Beta-thalassemia Major Patients Research Articles

Noninvasive assessment and risk factors of liver fibrosis in pediatric patients with beta thalassemia major using transient elastography

BackgroundIn beta-thalassemia major (TM) patients, levels of liver iron overload and the presence of chronic hepatitis C are directly correlated with the onset and severity of liver fibrosis. A noninvasive approach that can evaluate cirrhosis and liver fibrosis in these patients is transient elastography (TE). We aimed to find out the role of noninvasive elastography in the assessment of liver fibrosis in young beta-thalassemia major patients receiving frequent blood transfusions. Identifying the patients' risk factors for liver fibrosis is another goal. The study comprised 53 patients, all of whom had a thorough history-taking procedure, clinical examination, and investigations such as CBC, serum ferritin, HCV and HBV serology, and liver function testing. It was carried out transient elastography to find liver fibrosis.ResultsAccording to transient elastography, 52.8% of the patients had severe fibrosis (F2 and higher). 9.4% of people had positive HCV serology results. Significant liver fibrosis was correlated with all serum ferritin levels (708.2 ± 182.1, 3213.5 ± 1272.9, nonsignificant vs. significant fibrosis), HCV infection, age, blood transfusion frequency, and irregular chelation therapy. But no significant correlation regarding sex and BMI was detected.ConclusionTransient elastography is an alternate noninvasive approach that assesses liver fibrosis in beta-thalassemia major patients. The risk of liver fibrosis is related to iron overload, HCV seropositivity, advanced age, frequent blood transfusion, and irregular chelation therapy.

Grade of liver siderosis in beta-thalassaemia major patients receiving different amount of blood transfusion

Background and objectives: A progressive accumulation of body iron easily occurs as a result of long-term transfusions in patients with anaemia of genetic disorders such as thalassaemia. Iron deposit in liver biopsy sections was studied in beta-thalassaemia major patients to assess the grade of liver siderosis and to correlate the grade with amount of blood transfused. Materials and methods: Beta-thalassaemia major patients having splenomegaly and selected for splenectomy were enrolled. Liver biopsy was taken from every patient during the splenectomy. Liver tissue was sectioned and stained with Perls’ prussian blue method for the presence of iron deposition. The degree of iron deposition was expressed as grades of siderosis from 0 to 4. Results: A total of 30 beta-thalassemia patients were enrolled in the study. Out of 30 patients, 7 were males (23.3%) and 23 females (76.7%). The mean age of patients was 15.2 ± 1.4 years. The mean serum iron and ferritin levels of the study cases were above the normal range. Blood received by all patients was 51.5 ± 11.6 units (range 31 to 88 units). Out of 30 patients, grade 1, 2, 3 and 4 liver siderosis was present in 1, 3, 9 and 17 patients respectively. Serum ferritin level of patients with grade 4 siderosis was significantly higher (p = 0.03) compared to grade 3 cases. Pearson’s correlation coefficient test revealed significant positive correlation between grades of liver siderosis and amount of blood transfusion received (0.626, p < 0.01). Conclusion: Grade of liver siderosis is associated with increased units of blood transfusion and is a good indicator for transfusional iron overload in beta-thalassaemia major patients. IMC J Med Sci. 2023. 17(1): 004. DOI: https://doi.org/10.55010/imcjms.17.004 *Correspondence: Dr. Souvik Basak, Department of General Surgery, Medical College, Kolkata, West Bengal, India. Email: sb009cmc@gmail.com

