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Background and purpouse : Thalasemia major is one the most prevalent congenital anemia. These patients recieved much rate of iron, due to frequent blood transfusion. Indocrine disorders are prevalent due to replacement of iron in indocrinal gland system, example can be of hypogonadism. In this study the delay of puberty in thalasemic patients of Gorgan city and its relevance to the rate of ferritin was evaluated.
Materials and Methods : This was a case control study conducted on 110 thalasemia major patients in the age group of 8-18 referred to Taleghani pediatric Hospital to receive blood. In 62 control group patients (31 females and 31 males) electeropheresis was done on their blood. The talassemic patients were divided into two groups on the basis of ferritin level. Those with ferritin level less than 1500 mg/dl (31 females , 18 males) In all the groups the stages of sexual puberty was calculated on the basis of Marshal-Tanner table with the 1-5 scale. For better studying of the groups of one and two, they were divided in subgroups of 8- 11, 12- 14, 14- 18 years of age.
Results : There was no significant difference of puberty in the control and case group of age 8- 11 years old. But there was a significant difference (P<0.01) in the case and control group of 12- 14 years old. And in the age group of 14- 18 result was different, that means, none of female case group had complete puberty while in the female control group all had complete puberty. In case of males, onset of puberty was less as compaire to control group and none had complete puberty.
Coclusion : This study showed that if the ferritin level in talassemic patient is not under control, it would lead to delayed sexual puberty in both sexes

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Background and purpose: The zinc deficiency has a known side effects and zinc deficiency in Ïron is a known phenomenon. Thalassemic patients under treatment with Ïron shlator i-e Dispheral, discharge some amount of zinc. Hence zinc deficiency is a matter of thought. Ïn order to determine the relation between dispheral usage and zinc deficiency this study was conducted on the thalassemic patients referring to sari BoÂli Sina Hospital in 1377.
Materials and methods: This was a cohort study. The test group comprised of confirmed thalassemia major cases under the treatment of blood transfusion and the control group comprised of sisters and brothers of these patients. 5 ml blood was drawn from the fasting patients, and the 24 hours urine was collected. Zinc was measured by RÜNDÔX kit with the help of atomic absorption instrument and the urine creatinine was measured by RÂ-1000 auto analyser. Signed rank test was used for the comparison of the age between these two groups due to differences in the obtained data.
Results: 32 cases under study were in 16 similar pairs. The rate of serum zinc in the control group was 98 ± 17 microgram and in dl case group was 112.7 ± 23 micro gram/dl (P<0.001). The serum zinc level compairing with the normal range of the used kit was normal in micro gr/dl both groups. The 24hrs. urine zinc level was 96 ± 12 and 120 ± 66 micro gr/dl in control and case group respectively (P<0.05). The urine creatinine level (mg/kg) was the same in the both groups.
Çonclusion: Desferal user thalassemia major patients excrete more zinc than the control group but this phenomenon would not lead to serum zinc deficiency. Probably due to frequent blood transfusion these patients receive significant amount of zinc. More study on the effects and the role of frequent blood transfusion on the serum and urine zinc level is recommended.

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Background and purpose: There are many cases of Thalassemia major in mazandaran province, and cholelithiasis is a known complication of all hemolytic anemias. Ïn order to evaluate the incidence of this complication in Thalassemia patients of Sari city, a descriptive study was designed and performed in two hundred thalassemia patients referred to Thalassemia control center.
Materials and Methods: The assessments were done by Ültrasonography by two radiologists. GË machine RT 2800 model of Ültrasound was used. The patients were such as kept in 14 hours fasting condition. Ïn sonography the informations such as liver size and Ëchogenecity, the shape and size of gall bladder wall, portal system, bile ducts spleen and portal system, as well as, sex and age were registered. The patients with previous history of stone formation along with cholelilithiasis were considered as positive.
Results: The total incidence of stone formation was 14% (15.4% in females and 11.5% in males) in these patients. There was a correlation between the incidence of cholelithiasis with increase in age of the patient.
Çonclusion: Due to the high incidence of this complication in thalassemic patients, it is recommended to include Ültrasonography in routine examinations of these patients, in order to prevent such complications in the thalassemic patients.

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Background and purpose: Beta-thalassemia major (TM), a chronic, genetically determined hematological disorder, has received little investigation on the psychological aspects of the disease and the psychosocial adjustment of patients with this anemia. In the present study, the aim was to explore the nature of psychopathology according to age, sex, school performance, severity and complications of the disease in TM patients compared with demographically matched healthy persons.
Materials and Methods: A controlled anterograde cohort study was conducted at the Thalassemia Unit of Boo-Ali Hospital from June 2003 to November 2005 in Sari, Iran. Psychological aspects were evaluated by the Persian version of symptoms checklist-90-revised questionnaire. Information on relevant demographic characteristics, school performance, severity and complications of the disease was collected by one of the investigators who had created the questionnaire.
Results: 125 persons with TM completed the questionnaires and were compared with 125 controls and 250 totally. The mean age of the participants was 18.51± 2.0 years and with a range of 15-25 years. 132 (52.8%) were female with equal family status, social and economic status. Patients group reported a significantly lower level of marital status (P<0.01), education level (P<0.0001), school performance (P<0.0001). TM patients were found to have significantly more psychiatric disorders than the control subjects with GSI: 1.16 ± 0.47 vs. 1.01 ± 0.6 (P<0.03), PSD: 54.99 ± 12.59 vs. 46.42 ± 18.76 (P<0.0001), and PSDI 2.02±1.02 vs 2.45 ± 2.22 (P<0.05). We recorded significant changes in the mean scores of somatization (P<0.0001), interpersonal sensitivity (P<0.0001), depression (P<0.003), anxiety (P<0.05) and psychoticism (P<0.03) in the TM patients as compared to the control subjects.
Conclusion: These findings show that beta-thalassemia major patients are at risk for psychiatric symptomatology and need appropriate psychiatric consultation.

