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OBJECTIVE: To identify the effectiveness and safety of thalidomide in transfusion-dependent beta Thalassemia Major (TDBTM) patients. METHODS: A comprehensive search of PubMed, Cochrane Library, and Embase was conducted between January 01, 2014, and April 17, 2024, using terms like “thalidomide”, “thalidomide”, “α- phthalimidoglutarimide”, “thalassemia”, “beta thalassemia”, “transfusion dependent thalassemia” using Boolean or wildcard operators. Studies published in English with an observational or experimental design, including more than 10 TDBTM patients treated with thalidomide for at least 3 months, were included. The review focused on patients of all ages and genders, evaluating the impact of thalidomide on transfusion requirements. The included trials, involved 780 participants (age range=1.5-27.2 years), showing improvements in hemoglobin, fetal hemoglobin (HbF), serum ferritin, spleen size, and quality of life. National Institute of Health tool was used for quality assessment. RESULTS: After screening 19462 records, 18147 remained after duplicates were removed. Of these, 18138 were excluded, leaving 09 studies for inclusion in the review. Conducted in Pakistan, China, India and Iraq (2014-2024), the studies included a single-arm trial, a double-blinded RCT, and an open-label RCT and Pre-post enrolling 780 participants. Thalidomide (50-150 mg/day) improved hemoglobin, HbF, serum ferritin, spleen size, and quality of life, with a mean follow-up of 13 months. Thalidomide therapy resulted in transfusion independence in 69.6 % (n= 543) patients. Adverse effects were reported in 41.2 % (n=322) patients. Study quality was rated as good. CONCLUSION: Thalidomide is a well-tolerated, safe, and effective treatment for TDBTM patients, but these findings require confirmation through well-designed clinical trials.

OBJECTIVE: To investigate the relationship between repeated blood transfusions and growth retardation in children with transfusion-dependent Thalassemia in Peshawar, Pakistan. METHODS: This cross-sectional study was conducted on transfusion-dependent thalassemia patients receiving treatment at the Fatimid Foundation, Peshawar Pakistan, between February and August 2022. Participants were categorized by age (2 years) to assess and measure growth patterns over six months. RESULTS: Out of 93 children with thalassemia major, 55 (59.1%) males and 38 (40.9%) females. Mean age of the participants was 10.86 ± 5.72 years, and mean age at their first transfusion was 8.13 ± 5.78 months. Mean body mass index was 16.38 ± 1.82 kg/m². Short stature was observed in 49 patients (52.7%), of whom 57.1% (n=28) were male and 42.9% (n=21) were female. Serum ferritin levels were significantly elevated in patients with short stature, with 57.1% (n=28/49) having ferritin levels >4001 μg/L compared to 40.9% (n=18/44) with normal stature. Only 3.2% of patients had normal ferritin levels. The ROC analysis identified a ferritin cut-off of 1636 μg/L for predicting short stature (sensitivity 86%, specificity 68%). Growth assessment showed that 71% of the children were

Background: The immune response of thalassemia patients is different from usual; therefore, thalassemia patients are susceptible to infection. A study at Thalassemia Center in Cipto Mangunkusumo Hospital showed that all thalassemia patients experience zinc deficiency. The decreased cellular immune response is associated with zinc deficiency, whereas splenectomy exacerbates the condition. This study aims to evaluate the improvement of cellular immune response in splenectomized thalassemia major patients after zinc supplementation. Method: Randomized double-blinded controlled trial was conducted on splenectomized thalassemia major patients in 12 weeks period. The inclusion criteria were aged > 12 years and had negative HIV test results. The subject receiving corticosteroids were excluded. Fifty-six subjects were randomly divided into two groups, the zinc group and the placebo group. Between 2 groups, data on zinc serum, T lymphocyte count, CD4+ T lymphocyte count, CD8+ T lymphocyte count, and CD4+/CD8+ ratio were evaluated at the beginning and the end of the study and were analyzed with unpaired t-test, Mann Whitney test, and Wilcoxon Signed Rank test. Result: After zinc supplementation, only 18 of 28 subjects in the zinc group recovered normal zinc serum levels. There were no significant changes after zinc supplementation in all parameters of cellular immune response (p > 0.05) between the two groups. This might be due to the subjects' adherence, which was lower in the zinc group (75.82%) than in the placebo group (83.19%). Conclusion: The effect of zinc supplementation on cellular immune response in splenectomized thalassemia major patients had not been proven yet.

Abstract Background and Aim Due to the adverse effects of their disease, patients with thalassemia major face many problems such as a lack of proper growth, enlarged spleen and liver, bone disorders, especially the bones of the head and face. As a result of the long treatment process of this disease, other aspects of their life are also affected. The physical and mental problems in this population of patients will lead to frustration and lower social performance and quality of life. The present study aimed to explore the effect of an educational intervention based on the PRECEDE model on quality of life of adults with thalassemia major in Hormozgan province. Methods The present quasi‐experimental study was conducted on 234 adults with major thalassemia who visited the thalassemia medical centers in Hormozgan province in 2022. The participants were selected through a simple randomization from two cities of Hormozgan province (Bandar Abbas as the intervention group and Minab as the control). Each group had an equal number of participants (n = 117). The data were collected using a demographic information questionnaire, a researcher‐made questionnaire based on the model constructs and the SF‐12 quality of life questionnaire. The instruments were once administered before the intervention and once again 4 months after the intervention. The educational intervention included 3 sessions a week, each 60 min in length, shared in WhatsApp and Telegram. The data were analyzed in statistical package for the social sciences version 24 using Mann−Whitney U‐test, chi‐square test, Wilcoxon's test, and analysis of covariance. Findings The results showed that after the educational intervention, the mean scores of knowledge, attitude, self‐efficacy, enabling afctors, reinforcing factors and the mean score of overall quality of life increased significantly in the intervention group compared to the control (p

Background: Thalassemia major is a common type of genetic disorder found in improvement in the medical field, the life expectancy of these children was also increased so there arises a need to promote the quality of life of such patients. A lot of studies concentrate on various aspects of the life of thalassemia major, but no study was done to assess the physical fitness of such children which is quite important to regularize their exercise sessions. Objectives: The objective of this study was to find out the physical fitness of children with thalassemia patients. Materials and Methods: Nonexperimental study designed with 20 individuals diagnosed with thalassemia in major age groups between 7 and 18 both boys and girls. The outcome measure used in this study was 6-min walk test (6 MWT). Results: The mean distance covered in 6 min was 351.3 m on average. There was increase in heart rate and blood pressure after completion of the test, whereas oxygen saturation was found to drop post-6 MWT among these patients. Conclusion: Thalassemia major patients are found with this physical fitness when assessed with 6 MWT.