Right ventricular mechanics among beta-thalassemia major patients

Abstract Introduction Right ventricular function among β-thalassemia (β-TM) patients is affected by both pulmonary circulation pressure and primary iron deposition on right ventricular myocardium. Purpose In our study we aimed to detect the presence of impaired right ventricular function and right ventricle to pulmonary circulation (RV-PC) coupling by applying novel echocardiographic techniques among these patients. Methods 47 β-TM patients (mean age 39.02±8.76 years, 48.9% male sex) and 24, age and sex matched, healthy controls were examined by transthoracic echocardiography. Conventional echocardiographic parameters were estimated (LVEF, E/e' ratio, left atrial volume index (LAVI), right ventricular systolic pressure (RVSP), S' wave of tricuspid annulus), alongside with deformation indices (left atrial strain at reservoir phase (LASr), right ventricular free wall (RVfw) and 4 chamber (RV4c) strain), as well as RV-PC coupling expressed as S'/RVSP, RVfw/RVSP and RV4c/RVSP. T2* was calculated by cardiac magnetic resonance imaging in β-TM patient group. Results E/e' ratio, LVEF, LAVI, RVSP, LASr, RVfw, RV4c, S'/RVSP, RVfw/RVSP and RV4c/RVSP differed significantly between patients and controls (Table 1). T2* did not correlate with either right ventricular deformation indices or RV-PC coupling indices. A correlation was found regarding LASr with both S'/RVSP (r=0.383, p=0.008) and RV4c/RVSP (r=−0.388, p=0.007) but not with RVfw/PVSP (Table 2). Conclusions Right ventricular deformation indices were found to be impaired in β-TM patients compared to controls, but they do not seem to be associated with myocardial iron load (T2* index). RV-PC coupling was also found to be impaired among patients and a correlation was found between RV-PC indices and left atrial strain. The latter suggests that the presence of atrial cardiomyopathy in β-TM patients could be associated with the impaired RV-PC coupling. Funding Acknowledgement Type of funding sources: None.

Evaluation of immune system in patients with transfusion-dependentbeta-thalassemia in Rasoul-e-Akram Hospital in 2021: A descriptive cross-sectional study.

Background and aimsThalassemia 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.MethodsThis 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.ResultsOf 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 < 0.05). Moreover, there was a strong positive correlation between splenectomy and IgG immunoglobulin (p < 0.001) and CD16 (p = 0.005) lymphocyte marker.ConclusionIron chelator agents effectively improve patients' immune system with thalassemia major. The increase in IgG and IgM immunoglobulins levels is due to frequent blood transfusions, which stimulate the immune system.

Can immature granulocyte count be a practical marker for evaluating bone marrow activation in beta thalassemia patients?

Amaç: Çalışmamızda çocuk ve erişkin beta talasemi major ve intermedia hastalarımızın immatür granülosit ve immatür granülosit yüzdelerinin kemik iliği aktivasyonlarını değerlendirmede pratik bir belirteç olarak kullanılıp kullanılamayacağını araştırmayı amaçladık. Yöntem: Ocak 2017-Aralık 2021 tarihleri arasında Mersin Üniversitesi Tıp Fakültesi Çocuk ve Erişkin Hematoloji kliniğinde takipli 38 Beta talasemi major ve 12 Beta talasemi intermedia hastasının yaş, hemoglobin (gr/dl), hematokrit, beyaz küre sayısı (x103/µL), trombosit değeri (x103/µL), immatür granülosit sayısı ( x103/µL) , immatür granülosit yüzdesi (%), ferritin değeri (ng/mL), şelasyon durumu, transfüzyon sıklığı ve düzeni, son bir yıldaki ortalama hemoglobin düzeyi gibi parametreleri incelendi. Düzenli transfüzyon alan ve almayan hastalar immatür granülosit sayısı, yüzdesi ve diğer parametreler açısından karşılaştırıldı. Bulgular: Hastalar çocuk ve erişkin olarak gruplandığında; çocuk hastalarda son bir yıldaki hemoglobin düzeyi daha yüksek iken erişkinlerde beyaz kan hücresi, immatür granülosit ve trombosit sayısının anlamlı olarak daha yüksek olduğu görüldü (p&amp;lt;0.05). Çocuk hastaların daha düzenli transfüzyon aldığı gözlendi. Düzensiz transfüzyon alan hastalarda son bir yıldaki ortalama hemoglobin düzeyinin anlamlı oranda daha düşük ancak immatür granülosit düzeyi, trombosit ve beyaz küre sayısının daha yüksek olduğu görüldü (p&amp;lt;0.05). Son bir yıldaki ortalama hemoglobin düzeyi arttıkça ortalama immatür granülosit sayısı ve yüzdesinin azaldığı saptandı. Sonuç: Çalışmamızda immatür granülosit sayısı ve yüzdesi, total beyaz küre ve trombosit sayısının düzensiz transfüzyon alan hastalarda istatistiksel açıdan anlamlı olacak şekilde yüksek olması ve son bir yıldaki ortalama hemoglobin düzeyi azaldıkça immatür granülosit ve immatür granülosit yüzdesinin artması bu hastalarda kemik iliğindeki hematopoezin aktif olduğunu düşündürdü. Bu nedenle beta talasemili hastalarda inflamatuvar aktivitenin derecesini ve transfüzyon etkinliğini değerlendirmede hemen her merkezde bakılabilen immatür granülosit ve immatür granülosit yüzde parametrelerinin kullanılabileceğini düşünmekteyiz.

