high prevalency especially in the northern part of the country one of this disease side effect the hearing abnormality. This study has been carried out in Gorgan for the determination of the hearing level of ?-Thalassemic patients, and its relation with the level of serum Ferritin, the rate and the duration of blood transussion and dyspheral. Materials & Methods: In this study 95 patients with major ?-Thalassemia have been studied for the rate of hearing level. The variation parameter include age, gender, Ferritin level, the rate and duration of disferal consumption. Audiometry, tempanometry and physical examination carried out on all the patients. The findings from this research gathered and were analyzed using the SPSS statistical software. Results: 95 patients (190 ears) with age 3-29 year of old were gone under this study and only 72 ears had the threshold over 15 decibel, from this 43.9% were from sensorineural type of hearing. The 80% of ears’s thempograms were type A. The results from this study showed that there is a meaningful statistical correlation between the hearing loss and serum Ferritin level. The rate of dyspheral consumption, in each time and its duration (P

Objective(s): To evaluate bone mineral density (BMD) in children and young adults with thalassemia and its potential risk factors. Methods: Two hundreds and twelve thalassemic patients aged 10-20 were enrolled in the study. In all patients height, weight, body mass index, growth indices, pubertal development, calcium intake and serum ferritin were assessed. BMD was determined by dual-energy X-ray absorptiometry (Lunar). BMD z scores between -1 and -2.5 were identified as low bone mass and z scores less than -2.5 were considered severely low bone mass. Results: Prevalence of severely low bone mass was 50.7% in lumbar spine, 10.8% in femur and 7.9% in both areas. There was no statistically significant between males and females. Low bone mass was observed in 39.5% and 37.5% of patients in lumbar and femoral areas. Patients with severely low bone mass in lumbar spine were older than the others (P

Background and Objective: The life time of patients with Thalassemia major has been considerably increased in recent years. Long-term complications such as liver and heart complications that caused by hemosiderosis create some problems for these patients. This study was done to evaluate the long-term effects of this disease on lung tissue by pulmonary function test. Methods: This analytical study was performed on 91 normal persons and 100 patients with Thalassemia (Over 5 years old) who referred to Thalassemia research center. Both groups were matched according to their gender and age. After physical examination, ferritin was measured and chest X-ray and spirometry were taken for all persons in both groups. Parameters of pulmonary function test between two groups were compared and P

Background and Objective: The defensive role of saliva is mainly played via immunoglobulins. Periodontal infections are the most common diseases in oral cavity and therefore defensive function of saliva against these infections are important. With regard to the effects of systemic diseases on defensive function of saliva, the objective of this study was to survey the relationship between gingivitis and salivary immunoglobulins in patients with Thalassemia major. Methods: This study was done on 55 children (Between 5-12 years old) with Thalassemia major who referred to Thalassemia center of Amirkola children hospital. Of these, 30 cases with gingivitis and 25 without gingivitis were chosen as case and control groups, respectively. Questionnaires were prepared and then evaluation of gingiva condition was done. Saliva samples were taken and examined by ELISA. Findings: There was no significant difference between the rate of IgA, IgG and IgM in two groups. Also, there was not relationship between severity of gingivitis and the rate of salivary immunoglobulins. Conclusion: T-cells affect on the activity of B cells. With regard to reduction of activity of T-cells lymphocyte (CD4) in Thalassemia and the effect of these cells on promotion of B-lymphocyte function, B-cells not response to gingivitis by synthesis of immunoglobulins.

Objective: Congestive heart failure and other cardiac complications are the most important causes of death in patients with beta Thalassemia major. Iron overloads and chronic anemia are the major causes of cardiac involvement. The purpose of this study was to evaluate the ability of echocardiographic systolic function for diagnosis of cardiac involvement in patients with Thalassemia major. Methods: This descriptive study was done on patients with Thalassemia major (Over 12 years old) who referred to Booali Sina hospital in Sari. They were evaluated according to their ages, sex, age at beginning of transfusion of deferoxamine, the amount of deferoxamine, serum ferritin level, clinical manifestations, electrocardiogram, chest radiography and echocardiographic systolic function. Data was statistically analyzed by T-test. Findings: 55% of patients were male and the average age was 18±6 years. 22% of patients had systolic dysfunction. The difference of ejection fraction and fractional shortening between patients with and without cardiac involvement was significant (p