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: Pulmonary hypertension (PH) is associated with an increased risk of cardiac failure among asymptomatic patients with beta-thalassemia. This study aimed to determine the prevalence of PH and associated risk factors in patients with beta-thalassemia major (TM) and beta-thalassemia intermedia (TI) in Hormozgan province, Iran. Materials and Methods: Eighty-nine patients, including 65 TM and 24 TI, were enrolled in this cross-sectional study. The data regarding the gender, age, history of splenectomy, and type of chelating therapy were collected, and then hematocrit, hemoglobin (Hb), and serum ferritin levels were determined. Doppler echocardiography was performed for patients, and tricuspid regurgitation jet velocity (TRV)≥2.5 m/s was used to screen PH. Eventually, the data were analyzed using SPSS. Results: The prevalence of TRV≥2.5 m/s was 21.3% in all patients, indicating that it was not significantly different between patients with TM (21.8%) and TI (20.8%). There was a significant association between the prevalence of PH and the male gender. Based on the results, TRV≥2.5 m/s was observed in both children and adults. No significant association was detected between Hb, serum ferritin levels, and splenectomy with PH prevalence. Conclusion: The findings revealed that PH is common in both children and adult patients with TM and TI in our population and should be considered to choose the optimal treatment of patients with TM and TI.

Objective To evaluate the safety and efficacy of the collection of peripheral blood stem cells (PBSCs) in pediatric patients with thalassemia major (TM) weighing 20 kg or less. Methods PBSCs collection data of 170 pediatric patients with TM weighing 20 kg or less from January 2013 to December 2020 in our center were reviewed. Safety was assessed by the occurrence of adverse events during apheresis procedures, and efficacy was evaluated by the number of CD34+ cells collected. Results A total of 171 PBSCs procedures were performed on 170 patients with TM weighing 20 kg or less, with a median age of (4.98±1.53) years and a median weight of (17.30±2.18) kg. The probability of collecting at least 1×106 CD34+ cells/kg during a single course of apheresis was 99.41% (169/170), with a median (5.88±4.23) ×106 CD34+ cells collected per kg of weight of the recipient. A minimum pre-apheresis hemoglobin (Hb) of 60 g/L in patients with TM weighing 20 kg or less was safe and feasible. The most common adverse event of G-CSF mobilization in TM patients is bone pain, with the incidence of 7.65% (13/170), which was higher than that of healthy children donors in our center. The most common adverse events during the collection were pain at the puncture site of the femoral vein (6.47%, 11/170) and low pressure of the fluid (2.92%, 5/170). And no serious complications related to PBSCs mobilization, central venous catheter(CVC)placement or the apheresis procedure occurred. Conclusion PBSCs collection by COM.TEC blood cell separator in children weighing 20 kg or less is safe and efficacious.

Statement of the Problem: Iron overload in β-thalassemia major leads to oxidative damage to tissues, which may have an important role in the onset and progression of oral diseases.Purpose: The aim of this study was to evaluate the salivary oxidative stress indicators, total protein, iron, and pH in children with β-thalassemia major and their relationship with the status of dental caries in comparison with healthy children.Materials and Method: In this case-control study, 68 β-thalassemia major and healthy children, who were age- and sex matched, were selected. Two mililiters of saliva was collected from each child. The pH was measured using pH meter paper. Thiobarbituric acid reactive substances (TBARS) as salivary lipid peroxidation index, total antioxidant capacity (TAC), total protein, and iron were measured by spectrophotometry. Data were analyzed by SPSS ver. 22 software with Pearson and independent samples t-test.Results: TBARS, TAC, iron and dmft index in the β-thalassemia major group were significantly higher and pH was significantly lower than the control group (p< 0.001). The total protein difference between the two groups was not significant (p= 0.081).Conclusion: Considering the higher salivary TBARS in the β-thalassemia major group, oxidative stress can be considered as a risk factor for dental caries in children with β-thalassemia major. Prescription of antioxidant supplements especially natural antioxidants in the diet of children with β-thalassemia major is recommended to reduce oxidative stress.

Objective: To study the role of Hydroxyurea in patients suffering from β-thalassemia major. Study Design: Prospective longitudinal study. Place and Duration of Study: Department Paediatrics, Combined Military Hospital, Malir Cantt, Karachi Pakistan, from Nov 2019 to Oct 2020. Methodology: Data from 73 beta-thalassemia major patients was collected using a structured questionnaire containing inquiries about basic socio-demographic details, disease history, laboratory reports and particulars of former treatment regimens. Hydroxyurea was administered, and its effects were noted among the subjects regarding the frequency of transfusion, haemoglobin levels and severity of clinical symptoms. Results: The mean age of study subjects was 9.15±3.99 years, and most study subjects (56.14%) were boys. The preintervention (before administration of Hydroxyurea) levels of Hemoglobin (Hb), mean corpuscular volume (MCV), mean corpuscular haemoglobin concentration (MCHC), and serum ferritin levels were unremarkable when compared to postintervention levels. However, a significant difference was observed in HbF levels. In addition, an encouraging decrease was reported in the self-rated severity (using VAS) of symptoms such as fatigue (p0.05), and shortness of breath (p>0.05). In addition, the transfusion interval was significantly reduced (p