Awareness Among Educated and Uneducated Parents of Beta-Thalassemia Major Patients About Antenatal Screening.

Objective: This study aims to determine the awareness among educated and uneducated parents of beta-thalassemia major patients about antenatal screening.Setting: The study was conducted by the Lady Reading Hospital Medical Teaching Institution (MTI), Peshawar.Duration of the study: This study was conducted for six months.Materials and methods: A total of 296 parents of beta-thalassemia major were interviewed. Parents who were not ready to give consent and other hemolytic anemia patients were excluded. Data were collected on self-structure pretested Performa. The demography of the patients and awareness of the parents regarding antenatal screening were recorded from each participant. A chi-squared test was used to assess the association between parental education and antenatal screening. A p-value of less than 0.05 was considered significant.Results: A total of 296 patients were studied; 160 were males (54.1%), and 136 were females (45.9%). The mean age was 6.84 years. Mothers (74.3%) accompanied majority of the patients. Most of the parents (265; 89.5%) were uneducated, and 80% of the parents were not aware of any antenatal screening.Conclusion: There was a significant association between the education of parents and awareness of antenatal screening (p = 0.01).

Evaluation of Quantitative Aspects of Craniofacial Deformities in Transfusion Dependent Beta-Thalassemia Major Patients: A Cephalometric Study.

To evaluate the quantitative (cephalometric) aspects of the craniofacial deformities in transfusion-dependent beta-thalassemia (TDT) patients. Sixty-five TDT patients receiving a regular blood transfusion in the Department of Pediatrics of age group 5 to 8 years (younger) and 15 years and above (older) were compared with control groups of similar age using cephalograms (lateral and posterior-anterior view). The prevalence of skeletal class II tendency is higher in thalassemic patients that do not improve with age. The thalassemic patients were found to show large angle ANB i.e. Angle between points point a, nasion and point B (ANB), large flexure angle, small angle SNB i.e. Angle between points sella, nasion and point B (SNB), and normal angle SNA i.e. Angle between points sella, nasion and point A (SNA) angles suggestive of retrognathic mandible. The thalassemic patients were observed to have smaller transverse widths and thickened calvarium on the posterior-anterior view. The skeletal class II malocclusion appears to be a manifestation of generalized growth retardation/delayed puberty in thalassemic patients leading to diminished mandibular growth, rather than maxillary prognathism due to marrow hyperplasia.

New Entity-Thalassemic Endocrine Disease: Major Beta-Thalassemia and Endocrine Involvement.

New Entity-Thalassemic Endocrine Disease: Major Beta-Thalassemia and Endocrine Involvement.