Background and Objective: Deferoxamin is the current “gold standard” chelator in comparison with new chelators. Combined therapy of Deferiprone and deferoxamin reduces the cardiac iron overload in patients with major talassemia. This study was done to evaluate the effect of defriprone-deferoxamine on heart function in patients with major thalassemia. Methods: In this historical cohort study, 8 patients with major beta thalassemia treated by subcutaneous deferoxamine were randomly selected and LVEF (the rate of blood that exited from heart in each beat) and serum ferritin were measeared. The patients were treated by deferiprone (50-100 mg/kg/day) compained with dferoxamine (30-50 mg/kg as 3 times in a week). In the end of each year, LVEF and serum ferritin of patients were measured. Results: The ferritin level changed from 3243.12 in the first year to 2672.75 mg/kg at the end of third year. The mean of LVEF changed from 71.12% to 64.62%. The correlation of serum ferritin and LVEF only at the end of third year was significant (P<0.05). Conclusion: Combined therapy of deferiprone-deferoxamine during 3 years reduces ferritin and LVEF in patients with major thalassemia. [ABSTRACT FROM AUTHOR]

Introduction: Thalasemia Major is one of the most common anemia diseases that can be fatal if not promptly diagnosed. The survival analysis of these patients can be an appropriate strategy in determining risk factors for death in these patients. The purpose of this study was to choose the best model to determine the risk factors for death in patients with the thalasemia major using common methods in the survival analysis. Methods: The data of this retrospective cohort study, with 296 patients with thalassemia major, was collected in 2004- 2013 in Zafar Clinic in Tehran. Akaicke information Criterion was used for comparison of the models and the choice 351of the best model. Data analysis was carried out with R3.0.2 software at the significant level of 0.1. Results: The values of the Akaicke information criterion (AIC) for the parametric weibull, frailty weibull, log-normal, log-logistic, Gompertz, gamma and the semiparametric Cox were computed and found to be 27.56, 29.56, 18.73, 23.39, 26.26, 68.10, 24.73, respectively. The mean survival time for men and women were 40.2 and 39.7 years, respectively. The Log-normal model showed that age, age at the first desferal injection, onset of blood injection, the patient's birthplace, mother's education variables were significantly correlated with patient survival. Conclusion: According to the values of AIC, the parametric log-normal model was chosen and suggested as the best model. [ABSTRACT FROM AUTHOR]

Thalassemia, the most common genetic disorder worldwide is a genetic disease in globin chain. Complete absence of beta chain is defined as thalassemia major. These patients suffer from severe hemolytic anemia. Iron overload and chronic immune-stimulation by repeated blood transfusions, splenectomy and alteration in immune system posse these patients to increased risk for severe and serious infections. Several component of immune system may affected by this disease. Increase in number and activity of suppressor T cells (CD-8) in addition to decrease in number and proliferative capacity of helper T-Cells (CD-4) leading to decreased CD4/CD8 ratio, as well as defective activity of natural killer (NK) cells. Immunoglobulin levels and count of B-lymphocytes were found to be increased. Neutrophil chemotaxy activity and macrophages phagocytosis capacity has been defective. Suppressed function of complement system both in classic and alternative pathways, with reduced levels of C3 and C4 has also been reported. Results of various researches about immune system in thalasemic patients are not conclusive and in a lot of reports are controversial. [ABSTRACT FROM AUTHOR]

Background and Objectives The severest form of thalassemia is beta thalassemia with regular need for blood transfusion and medical care. Treatments such as desferrioxamine injection and regular blood transfusion cause several complications in patients' vital organs including eyes. The vision is considerably more sensitive than other senses in perceptual-motor responses and it is also sensitive to details, depth, and color. As a result, it has a decisive role in controlling movement. This study has been conducted to draw a comparison of depth perception in healthy adolescents and patients with thalassemia major. Materials and Methods This method is semi-experimental and the sample was 18 thalassemic patients and 18 healthy subjects within the age range of 13-17 years (15.33 ± 1.37) who were selected through sampling. These subjects have been evaluated three times by the depth perception device made by the researchers; it has the capability to assess the depth perception with the smallest error (one ten thousandth cm). The average of these three time tests was used as depth perception score. Results Independent T-test results showed significant difference between healthy adolescents and patients with thalassemia major (t=-18.52, p< 0.0001, df = 34). The average error in the healthy adolescents was significantly lower (MD = 14.01). Conclusions The probable reasons that affect the loss of depth perception in thalassemia major were anemia, chronic hypoxia, inactivity or increased iron deposition in tissues of the eye. It requires a solution in this regard due to the importance of depth perception in human motor behavior. [ABSTRACT FROM AUTHOR]