Abstract Background and aims Thalassemia syndromes are the most common hemoglobinopathy globally related to blood transfusion and iron overload in the body. Splenectomy, excessive iron overload, and repeated exposure to antigens in blood transfusions can cause severe damage to the patient's immune system making the patient prone to frequent infection. This study evaluates the immune system status and infection rate in beta‐thalassemia major patients receiving iron chelators. Methods This descriptive cross‐sectional study was performed in Rasoul‐e‐Akram Hospital on patients with a beta‐thalassemia major who had iron overload due to frequent blood transfusions. The percentage of lymphocyte markers was determined by flow cytometry. Serum levels of immunoglobin were measured by nephelometric assay. Also, Nitro blue tetrazolium and dihydrorhodamine assays were used to evaluate the phagocytic function. Results Of the 106 patients participating in this study, 59 (55.7%) and 47 (44.3%) are male and female, respectively. The mean age ± SD of participants was 24.7 ± 12.1 years with 4 to 55 years. There was no significant correlation between sex, the C3 and C4 complements, the lymphocyte markers, and the immunoglobulin levels. Furthermore, all of these variables increased significantly over 30 (p

Background Endocrine disorders in thalassemia major children patients occur due to iron overload and hemosiderosis in endocrine organs. Early detection is needed to prevent complications and improve the quality of life. An association between serum ferritin and endocrine disorders in thalassemia patients has been inconclusive to date. Objective To analyze for possible associations between serum ferritin and endocrine disorders (short stature, delayed puberty, delayed bone age, hypothyroidism, impaired glucose tolerance, and diabetes mellitus) and the incidence of each disorder in thalassemia major. Methods There were 115 thalassemia major patients aged 10-18 years involved in our cross-sectional study from June 2019 - June 2020 in the Pediatrics Department, DR. M Hosein Hospital, Palembang, South Sumatera. Anthropometry and socioeconomic status informations were collected from physical examination and interview. Ferritin, FT4, TSH, Hb and glucose levels measured by using standard methods for each item in the laboratory, mean while the skeletal age assessment was determined by using FELS method. Results This study included 83 (72.2%) girls and 32 (27.8%) boys. There were 89 (77.4%) subjects with short stature, 74 (64.4%) with delayed bone age, 30 (26.1%) with impaired glucose tolerance, 25 (21.7%) with delayed puberty, 4 (3.5%) with diabetes mellitus (DM), and none with hypothyroidism. Bivariate and multivariate analyses revealed no associations between serum ferritin and short stature, delayed bone age, impaired glucose tolerance, delayed puberty, and DM. Conclusion There is a high prevalence of endocrine disorders in pediatric thalassemia patients, especially short stature and delayed bone age. However, there are no associations between serum ferritin and endocrine disorders in these patients.

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

Abstract Extramedullary hematopoiesis is a normal compensatory reaction that can affect the epidural space, leading to spinal cord compression syndrome. MRI is the imaging method of choice for diagnosis and monitoring. Treatment is still controversial.

Background: Vitamin C is a well-known powerful water soluble antioxidant and very powerful iron absorption enhancer. It was used for a long period in the management of thalassemia major (TM) patients hoping to decrease the oxidative stress initiated by iron overload resulted from hemolysis of erythrocytes and frequent blood transfusions (BT). Iron overload from BTs may be exacerbated in patients due to increased absorption of iron from the diet in response to ineffective erythropoiesis or vitamin C supplementation. Aim: To evaluate the impact of vitamin C supplementation on the iron overload and oxidative stress in beta thalassemia major patients Methods: Fifty five children were investigated in this study. Thirty two of them were patients with TM on frequent BTs and oral chelating drug (deferasirox), they were diagnosed by hemoglobin electrophoresis with a mean age of 9±4.3 y. Patients were attendees of the Blood Disease and Thalassemia center in Maysan Province-Iraq in the period between November 2013 and November 2014. Twenty three apparently healthy children with a mean age of 8±3.2 y were included as a control group. Patients were subjected to vitamin C supplementation (200mg/day) for 2 months. Serum; vitamin C, iron, malondialdehyde (MDA), ferritin and UIBC and complete blood count were determined. Results: There were significant increases of serum iron (P

Growth disorders in short stature are often found in patients with β-thalassemia major. It is caused by several factors such as hypoxia, hemosiderosis, deficiency of nutritional intake, and micronutrient. Disorder in growth will affect the patient's quality of life. This study aims to determine the prevalence of growth disorders and analyze the factors associated with thalassemia child growth disorders. This study used an observational analytic study with a cross-sectional design on 167 patients with β-thalassemia major at the Palang Merah Indonesia Hospital, Bogor, West Java, in October–December 2018. Data was collected using a transfusion compliance questionnaire and the Morisky Medication Adherence Scale (MMAS-8), while growth was assessed using the CDC 2000 height/age curve. Data analysis used SPSS for Windows version 21.0. Of 167 subjects, 86 subjects (51.5%) were not adherent to transfusion, 97 subjects (58.1%) had low consumption of chelation iron, and 146 subjects (87.4%) had growth problems. The results of bivariate data analysis using the chi-square test for transfusion compliance and parental education on growth obtained p=0.000 and p=0.032. Likewise, for compliance with iron chelation consumption and parents' income to growth, the p value=0.000 was obtained. It was concluded that the prevalence of growth disorders was 87.4%, and there was a relationship between transfusion compliance, parental education level, parents' income, and compliance with iron chelation consumption on growth disorders in thalassemia children. FAKTOR-FAKTOR YANG BERHUBUNGAN DENGAN GANGGUAN PERTUMBUHAN PADA ANAK TALASEMIA MAYOR Gangguan pertumbuhan berupa perawakan pendek sering ditemukan pada penderita talasemia β mayor. Hal ini disebabkan oleh beberapa faktor seperti hipoksia, hemosiderosis, kekurangan asupan nutrisi, dan mikonutrien. Gangguan pertumbuhan akan memengaruhi kualitas hidup pasien. Penelitian ini bertujuan mengetahui prevalensi gangguan tumbuh kembang dan menganalisis faktor-faktor yang berhubungan dengan gangguan tumbuh kembang anak talasemia. Penelitian ini menggunakan jenis penelitian observasional analitik dengan desain cross-sectional pada 167 pasien talasemia β mayor di RS Palang Merah Indonesia, Bogor, Jawa Barat pada bulan Oktober–Desember 2018. Pengumpulan data dilakukan dengan menggunakan kuesioner kepatuhan transfusi dan Morisky Medication Adherence Scale (MMAS-8), sedangkan pertumbuhan dinilai menggunakan kurva tinggi/usia CDC 2000. Analisis data menggunakan SPSS for Windows versi 21.0. Dari 167 subjek, 86 subjek (51,5%) tidak patuh pada transfusi, 97 subjek (58,1%) memiliki konsumsi kelasi besi rendah, dan 146 subjek (87,4%) mengalami gangguan pertumbuhan. Hasil analisis data bivariat menggunakan uji chi-square untuk kepatuhan transfusi dan pendidikan orangtua tentang pertumbuhan diperoleh p=0,000 dan p=0,032. Begitu pula untuk kepatuhan konsumsi kelasi besi dan pendapatan orangtua terhadap pertumbuhan diperoleh p=0,000. Disimpulkan bahwa prevalensi gangguan tumbuh kembang sebesar 87,4% dan terdapat hubungan kepatuhan transfusi, tingkat pendidikan orangtua, pendapatan orangtua, dan kepatuhan konsumsi kelasi besi dengan gangguan tumbuh kembang anak talasemia.