Frequency of Fetal Hemoglobin Level in Siblings of Betathalassemia Major patients

Aim: To see the frequency of fetal haemoglobin level in siblings of beta thalassemia major (BTM) patients. Study population: A total of 400 subjects were included. Grouping: Group A includes normal siblings of BTM cases, B includes beta thalassemia trait (BTT) siblings of BTM cases and group C were healthy controls. Selection criteria: Asymptomatic siblings of diagnosed cases of beta thalassemia major were included. Patients with history or diagnosis of any acute or chronic illnesses were excluded from the study. 5 ml of blood was taken in EDTA vial and used for haemoglobin electrophoresis. Results: In 200 cases, 78% (156) were beta thalassemia trait (BTT) siblings and fetal Hb was also more in BTT siblings i.e. Group B. Conclusion: Fetal haemoglobin (HbF) was increased in BTT siblings (group B) of BTM cases. Keywords: Hb electrophoresis, fetal Hb, beta thalassemia major

Influenza Vaccine Booster Stimulates Antibody Response in Beta Thalassemia Major Patients.

The aim of this study was to evaluate antibody response against influenza vaccine in beta thalassemia major patients from Iran. Thirty beta thalassemia major patients were enrolled and divided into three groups: single dose (group 1), double dose (group 2), and control (group 3). Seroconversion, seroprotection, and geometric mean titer (GMT) assays were performed through hemagglutination inhibition (HI) on days 0, 14, and 60. Based on the results, the level of antibody titer was increased in group 2. Two weeks after vaccination, seroconversion rate was about 20% and 30% in groups 1 and 2. Sixty days after vaccination, the seroconversion rate was around 70% and GMT showed a more than 2-fold increase in group 2. Based on the results, the immunogenicity of double dose vaccination against influenza infection appears to be higher than the single dose vaccine in beta thalassemia major patients, and thus it is recommended to use two doses of vaccine, especially in splenectomized patients who are more sensitive than others.

Evaluation of serum Fibroblast growth factor-23 in patients with beta-thalassemia major compared to healthy population

Background: The role of phosphate hemostasis in development of thalassemia bone disease has not been extensively studied yet. Due to the lack of sufficient human studies about the changes of serum Fibroblast growth factor-23(FGF23) in patients with beta-thalassemia major as the first step of investigating the role of FGF23 in thalassemia bone disease, the present study aimed to investigate the serum level of FGF23 in patients with thalassemia major.&#x0D; Material and Method: In this case-control study, 25 patients with beta thalassemia major and their age- and sex-matched healthy volunteers were enrolled. Serum phosphorous, calcium, parathyroid hormone (PTH), 25(OH) D, erythropoietin (EPO), serum intact FGF23 (iFGF23) and 1,25 (OH)2 D were checked and analyzed.&#x0D; Result: Patients with beta-thalassemia major had lower 1,25 (OH)2D, (p = 0.025), higher phosphate (p = 0.002), and higher PTH (P &lt;0.001) compared to the control group; however, all of them were in their normal blood range. They also had higher serum FGF23 (p = 0.007) and higher EPO (P&lt;0.001). Serum FGF23 had an independent association with serum Iron (p=0.016), 1, 25(OH)2 Vitamin D (p&lt;0.001), and hemoglobin (p=0.002).&#x0D; Conclusion: Serum FGF23 was associated with serum Iron, 1, 25(OH)2 Vitamin D, and hemoglobin in beta-thalassemia major patients. Hence, it seems that regular transfusions and chelating agents which can decrease the serum iron and increase hemoglobin level can be associated with lower iFGF23.

Role of Carotid Ultrasound in the Evaluation of Atherosclerotic Changes in Beta Thalassemia Major Patients

Background: Iron overload in beta thalassemia patients may lead to the alteration of arterial structures together with hyperlipidemia and other risk factors that may lead to the atherosclerotic changes and thickening of carotid arteries. Carotid ultrasound is easy, noninvasive, and rapid test that can detect atherosclerotic changes by the assessment of carotid intima-media thickness, which is a structural marker of atherosclerosis, and it correlates well with vascular risk factors and its relation with stroke and coronary artery disease. Aim: The aim was to evaluate the role of carotid ultrasound in the assessment of atherosclerotic changes in iron-overloaded beta thalassemia major patients. Patients and Methods: This is a case–control study done in Babylon Gynecology and Children Teaching Hospital from July 1 to December 31, 2018 on 60 patients with beta thalassemia major and 30 normal children as a control group. Their age ranges from 3 to 14 years old. Carotid ultrasound examination was done on all patients and the control group. Results: In this study, carotid intima-media thickness was significantly higher in patients than in controls (P &lt; 0.001). Carotid intima-media thickness significantly increases in patients with beta thalassemia major with increasing duration of blood transfusion (P &lt; 0.001), with increasing serum ferritin (P = 0.004), and with increasing serum cholesterol (P &lt; 0.001). The study also showed no correlation between body mass index and blood pressure with carotid intima-media thickness. Conclusion: Carotid ultrasound is useful in the assessment of carotid intima-media thickness, which is a marker of atherosclerosis and has a strong positive correlation with markers of iron overload and hyperlipidemia.