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Background and purpose: Threshold contrast sensitivity could be considered as an important indicator of visual function in thalassemic patients. In this study the threshold of contrast sensitivity was investigated in patients with major and minor thalassemia and normal individuals. Material & methods: This cross-sectional study was conducted in 30 patients of thalassemia major (Desferal dependent), 30 patients of thalassemia minor and 30 healthy individuals as control group. After determining the best-corrected vision, contrast sensitivity was measured using Freiburg Vision Test at frequencies of 1, 5, 15 cpd at distance of 4 meters. The results were then analyzed using SPSS. Results: Contrast sensitivity was higher in patients with thalassemia major at all spatial frequencies (P<0.001), however, there was no significant difference between minor and healthy subjects. We found no correlation between transfusion duration, dose of chelator and Ferritin with contrast sensitivity. Conclusion: Patients with thalassemia develop visual disorders that could result in reduction of contrast sensitivity. Therefore, contrast sensitivity testing could be of great benefit in detecting early changes in the visual function of beta-thalassemic patients.

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Background and purpose: Thalassemia major is a hematic hereditary disease with high rate of incidence in Iran. Malnutrition and growth retardation is prevalent in these patients. This study was done to assess the nutritional status and blood indices in patients with β-thalassemia major. Material and methods: This cross sectional study was conducted in 72 individuals with thalassemia major aged 10-20 years. Systematic sampling was done in patients attending Mashhad and Sabzevar thalassemia clinics. Written informed consent was obtained from patients who were willing to participate in the study. Data was collected through the semiquantitative food frequency questionnaire which was completed by trained interviewers. Nutritional information was studied in Nutritionist IV. To measure blood indices 5 ml blood was drawn from the patients. Descriptive statistics, one sample t-test and Kolmogorov-Smirnov tests were performed in SPSS V.16. P<0.05 was considered as significant level. Results: In this study there were 55.6% female and 44.4% male. The mean BMI of patients were 19.05±3.74 and 18.99±3.7 kg/m2, respectively. Dietary assessment showed that the mean of macronutrient intakes were more than dietary standard values except for cholesterol and fiber. Also, intake of micronutrient, were less than dietary standard values except in taking B vitamins, calcium, phosphorus, potassium, and sodium. Values of Hb and Hct were found to be less than the standard level but serum ferritin was more than normal range. Conclusion: The findings showed that deficiency of micronutrients due to malnutrition is prevalent in thalassemia patients.

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Background and purpose: Patients with Beta thalassemia major need consistent blood transfusion from early years of life. Deferasirox is used as an oral chelating agent (once daily) to excrete excess iron. This study aimed to compare the efficacy of deferasirox twice daily and the usual once daily dosing. Materials and methods: This before after clinical trial was performed in 2013-2014 in patients who were at least 2 years of age and received only deferasirox as the chelating agent. All patients had received deferasirox for at least six month once daily. The last ferritin before entering the study and the mean deferasirox daily dose during the previous six months were considered as baseline ferritin and deferasirox dose, respectively. Laboratory tests were performed including CBC-diff, serum ferritin, Creatinine (Cr), Aspartate Aminotransferase (AST) and Alanine Aminotransferase (ALT) to evaluate the efficacy and safety of deferasirox. Results: A total of 21 transfusion-dependent patients (mean age: 21±6 years old) were included of whom 67% were male. The mean ferritin level decreased significantly from 1814± 922 ng/ml to 1472± 907 ng/ml (P= 0.02). There were no any significant changes in AST, ALT and Cr levels compared to baseline values. Conclusion: Twice daily dosing of deferasirox was associated with more decrease in ferritin level compared to baseline single daily dose values without any hepatic or renal adverse effects.

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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.

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Background and purpose: The electronic registry of patients with beta thalassemia major was developed in Thalassemia Research Center affiliated with Mazandaran University of Medical Sciences in order to provide an accurate database for researchers and health planners. This study reports the condition of patients registered in Mazandaran province till summer 2016.

Materials and methods: In this descriptive study, the following information was recorded in the registry system: epidemiologic data, clinical examination data, complications, routine laboratory test results, medications, new cases, and death. Descriptive statistics were used to summarize the data.

Results: Until the summer 2016, 1053 patients including 500 (47.5%) males and 553 (52.5%) females were registered in 14 hospitals. These were half of the number of patients receiving care by the aforementioned University.  Among the patients, 920 (87.4%) were transfusion dependent. Single patients included 54.2% (n= 571). The level of formal education was beyond high school in 29.8% (n= 314). Three hundred and fifty (33.2%) patients were employed. Splenectomy was done in 606 (58%) patients. Diabetes mellitus, hypoparathyroidism, and hypothyroidism were diagnosed in at least 12% (n= 120), 14.3% (n= 145), and 11% (n= 112), respectively. Cardiomyopathy was observed in 113 (11.2%) cases. The most common iron chelators medication was desferrioxamine, which was used alone (50.4%) or in combination with deferiprone (24.3%).

Conclusion: Current research showed that in spite of incompleteness of records at the electronic registry of patients with beta thalassemia major, a comprehensive electronic system could enhance the level of care provided for involved patients.

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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.
 

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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.