Showing 5 results for Major Thalassemia
Bijan Keikhaei,
Volume 1, Issue 1 (11-2010)
Abstract
Abstract
Background
Iron overload is a major problem in patients with major thalassemia. An effective and safe iron chelator protocol with high compliance rate plays an important role in treatment of these patients. This study was done to assess the efficacy and safety of the sequential deferoxamine and deferasirox protocol in major thalassemia patients in Khuzestan province, Iran.
Material and Method
Sixty two patients were studied aged between 2 to 30 years old. A regimen consisted of 4 days deferasirox followed by 3 days deferoxamine. The duration of trial was 6 months. The efficacy was determined by comparison of ferritin level before and after treatment.
Results
Serum ferritin changed from 3590 ng/ml to 2563 ng /ml, which decreased significantly. During study 21% of patients experienced at least one side effect.
Conclusion
This is a new regimen with high efficacy, low toxicity and acceptable compliance.
Dr A Eghbali, Dr H Taherahmadi , Dr M Shahbazi, Dr B Bagheri , Mrs L Ebrahimi ,
Volume 4, Issue 1 (3-2014)
Abstract
Background
Frequent blood transfusion is often associated with iron overload. Proper use of iron chelators to treat iron overload requires an accurate measurement of iron levels. Magnetic resonance T2-star (T2* MRI) can measure iron level in the heart and liver. Our goal was to see whether an association exists between serum ferritin level and T2* MRI in patients with major beta thalassemia.
Materials and Methods
Sixty patients with a diagnosis of major beta thalassemia were enrolled in the study. They were older than five years old and needed regular transfusion.
Cardiac and hepatic T2*MRI and mean serum ferritin levels were measured within 3 months.
Results
No significant correlation was observed between serum ferritin level and cardiac T2*MRI (p=0.361, r=-0.120).However, a significant correlation was observed between serum ferritin and liver T2*MRI (p=0.021, r=-0.297).
Conclusion
Our results showed an association between hepatic T2*MRI and serum ferritin level.
Dr Noormohammad Noori, Dr Alireza Teimouri, Dr Ghasem Miri- Aliabad,
Volume 7, Issue 1 (2-2017)
Abstract
Background: Cardiac dysfunction is one of the major causes of morbidity and mortality in thalassemia patients. This study aimed to compare the effect of Brain Natriuretic Peptides (BNP) and Calcitonin Gene-Related Peptide (CGRP) with echocardiographic findings in early diagnosis of cardiac disease in major thalassemia patients.
Materials and Methods: This study was performed on 80 patients among 500 major thalassemia patients and 80 healthy people. Those with metabolic, endocrine disorder, hypertension, heart failure, and valvular disease excluded from the study. These two groups were matched based on age and sex. Essential heart findings were analyzed using Mylab 60. After blood sampling, levels of CGRP and BNP were measured by use of ELISA kit from extracted plasma. Mann-Whitney test, independent t-test, and Pearson correlation were used to analyze data and P< 0.05 was considered significant.
Results: The results showed that mean age of all participants was 17.581±5.344 years when distributed between case and control as 18.21 ±5.14 and 16.95 ±5.49 respectively without significant difference. Means of weight, length, hemoglobin, systolic and diastolic pressure were lower in patients group. The majority of echocardiography findings of left and right heart were higher in case group. The average of CGRP and BNP level were more in case group (p<0.05). A positive correlation was observed between BNP (R=0.229, P=0.041) and right MPI. There was also correlation between PEP(R=0.0238,P=0.035) , PEP/ET(P=0.005) of right heart and peak A velocity of left heart (R=0.245,P=0.03) with CGRP.
Conclusion: Findings of this survey showed that systolic and diastolic function of left heart would be changed in patients with major beta thalassemia. Therefore, monitoring BNP and CGRP in symptom free thalassemia patients as well as serial echocardiography is recommended.
Dr Farhad Iranmanesh, Dr Azam Hashemi, Dr Alireza Jenabzade, Dr Mahvash Akhavanghalibaf, Tania Dehesh, Dr Marzieh Abutorabi-Zarchi,
Volume 11, Issue 3 (6-2021)
Abstract
Background: β-thalassemia, a severe form of anemia, is an inherited blood disorder characterized by growth retardation, splenomegaly, and bone abnormalities. Complications related to treatment-induced iron overload also affect the quality of life of patients with major β-thalassemia. Some recent studies indicated cerebral hemodynamic disorders and increased risk of stroke in these patients. The aim of this study was to evaluate mean flow velocity (MFV) in some cerebral arteries of patients with major thalassemia using transcranial Doppler ultrasonography.
Materials and Methods: In this cross-sectional study, 26 patients with major thalassemia were investigated. The definitive diagnosis was based on serum hemoglobin electrophoresis. Transcranial Doppler ultrasonography was performed in patients and MFV of internal carotid, anterior cerebral, and middle cerebral. Posterior cerebral arteries were measured. Demographic characteristics, duration of treatment, number of blood transfusions per month, the interval between the last blood transfusion, and the ultrasonography were recorded and analyzed statistically.
Results: Ten female and 16 male patients participated in this study. Results showed that 57.7% of patients had a hemodynamic abnormality in at least one vessel. The abnormality was significantly higher in the anterior and middle cerebral arteries (p<0.001 and p=0.005, respectively). Among the variables evaluated, age was significantly associated with hemodynamic dysfunction. This relationship remained significant after using the logistic regression analysis (p=0.0267).
Conclusions: Some patients with major thalassemia have a cerebral hemodynamic abnormality. Aging is associated with the higher frequency of this abnormality.
Dr Bijan Keikhaei, Dr Homayoun Yousefi, Dr Arash Alghasi, Dr Asaad Sharhani, Dr Roya Khazaei,
Volume 13, Issue 4 (10-2023)
Abstract
Background: The mainstay of managing severe β-thalassemia remains lifelong blood transfusion. Mismatched red blood cell phenotypes between donors and recipients in multiple blood transfusions can result in the development of alloimmunization in recipients. The aim of this study was to determine the frequency of major and subgroup antigens and their phenotypes in thalassemia major patients.
Materials and Methods: This cross-sectional descriptive study was performed on 105 patients with thalassemia major who referred to Baghaei Hospital in Ahvaz in 2021. Their alloimmunization to erythrocyte antigens was determined with standard tubular antibody search kits.
Results: Among the thalassemia major patients participating in the study, 51 were female (48.45%). The mean age of the participants was 21.10 ± 5.8 years. Out of the 105 patients studied, 26 had detectable alloantibodies in the serum (24.7%). The two groups of patients with positive and negative alloantibodies were significantly different in terms of Rh and C blood groups (P-values of 0.03 and 0.05, respectively). There was no significant association between the existence alloantibody and age, gender, spleen condition and the time of first transfusion (P > 0.05).
Conclusion: It was concluded that red blood cell matching, at least for Rh and C groups, is necessary to prevent alloimmunization in thalassemia major patients.