Prevalence of metabolic syndrome in beta-thalassemia major adult patients in Tanta University Hospitals

Introduction: one of long-term complications of Beta-thalassemia major (β-TM) disease is metabolic syndrome, which has a significant influence on patients' quality of life. Objective: to investigate the prevalence of metabolic syndrome in β-thalassemia major patients in Tanta University Hospitals. Materials and methods: our study included two groups. Group I: consist of (100) adult beta-thalassemia major patients. Group II: consist of (100) sex and age matched healthy subjects as control group. Results: the present study revealed that 29% of our β-thalassemia major patients had metabolic syndrome while in control group it was 2.0%, and low high-density lipoprotein (HDL)was the most frequent positive criterion it was 86.0% in β-thalassemia major patients' group. Prevalence of hyperglycemia, abdominal obesity, hypertriglyceridemia, and hypertension in patients with thalassemia was 29, 41, 21and 7%, respectively. Prevalence of metabolic syndrome in female patient was 34.3% while in male beta thalassemia major patient it was 18.2%. the prevalence of metabolic syndrome in well chelated beta thalassemia major patient was 0.0%, while in poorly chelated patients it was 35.4%. Conclusions: this study revealed that 29% of β-thalassemia major group patients had International Diabetes Federation (IDF) criteria of metabolic syndrome and low HDL was the most frequent positive criterion.

Prevalence of metabolic syndrome in beta-thalassemia major adult patients in Tanta University Hospitals

Introduction: one of long-term complications of Beta-thalassemia major (β-TM) disease is metabolic syndrome, which has a significant influence on patients' quality of life. Objective: to investigate the prevalence of metabolic syndrome in β-thalassemia major patients in Tanta University Hospitals. Materials and methods: our study included two groups. Group I: consist of (100) adult beta-thalassemia major patients. Group II: consist of (100) sex and age matched healthy subjects as control group. Results: the present study revealed that 29% of our β-thalassemia major patients had metabolic syndrome while in control group it was 2.0%, and low high-density lipoprotein (HDL)was the most frequent positive criterion it was 86.0% in β-thalassemia major patients' group. Prevalence of hyperglycemia, abdominal obesity, hypertriglyceridemia, and hypertension in patients with thalassemia was 29, 41, 21and 7%, respectively. Prevalence of metabolic syndrome in female patient was 34.3% while in male beta thalassemia major patient it was 18.2%. the prevalence of metabolic syndrome in well chelated beta thalassemia major patient was 0.0%, while in poorly chelated patients it was 35.4%. Conclusions: this study revealed that 29% of β-thalassemia major group patients had International Diabetes Federation (IDF) criteria of metabolic syndrome and low HDL was the most frequent positive criterion.

Study of Dyslipidemia and its Association with Serum Ferritin and Amount of Blood Transfusion in β- Thalassemia Major Patients in a Tertiary Level Hospital