BACKGROUND Basic knowledge of sexual and reproductive health is important to be introduced during the premarital period, to reduce high-risk sexual behavior among adolescents, as well as to increase the correct attitude in their future marriage. This study explored the knowledge of HIV/AIDS, sexually transmitted diseases (STDs) and anemia among unmarried adolescents. METHODS This was a cross-sectional analytical study on secondary data from the Indonesian Demographic and Health Population Survey 2017, initially conducted by the National Population and Family Planning Board (BKKBN). Knowledge of HIV/AIDS, STDs and anemia among unmarried adolescents aged 15-24 years in West Java Province and Jakarta, the capital city of Indonesia, was compared and analyzed using the chi-square test. RESULTS In total, 5,389 weighted data were retrieved. In general, adolescents in Jakarta had better knowledge of all diseases compared to those in West Java (p90%) had their own mobile phone with almost daily internet access, however most of their information sources on health education were from their school period. CONCLUSION Premarital education in schools is imperative to enhance the knowledge of STDs and anemia. Moreover, poor knowledge of anemia is a challenge for introducing the topic of thalassemia major, an inherited autosomal recessive type of anemia due to hemoglobinopathy. Since Indonesia is harboring 6-10% of thalassemia carriers, integrative premarital education is needed for better family planning.

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 Insulin-like growth factor-1 (IGF-1) deficiency is the major cause of growth disorders and delayed puberty in children with thalassemia. Hence, identifying factors contributing to IGF-1 deficiency in thalassemia is of importance. Objectives To evaluate the correlation between IGF-1 level and nutritional status, ferritin level, pre-transfusion hemoglobin, thyroid, as well as alanine transaminase level. Methods We conducted a study in children aged 2 to 18 years with thalassemia major who visited outpatient clinics at two hospitals in Indonesia, Dr. Sardjito Hospital, Yogyakarta and Dr. Moewardi Hospital, Surakarta, Central Java, from July to December 2015. Clinical, laboratory, and demographic data were reviewed from medical records. IGF-1 levels were measured using an immunochemiluminiscent method. Results A total of 48 children were recruited into the study. Subjects mean IGF-1 level was 109.28 ng/mL (SD 90.26) ng/mL. Seventy-five percent of the children had IGF-1 level < -2SD. Subjects mean ferritin, pre-transfusion hemoglobin and ALT levels were 3.568 (SD 2131.31) ng/mL; 7.97 (SD 0.85) g/dL and 49.7 (SD 43.1), respectively. Most of the children (91.7%) was eutyroid, with a mean of TSH and FT4 level was 2.7 (SD 1.5) nmol/L and 12.3 (SD 7.1) μIU/ml, respectively. Ferritin level had no significant correlation with IGF-1 level (r=-0.794; P=0.431). However, a strong, positive correlation was documented between pre-transfusion hemoglobin level and IGF-1 level (r=2.380; P=0.022). Multivariate linear regression analysis revealed that factors with significant correlations to IGF-1 level were pre-transfusion hemoglobin level

Background: Hepatitis C virus (HCV) was encountered as one of the most common infections transmitted through blood transfusion to thalassemic patients. After the discovery of new generations of antiviral drugs labeled direct-acting antiviral (DAA) drug since 2014, promising results were reported compared to older regimen of Peginterferon with or without ribavirin (RBV). Objective: The main objective of the study is to assess the hepatitis C viral status of multitransfused beta-thalassemia major patients and the sustained viral response rate to different modalities of therapy. Materials and Methods: A cross-sectional analytical study was conducted in Erbil Thalassemia Center. A sample of all children and adolescent (18 years or younger) patients of beta-thalassemia major with HCV antibody positive were reviewed according to the available medical records in the center. They were divided into two groups ( first who received interferon ([IFN] ± RBV and second who received sofosbuvir (SOF) and daclatasvir [DCV]) for the aim of the study. Results: Among registered 695 patients with thalassemia major screened for HCV antibody, 659 children and adolescents were included and 186 were tested seropositive (28.22%), and they had been submitted to polymerase chain reaction analysis with HCV-RNA identified in 110 (59.13% of initially ELISA test positive). IFN-dependent therapy was given to 87 patients, while sofosbuvir and DCV for remaining 21 patients, sustained viral response was 100% among those received latter therapy with no reported relapse compared to former regimen of 44.3% sustained response and 6.33% relapse rate. Conclusion: DAA drug has a promising therapeutic result replacing the old therapy of IFN-RBV among thalassemic patients with 100% response rate in the study group.