Background and Objectives Beta-thalassemia major is a chronic, hematological disorder with psychological adverse effects on patients and their families. Parents, in particular mothers who are mostly the primary caregiver, are the ones to deal with the stress of their kid thalassemics and use different coping styles either adaptive or maladaptive. The aim of this study is to assess the relationship of coping strategies of mothers with the mental health of their thalassemic children. Materials and Methods In this cross-sectional study, out of the patients referring to the Thalassemic Clinic of Ahwaz Shafa Hospital, 36 patients with thalassemia major (12-16 y/o) and their mothers were selected by the convenient sampling method. The subjects were assessed using the demographic data questionnaire, the coping styles questionnaire (CSQ) (for mothers), and SCL90-R questionnaire (for children). The data were finally analyzed by the spss software. Results Out of the total number of the children with thalassemia major, 91.2% suffered from psychosocial problems. Among the mothers, 80.55% more frequently used the adaptive coping styles; a significant positive correlation was found between mothers' maladaptive coping mechanism and psychological problems of their children (p= 0.33)(r= 0.356). Conclusions Educational planning aimed at increasing the coping skills in mothers with thalassemic children can be useful in decreasing psychological problems of children. [ABSTRACT FROM AUTHOR]

Background and Objectives Thalassemia is one of the most common chronic genetic diseases whose psychological issues can affect quality of life in patients. Research has shown that regular physical activity can increase well-being in a person. This study compared the quality of life in patients with thalassemia major based on their participation in group activities in Bandar Abbas in 2011. Materials and Methods In this descriptive analytical study, 35 patients with thalassemia major having participated in group activities were selected as the case group and 75 patients not participating as the control. Data were collected with quality of life questionnaire SF-36 and were analyzed by using SPSS13 statistical software, Mann-Whitney and Kruskal Wallis tests. Results The difference between the quality of life scores in the case and control groups for dimensions of general understanding of health, role limitation due to physical health problems, energy and vitality, physical functioning, emotionally related role limitations, body pain, mental health, and overall quality of life score was significant. The mean values of overall quality of life score in the case and control groups were 81.40 ± 3.64 and 58.35 ± 6.14, respectively. The relationship between the overall quality of life score and participation in group activities, starting age for desferal medication, and the number of desferal injections per month was significant in the case group. Conclusions Patients should be encouraged to participate in group activities and rehabilitative centers should be constructed to improve the level of life skills so as to lead to greater patients independence. [ABSTRACT FROM AUTHOR]

Background: Frequent transfusion of blood and blood products to patients with thalassemia major and hemophilia and under hemodialysis patients may cause infections such as hepatitis infection in them. This study aimed to determine the prevalence of hepatitis C and associated risk factors in these patients in Isfahan, the second big province in Iran. Methods: In a descriptive study, all the patients with hemophilia and thalassemia and under hemodialysis in Isfahan province were enrolled. A questionnaire, including demographic and risk factors of hepatitis C was completed through a structured interview with closed questions by a trained interviewer for each patient and HCV-Ab test results were extracted from patient records. Finding: In this study, 60of 570 patients with thalassemia major (10.5%), 232 of 350 patients with hemophilia A and B (66%) and 17 of 800 under hemodialysis patients (2.1%) were positive for hepatitis C. Based on Multivariate Logistic Regression model, no independent risk factor was found. Conclusion: Prevalence of hepatitis C in patients with recurrent blood transfusion in Isfahan is high. Since no independent risk factor for hepatitis C disease was found in these three groups, it can be concluded that multitransfusion is the only predictor for hepatitis C. [ABSTRACT FROM AUTHOR]