Introduction: Beta-thalassemia is thought to be the most common genetic blood condition in the world. Different kinds of Beta-thalassemia major have been shown to have lipid problems. The goal of this study is to evaluate the lipid profiles of beta-thalassemia major patients. The aim of the study was to study the association of dyslipidemia with serum ferritin levels and amount of blood transfusion among Beta thalassemia patients. Methods: This descriptive cross-sectional study was conducted at the Department of Medicine and Hematology, Sir Salimullah Medical College Hospital, Bangladesh. The study duration was 6 months, from January 2017 to July 2017. The targeted study sample size was 96, but due to the short duration of the study, a total of 40 patients were selected as the study population. Result: Hematological tests showed the mean hemoglobin level in the thalassemia group was 7.23 gm/dl, with a standard deviation of 1.23. Mean MCV, MCH, and MCHC in the thalassaemic group were significantly lower at 69.83 fl (SD8.34), 23.10 PGM (SD3.57), and 28.03 % (SD 2.58) compared to the normal range (P-value &lt;0.001 in all parameters). Beta-thalassemia major patients had significantly lower high-density lipoprotein and low-density lipoprotein (LDL) (p&lt;0.001) compared to the normal range. However, serum triglycerides levels of Beta-thalassemia patients (211.5±31.54 mg/dl) were significantly higher [p-value &lt; 0.001].But total cholesterol level was not statically significant among participants ( P-value 0.428). High levels of serum triglycerides were associated with high ferritin levels and an increased amount of blood transfusion. Low levels of HDL-C and LDL-C were associated with a high level of ferritin and an increased amount of blood transfusion. Conclusion: Our study revealed that there was a significant difference in various lipid levels between patients with beta-thalassemia major and normal values which may help physicians to design the therapeutic module for the treatment of such patients.

The role of cardiac T2* magnetic resonance imaging in the assessment of myocardial iron concentration in patients with beta-thalassemia major

Background: Beta-thalassemia is a group of inherited blood disorders that are characterized by reduced levels of functional hemoglobin. These patients require lifelong blood transfusions, leading to iron deposition in various tissues. Prompt detection of myocardial iron helps to assess the extent of myocardial damage and its complications. Aims and Objectives: Our study aims to establish the role of T2* cardiac magnetic resonance imaging (MRI) and its comparison with serum ferritin levels in detecting iron overload in beta-thalassemia major (TM) patients. Materials and Methods: This prospective study included 30 pediatric patients admitted to our institute with beta-TM. The patients underwent T2* cardiac MRI examinations, myocardial iron quantification was assessed, and patients were divided into groups based on the severity of iron overload. Results: In this study, there were 18 males and 12 females. Out of the total studied cases, 15 (50%) showed no evidence of iron overload, 8 (26.67%) showed mild, 4 (13.33%) showed moderate, and 3 (10%) showed severe cardiac siderosis, based on the cardiac T2* values obtained. Conclusion: T2* cardiac MRI is an accurate and invaluable tool that helps to detect iron overload in beta-TM.

A two-dimensional speckle-tracking echocardiography for the diagnosis of early myocardial disease in beta-thalassemia major patients.

Although magnetic resonance imaging T2* is considered the gold standard to assess myocardial iron overload in β-thalassemia patients, its routine use is limited by the high cost and limited availability. Recent data demonstrated that strain imaging by speckle tracking is a sensitive tool for early assessment of the left ventricular myocardial dysfunction. This study aims to evaluate the clinical utility of two-dimensional (2D) speckle-tracking echocardiography (STE) for the detection of early myocardial disease in beta-thalassemia major (β-TM) patients. 2D STE, magnetic resonance imaging (MRI) heart T2* and MRI liver iron content were done for 30 β-TM patients with no clinical heart disease, compared to 2D STE in 30 healthy age- and sex-matched controls. There was a significant reduction in the longitudinal systolic strain values by STE among β-TM patients compared to controls (P = 0.05). A longitudinal peak systolic strain cutoff values of ≤-19 was able to detect β-TM patients having subclinical cardiac iron overload by MRI T2* (sensitivity = 90%-93.3%, specificity = 83%-100%). Mean serum ferritin in the past 2 years correlated negatively to longitudinal systolic strain values global longitudinal peak systolic strain average (P = 0.05).