Background: In patients with β-thalassemia major (TM), one of the long-term complications of regular blood transfusion is renal dysfunction. The purpose of the current study was to evaluate the renal function in TM patients receiving Exjade® dispersible tablets and a new film-coated tablet formulation of deferasirox (Nanojade®). Materials and Methods: In this descriptive cross-sectional study, a total of 80 TM patients aged 11–48-year-old entered the study. Patients received 20–30 mg/kg/d (single dose) Exjade® (Exjade group, n = 40) and Nanojade® (Nanojade group, n = 40) orally. To evaluated renal function, serum creatinine (SCr), estimated glomerular filtration rate (eGFR), blood urea nitrogen (BUN), 24-h urine protein (UPro), UCa/UCr, spot UPro/UCr ratio, and serum ferritin were calculated at baseline and every 3 months to 9 months. Results: There was no significant difference in SCr, BUN, eGFR, 24-h UPro, UPro/UCr ratio, UCa/UCr ratio, and serum ferritin between groups at baseline and end of study (Pbaseline > 0.05, Pend of study > 0.05). There was no significant difference in proteinuria between groups at baseline and end of study (Pbaseline > 0.05, Pend of study > 0.05). Conclusions: The proportion of SCr, BUN, eGFR, 24-h UPro, UPro/UCr ratio, and UCa/UCr ratio was not significantly different in TM patients treated with Nanojade® compared to patients' received Exjade®. Nanojade® had similar effects to Exjade®, and therefore, the use of Nanojade® is safe in TM patients and does not seem to be associated with increased renal failure, proteinuria, and hypercalciuria.

Background Thalassemia major is a chronic disease requiring lifetime treatment. A recent study showed that 11-62% of thalassemia patients developed depression, which is associated with high morbidity and mortality. Understanding the extent of the problem related to depression and its contributing factors is important for early management. Objective To determine the prevalence and contributing factors for depression in children with thalassemia major. Methods This cross-sectional observational analytic study included thalassemia major patients aged 7 to 13. Data were analyzed using SPSS for Windows ver. 22.0. Results There were 64 patients included in this study, with mean age 12 (SD 3) years and 82.8% female. Most subjects came from families with low socio-economic status and low parental education. Deferiprone was the most commonly used type of iron-chelating agent. Depression was detected in 34.4% of respondents. Multivariate analysis revealed that factors affecting depression in children with thalassemia major were low maternal education (OR 4.014; 95%CI 1.066 to 15.112) and use of deferasirox (OR 4.129; 95%CI 1.168 to 14.601). Conclusion Prevalence of depression in children with thalassemia major is 34.4%. Low maternal education and deferasirox use as an iron-chelating agent are associated with depression in children with thalassemia major.

Sickle cell disease and homozygous β-thalassaemia are common haemoglobinopathies in Oman, with many implications for local healthcare services. The transfusions of such patients take place in many hospitals throughout the country. Indications for blood transfusions require local recommendations and guidelines to ensure standardised levels of care. This article summarises existing transfusion guidelines for this group of patients and provides recommendations for blood banks and transfusion services in Oman. This information is especially pertinent to medical professionals and policy-makers developing required services for the standardised transfusion support of these patients.

Aim: Endocrinopathies are common in patients with thalassemia major (TM) and affect their life quality. Our aim was to identify the frequency of growth retardation and endocrine complications in these patients. Materials and Methods: Sixty-two patients aged 3-18 years with TM were evaluated retrospectively for height, weight, body mass index (BMI), and pubertal stage. Blood tests for endocrine function, and oral glucose tolerance test (OGTT) results were recorded. Results: The mean age of 62 subjects (33 females/29 males) was 10.4±3.9 years. The frequency of ≤-2 standard deviation scores was 37.1% for height, 33.9% for weight and 11.3% for BMI. Short stature, being underweight, and low BMI were significantly more prevalent in children over 7 years old (p

Background Thalassemia major patients who undergo routine transfusion have an increased risk of acquiring transfusion-transmitted infections (TTI), including hepatitis B and C. These diseases have serious implications and may affect the serum ferritin and aminotransferase levels of thalassemia major patients. Objectives To identify the prevalence of hepatitis B and/or C infections among thalassemia major patients and to evaluate its correlation with serum ferritin and aminotransferase levels. Methods This was across-sectional study conducted at the Thalassemia Center of Dr. Cipto Mangunkusumo Hospital in Jakarta, Indonesia. The subjects were screened for hepatitis B and C infections, and their serum ferritin and aminotransferase levels were also measured. Results In total, 621 subjects were included in the study, among which 5 subjects tested positive for hepatitis B surface antigen (HBsAg) (0.8%), 111 subjects tested positive for anti-HCV (17.8%), and 5 subjects tested positive for both HBsAg and anti-HCV (0.8%). The subjects who tested positive for hepatitis B, hepatitis C, or both showed significantly higher values of aspartate aminotransferase (AST), alanine aminotransferase (ALT), and serum ferritin compared to their negative counterparts. Moreover, serum ferritin showed a positive, moderate correlation with both AST and ALT. Conclusion This study shows a significant association between hepatitis and serum ferritin as well as aminotransferase levels. Early detection and early management of hepatitis B and C infections is warranted to minimize the occurrence of liver damage in thalassemia major patients.

Background Hemolysis and repeated blood transfusions in children with thalassemia major cause iron overload in various organs, including the brain, and may lead to neurodegeneration. Hemolysis also causes decreased levels of nitric oxide, which serves as a volume transmitter and slow dynamic modulation, leading to cognitive impairment. Objective To assess for correlations between serum ferritin as well as nitric oxide levels and cognitive function in children with thalassemia major. Methods This analytical study with cross-sectional design on 40 hemosiderotic thalassemia major patients aged 6−14 years, was done at the Thalassemia Clinic in Dr. Hasan Sadikin Hospital, Bandung, West Java, from May to June 2015. Serum ferritin measurements were performed by an electrochemiluminescence immunoassay; serum nitric oxide was assayed by a colorimetric procedure based on Griess reaction; and cognitive function was assessed by the Wechsler Intelligence Scale for Children test. Statistical analysis was done using Spearman’s Rank correlation, with a significance value of 0.05. Results Abnormal values in verbal, performance, and full scale IQ were found in 35%, 57.5% and 57.5%, respectively. Serum nitric oxide level was significantly correlated with performance IQ (P=0.022), but not with verbal IQ (P=0.359) or full scale IQ (P=0.164). There were also no significant correlations between serum ferritin level and full scale, verbal, or performance IQ (P=0.377, 0.460, and 0.822, respectively). Conclusion Lower serum nitric oxide level is significantly correlated to lower cognitive function, specifically in the performance IQ category. However, serum ferritin level has no clear correlation with cognitive function.

OBJECTIVE: To determine the benefits of elective splenectomy in selected patients with thalassemia major. STUDY DESIGN: Prospective descriptive study. PLACE OF STUDY: Liaquat University Hospital Jamshoro. DURATION OF STUDY: July 2014 to June 2015. PATIENTS AND METHODS: 27 selected patients of Thalassemia major were evaluated after open elective splenectomy via midline incision for the benefits achieved post operatively. RESULTS: Out of 27 patients 19 (70.3%) were male and 08(29.6%) were female, age ranges from 5-10 years with a mean age of 7 years. Out of 27 patients, 25 patients (81.48%) had a uneventful recovery, only 2 patients (7.4%) developed post-operative complications. No mortality was observed in this series. Post-operative long term maintenance of adequate hemoglobin and improved quality of life was observed in 26(96.29%) patients. Only 1(3.7%) patient who developed postoperative incisional hernia was not satisfied with the procedure. CONCLUSION: We conclude that splenectomy is a safe and beneficial procedure in selected patients of thalassemia major.

Latar belakang. Iron overload pada pasien 􀁂-thalassemia mayor terjadi akibat transfusi berulang. Pengendapan besi terjadi terutama di jantung, hati dan kelenjar endokrin. Prevalensi hipotiroid akibat toksisitas besi pada pasien 􀁂-thalassemia berkisar 0-35%. Terapi kelasi besi seperti deferasirox sangat efektif menurunkan kandungan besi. Diharapkan timbunan besi pada kelenjar tiroid berkurang, sehingga disfungsi tiroid tidak terjadi. Tujuan. Membuktikan dan menganalisis pemberian deferasirox pada pasien 􀁂-thalassemia mayor dengan kadar ferritin yang tinggi akan menaikkan kadar T4 dan menurunkan kadar TSH serum. Metode. Penelitian pre dan post test desain dilakukan antara bulan September 2009-Maret 2010 terhadap pasien 􀁂-thalassemia mayor yang mendapat transfusi berulang di bangsal thalassemia anak RS Dr. Kariadi Semarang. Kadar ferritin, T4 dan TSH diperiksa pada awal penelitian dan 6 bulan setelah pemberian deferasirox. Pemeriksaan ferritin, T4 dan TSH menggunakan alat Vidas® dengan metode ELFA. Analisis statistik yang digunakan adalah uji t-berpasangan. Hasil. Subyek penelitian 20 orang yang memenuhi kriteria inklusi.Hipotiroidisme ditemukan sekitar 20%. Rerata kadar ferritin setelah 6 bulan lebih rendah dibanding awal penelitian, namun perbedaan tersebut tidak bermakna (I : 1182,7±53,64 dan II : 1182,3±48,42; p=1,0). Rerata kadar T4 tidak berbeda bermakna setelah 6 bulan pemberian deferasirox (I : 91,8±22,37 dan II : 88,6±20,46 ; p=0,5). Dua pasien dengan kadar TSH yang awalnya tinggi menjadi normal, namun penurunan tersebut tidak berbeda bermakna secara statistik (p=0,2). Kesimpulan. Tidak ada perubahan kadar T4 dan TSH pada pasien 􀇃-thalassemia mayor dengan ferritin tinggi yang mendapat deferasirox per oral.

Background Delayed puberty is the most common endocrine com- plication in thalassemia major. The main cause of delayed pu- berty in thalassemia major is the failure of the hypothalamic-pitu- itary axis due to iron accumulation in the pituitary. Objectives The purpose of this study was to determine the preva- lence of delayed puberty in β-thalassemia major patients in the Department of Child Health, Cipto Mangunkusumo Hospital, Jakarta. This study also evaluated the adequacy of chelation therapy and determined serum gonadotropin and sex hormone levels in these patients. Methods Seventy-two patients with β-thalassemia major aged 13- 18 years old who visited the Thalassemia Outpatient Clinic of Cipto Mangunkusumo Hospital during February-July 2003 were included in the study. Each subject underwent examinations to determine the body weight and height, pubertal status, serum iron level, total iron binding capacity, and the levels of serum LH, FSH, estradiol (in girls) or testosterone (in boys). Results Delayed puberty occurred in 40 of 72 patients (56%). The majority of patients with delayed puberty showed low levels of se- rum LH, estradiol, and testosterone whereas low levels of serum FSH only occurred in 6 of 21 boys and 11 of 19 girls. Most of the patients without delayed puberty had normal levels of serum LH, FSH, and estradiol, but 8 of 16 boys showed decreased serum testosterone levels. Only 3 patients used chelation therapy ad- equately, all of them showed normal puberty. Conclusions The prevalence of delayed puberty in β-thalassemia major patients in this study was still high (56%). Periodic examina- tion and recording of pubertal stage need to be done in girls who have reached 8 years old and boys who have reached 9 years old so that early detection and management of delayed puberty can be done.

Background Thalassemia major (TM) is a transfusion-depen- dent disease. Multiple transfusions cause an accumulation of iron in the body. On the heart, iron can induce cardiomyopathy, the leading cause of death in TM. Objective To evaluate left ventricular functions and mass among adolescents and young adults with TM. Methods A cross-sectional study was conducted on 58 patients with TM in Cipto Mangunkusumo Hospital, Jakarta. Hematological data was collected from medical record. Left ventricular systolic func- tions (EF, FS), diastolic functions (A, E, E/A ratio, IVRT), and mass (LVDDi, LVDSi, LVMi) were detected using an echocardiography Sonos 4500. T test, linier regression, and multiple regression analy- sis were performed. Results Out of 32 TM patients, 30 were enrolled to study. Left ventricular systolic and diastolic functions of TM patients were lower than those of the control and statistically significant [EF 66.1% (SD 4.9) and 71.6% (SD 5.6); P

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

Magnetic resonance imaging using T2* (MRI T2*) is a highly sensitive and non-invasive technique for the detection of tissue iron load. Although the single breath-hold multi-echo T2* technique has been available at the Sultan Qaboos University Hospital (SQUH), Muscat, Oman, since 2006, it could not be performed on younger patients due to their inability to hold their breath after expiration. This study was carried out between May 2007 and May 2015 and assessed 50 SQUH thalassaemic patients aged 7‒17 years old. Seven of these patients underwent baseline and one-year follow-up MRI T2* scans before receiving physiotherapy training. Subsequently, all patients were trained by a physiotherapist to hold their breath for approximately 15‒20 seconds at the end of expiration before undergoing baseline and one-year follow-up MRI T2* scans. Failure rates for the pre- and post-training groups were 6.0% and 42.8%, respectively. These results indicate that the training of thalassaemic patients in breath-hold techniques is beneficial and increases rates of compliance for MRI T2* scans.