Frequency And Significance of Electrocardiographic Changes in Beta Thalassemia Major Patients

Objective: To find out the electrocardiographic changes among beta thalassemia major patients as the marker of cardiac complications at later stages of life.&#x0D; Study Design: Prospective observational study.&#x0D; Place and Duration of Study: Department of Pediatrics and Neonatology, Pak Emirates Military Hospital Rawalpindi from Jul 2018 to Dec 2018.&#x0D; Methodology: One hundred and thirty patients of age 5-20 years with beta thalassemia major were included in the study. Patients were attending the thalassemia centre regularly.&#x0D; Results: Out of total 130 patients, 70 (53.84%) were males and 60 (46.15%) were females. Out of 81 (56.92%) patients showed at least one electrocardiographic change while 49 (37.69%) patients showed normal electrocardiography. Remarkable electrocardiographic changes were sinus tachycardia, arrhythmias, T-wave inversions, long QTc interval and sinus bradycardia. There was statistically significant association in the increased serum ferritin levels, electrocardiographic changes, number of blood transfusions and occurrence of ECG changes (p&lt;0.05).&#x0D; Conclusion: Regular electrocardiographic monitoring has significant role in cardiac evaluation of beta thalassemia major patients as an indicator of evolving cardiac complications that can arise from iron overload secondary to multiple blood transfusions.

Evaluation of hearing and inner ear functions of beta-thalassemia major patients receiving regular chelation therapy with desferrioxamine

OBJECTIVE: In this study, the hearing functions and inner ear conditions of beta (b)-thalassemia major patients who were treated with desferrioxamine were evaluated. METHODS: This study was conducted with 69 pediatric patients aged seven years and older (Thalassemia group; 42 males, 27 females; mean age 11.0±4.1 year; range, 7 to 15 year), diagnosed with b-thalassemia major, treated with regular iron-chelating agent desferrioxamine for at least three years, and followed in the pediatric hematology and oncology clinic and 67 children (Control group; 36 male, 31 female; mean 12.4±6.8 year; range, 5 to 17 year) without hearing loss and chronic ear disease between January 2017 and January 2020. The patient and control groups were examined by an otolaryngologist. Pure-tone audiometry and distortion product otoacoustic emission tests were performed on the patient and control groups, and the results were compared. RESULTS: No significant difference was found in terms of age and sex in the thalassemia and control groups. In the comparison of hearing thresholds between groups with pure-tone audiometry, the hearing thresholds of the thalassemia group were higher than the control group at a frequency of 8000 Hz in the right and left ears, and the difference was statistically significant. In the comparison of the signal-to-noise ratios between the groups with the distortion product otoacoustic emission test, the decibel values in the right ear at frequencies of 1000 Hz and 6000 Hz were lower in the thalassemia group than in the control group, and the difference was statistically significant. CONCLUSION: In the study, the inner ear functions were found to be affected in patients treated with desferrioxamine. Even if b-thalassemia major patients do not clinically describe hearing loss, we believe their hearing thresholds will increase starting from high frequencies with repetitive desferrioxamine treatment, and they may experience a hearing loss.

Effect of Iron Chelator on Liver Function in Beta Thalassemia Major Patients

Background: Due to Regular blood transfusions and Desferrioxamine therapy, beta-thalassemic patients can live for over 30 years. It is becoming increasingly common for liver diseases to cause mortality and morbidity in these patients. Chelation compliance has improved with the recent addition of the oral single-dose therapy Deferasirox (DFX). This study will investigate the impact of iron therapy on liver function tests in BMT patients. Methodology: The patients enrolled in the study were HBV negative and treated with DFO and DFX after BTM. The LFTs include alanine transferase, Albumin, serum bilirubin and aspartate transferase. Sixty patients with Beta-thalassemia were followed every six months for serum ferritin concentrations. Results: Therapy with Desferrioxamine significantly decrease serum ferritin levels inbeta-thalassemia patients and isconcerned with high reduction in serumalanine transaminase and serum aspartate transaminase. Albumin concentrations remain the same in DFX treatment. Association between serum ferritin levels ALT and AST levels were found. There is a negative correlation between serum ferritin concentration and ALT. Conclusion: Patients with Hepatitis negative having thalassemia with iron overload may have some liver function impairment. As a result of DFX treatment, AST, ALP and ALT levels were significantly reduced.