Background: Since the reduction of mental health in patients with Thalassemia may affect the quality of life and cause dysfunction in various dimensions, so the assessment of this disorder is necessary to choose the appropriate treatment. Therefore, the aim of this study was to investigate the mental health and quality of life in patients with Thalassemia major. Methods: In this cross-sectional descriptive study, all Thalassemia major patients with 15 or more than 15 years old, referred to Amirkola Institute, were recruited. Mental health status and quality of life were measured via GHQ28 and SF-36 standard questionnaires, respectively. Results: A total of 80 women and 70 men with the mean âgé of 24.17±7.34 were included in the study. The results indicated that in case of quality of life, the physical activity part was singnifantly correlated with stress, social dysfunction, physical symptoms and depression (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

Background: Previously many patients with thalassemia major died from severe anemia during first decade, but with modern transfusion therapy many now survive beyond childhood. Because of such therapy, however excessive iron deposition occurs in lungs and causing respiratory dysfunction. Respiratory impairment occurs because of defective chelation and repeated transfusions. Objectives: To a) study pattern of respiratory impairment using spirometry, b) estimate iron overload by measuring serum ferritin levels, c) correlate a&b. Methodology:Thalassemia children >7year, on regular blood transfusion were included in the study after getting institutional ethical clearance .Standardised pulmonary function test was done using spirometry. Iron overload was assessed using serum ferritin levels. Severity of pulmonary dysfunction was correlated with serum ferritin levels. Results:Total of 42 children were included 62% were males and 38% were females (with median age 12yrs). By spirometry 95% had restrictive pattern of respiratory dysfunction. Mean ferritin value was 4152. Out of them10 (23.8%) mild, 25(59%) moderate and 5(12%) severe dysfunction based on FEV1 and FVC. The mean ferritin values in severe respiratory dysfunction is 6275 which is significantly higher when compared to moderate (4249) and mild (3066) pattern of respiratory dysfunction. None of the children had evidence of CCF. Significant correlation (p value=0.003) was found between severity of pulmonary dysfunction with ferritin values and also with weight of the child (p value=0.007). No other significant correlation found between severity pattern and transfusion index, age or height. Conclusion:Restrictive pattern is most common pulmonary dysfunction seen in chronic iron overloaded thalassemia major children. Regular blood transfusions with adequate chelation decrease incidence of pulmonary dysfunction. Screening of all thalassemia children using spirometry is need of the hour.

Background The life of patients with thalassemia major depends on blood transfusions, while repeated blood transfusions may cause adverse effects such as iron deposition in various organs, includ- ing heart and lungs, which eventually increases the pulmonary arterial pressure. Objective This study was proposed to know the occurrence of pulmonary hypertension in patients with thalassemia major, mea- sured by echocardiography in the Thalassemia Clinic, Department of Child Health, Medical School, Padjadjaran University/Hasan Sadikin Hospital, Bandung. Methods A descriptive cross-sectional study was carried out on 30 patients with thalassemia major, aged 10-14 year-old who received repeated blood transfusions. The study was conducted from April to May 2002. Subjects were examined right after a blood transfusion completed and the pulmonary arterial pres- sure was assessed using Doppler–echocardiography and 2-D echocardiography. Results Twenty two out of 30 subjects showed pulmonary hyper- tension, with pulmonary arterial pressure ranged between 32.3 to 46.2 mmHg. According to the age group, pulmonary hypertension was found in 12 out of 17 subjects aged 10-12 years old and 10 out of 13 subjects aged 13-14 years old. Conclusion The occurrence of pulmonary hypertension in patients with thalassemia major at Hasan Sadikin Hospital was 22/30 and seemed to increase with the age of the patients

Background and Objective: The people with Thalassemia, because of frequent blood transfusion and sever complication ,require constant medical supervision, and their psychological problems are paramount. This study aimed to determine Self-Concept and related factors in children and adolescents with Thalassemia major aged 7-18 in Golestan province ,2012. Material and Methods: This cross-sectional study was conducted on 155, using census sampling, children and adolescents with Thalassemia major refereeing to therapeutic and health centers for blood transfusion. The instruments were Piers-Harris self-concept questionnaire and demographic data . The analysis was performed by SPSS software using ANOV and t-test. Results: The mean of self-concept was low, and there was significant relationship between self-concept and variables such as age, educational status, dropout , order of birth, the number of children in family and parents’ education level. But no significant difference was observed between self-concept and the factors such as sport, the frequency of transfusion, economic status. Conclusion: The results revealed that the self-concept of the thalassaemic patients is low. Some non-adjustable factors exacerbate this condition, but encouraging them to continue their education, informing their parents more about their children’s disease can be helpful to improve their self-concept.

Background: Diabetes mellitus is a major complication of iron overload in patients with beta thalassemia major. Design: This is a descriptive study conducted in a Tertiary Care Teaching Hospital to analyze beta cell function and insulin resistance, and their relation to iron overload status in beta thalassemia major. Fasting glucose, two-hour post load glucose, fasting insulin, alanine amino transaminase (ALT), and ferritin were used as outcome measures. The homeostatic model assessment (HOMA model) was used to calculate the beta cell function and insulin resistance index. Results: Of the 30 cases, 20% had impaired fasting glucose, 3.3% had impaired glucose tolerance, and none had diabetes. Fasting glucose was not significant between the cases and controls (P = 0.113). Fasting insulin (P = 0.001), ferritin (P = 0.001), and ALT (P = 0.001) levels were significantly high in the cases. Insulin resistance index was significantly higher in the cases (P = 0.001) as also the beta cell function (P = 0.001). With increase in age and the number of units transfused there is a decline in beta cell function, fasting insulin, and insulin resistance after attaining the maximum level. This suggests that initial insulin resistance is followed by insulin depletion due to loss of beta cell function, leading to diabetes mellitus. Conclusion: Impaired glucose tolerance (IGT) and insulin resistance precede the onset of insulin-dependent diabetes and adequate chelation therapy is essential for delaying the onset or for prevention of diabetes.

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.

Aim: Fragile bones develop due to various factors in thalassemic patients. Even with optimum management, osteoporosis occurs, contributing to morbidity in majority of patients with thalassemia major (TM). Our aim was to evaluate bone health of thalassemic children using biochemical parameters and bone mineral density (BMD), and to emphasize the precautionary measures and early diagnosis of osteoporosis. Material and Methods: Thirteen children (5 females, 8 males, age

Background In thalassemia major (TM) patients, major mortality is due to cardiac hemosiderosis. Several types of iron chelating agent available recently are given to overcome this problem Objective To compare cardiac dysfunction in thalassemia major patients who used subcutaneous deferoxamine (DFO) to those who used oral deferiprone (DFP) as an iron􀁆chelating agent. Methods This cross􀁆sectional study was held at the Thalassemia Center, Department of Child Health􀁆Cipto Mangunkusumo Hospital (DCH􀁆CMH), Jakarta. We included TM patients aged 1O􀁆18 years Mth a mean pre􀁆transfused hemoglobin level of 2:.7 g/dL in the prior year, and who had used DFO or DFP for at least 1 year v.ith good compliance, at a standard dose of DFO at 40-60 mg/kg/day for 5 days a week or DFP at 50-100 mg/kg/day. We excluded TM patients v.ith congenital heart disease or overt heart failure. Trans􀁆thoracal echocardiography was performed at the Integrated Cardiac Service, CMH by a pediatric cardiologist using the conventional method and tissue Doppler imaging (TD I) consecutively, and within 2 weeks of the subject's receiving a packed red blood cell (PREC) transfusion. The 57 TM subjects consisted of 19 DFO users and 38 DFP users. Results In our subjects, diastolic dysfunction was more commonly seen than systolic dysfunction, especially moderate diastolic dysfunction. In the DFO group, diastolic dysfunction only was detected in 3/19 subjects, systolic dysfunction only in 1/19 subjects, and both diastolic and systolic dysfunction in 15/19 subjects. None of the DFO users had normal cardiac function. In the DFP group, diastolic dysfunction only was seen in 6/38 subjects, and both diastolic and systolic dysfunction in 30/38 subjects, while 2/38 subjects had normal cardiac function. Conclusion Diastolic and/or systolic dysfunction was detected in the majority of subjects, but with preserved global cardiac function. We found that cardiac dysfunction was not significantly different in the two iron chelator groups. For all subjects, diastolic dysfunction was seen in 89% of cases, while systolic dysfunction was detected in 77% of cases. [Paediatr Indones. 2012;52:272,9].

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

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: The reproductive and sexual health issues concerning persons affected by thalassemia major are complex. The study was planned to investigate the psychological attitudes and expectations in a group of thalassemic pregnant women attending hospital for regular blood transfusion. Methods: This is a preliminary cross-sectional study involving 20 consecutive thalassemic patients and a control group of 42 healthy pregnant volunteers. The personality structure was evaluated by Rorschach′s test and the presence of psychic symptoms by SCL-90-R and STAI. Results: Narcissism and sexual traumas are significantly higher in thalassemic women with respects to the control group. Also the percent of anxiety and depression observed with the SCL-90-R was significantly higher than in control group (45% vs. 3%, p < 0.001, mean and SD values are 1.65 ΁ 0.15 vs. 0.43 ± 0.18 for anxiety; 55% vs. 12%, p < 0.001, mean and SD values are 1.76 ± 0.18 vs. 0.85 ± 0.25 for depression). The score observed with the STAI shows that the trait of anxiety differed between thalassemic pregnant women and the control group, even though the score values aren′t pathologic in neither group (87% vs. 42%, p < 0.05, mean and SD values are 33 ± 0.8 vs. 22 ± 0.2). Conclusions: This study addresses the need for developing, implementing and evaluating proper psychological support for thalassemic pregnant patients. Moreover, psychological screening and support prior to, during and following pregnancy would be indicated.

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.

Background Multiple blood transfusions in thalassemia patients lead to iron overload in bone tissue. Iron overload can be determined by serum ferritin measurement. Several studies have evaluated association between serum ferritin level and growth, but without bone age examination. Objective To determine the association between age and serum ferritin level with bone age in children with thalassemia major. Methods This study was conducted at Hasan Sadikin Hospital Bandung during March-May 2007. We performed physical examination, serum feritin measurement, and bone age examination. Data were analyzed with x 2 to determine association between variables. The association between age and serum ferritin level with bone age deficit was analyzed with regression logistic model. Results Subjects consisted of 49 patients with thalassemia major. All subjects had bone age deficit. Most boys were in age group of >10 years and had bone age difference >36 months, while most girls were 36 months had serum ferritin level =5,000 ng/dL. This was statistically significant (x 2 =4.573, P=0.032). There was association between age and bone age deficit (OR=13.461, 95% CI 3.199;56.640), but not with serum ferritin level (OR= 2.199, 95% CI 0.532;9.095). Conclusion In thalassemic children, bone age deficit is associated with age, but not with serum feritin level.

Objective: To evaluate the prevalence of hypoparathyroidism in adult transfusion-dependent patients with beta-thalassemia major in a teaching referral hospital in Oman. Methods: All adult (>13 years) patients with beta-thalassemia major seen at Royal Hospital in Oman between 2004 and 2006 were studied. Demographic, pharmaceutical, clinical and biochemical data were collected for all the subjects. Analyses were performed using both descriptive and univariate statistics. Results: A total of 31 patients were included into the study with an overall mean age of 19±3 years ranging from 14 to 30 years. Just over half of the subjects were males (n=16; 52%). All the patients were on hypertransfusion and combined chelation therapy with desferrioxamine 40-60 mg/kg 5 days per week and deferiprone 75 mg/kg/day. Three of the patients had low levels of parathyroid hormone (