Left ventricular dysfunction in transfusion dependent beta Thalassemia major patients in Bangladesh

Background: Thalassemia major is an inherited haemoglobin disorder resulting in chronic haemolytic anaemia. Patients with beta thalassemia major are maintained on continuous blood transfusion regimens resulting in iron overload that adversely affects both the structure and function of the heart and other vital organs which can be easily prevented with iron chelating therapy. The aim of the study was to detect left ventricular dysfunction at an early stage so that early effective intervention can be done.Methods: A total of 50 patients with beta thalassemia were included in the study by non randomized qualitative purposive sampling from July 2013 to June 2014. Their total body iron status was be assessed by doing serum ferritin level. Left ventricular systolic and diastolic function was assessed by echocardiographyResults: Cardiac dysfunction was present in 11 patients with high incidence in patients with low pre-transfusional haemoglobin group (p=0.4) and in patients having high serum ferritin level (p=0.02). Systolic cardiac dysfunction was present in 7(14%) of patients and diastolic dysfunction was present in 4(8%) of patients. There was a weak but significant correlation between left ventricular ejection fraction and serum ferritin concentration (r=-0.22; p=0.03). Only few (8%) patients had diastolic dysfunction.Conclusion: Patients with beta thalassaemia on an adequate transfusion showed an abnormal left ventricular systolic function. In early stage of disease diastolic function was normal but after repeated transfusion there were impaired relaxation indicating diastolic dysfunction. These findings seem mainly to be related to chronic anaemia and serum ferritin levelCardiovasc. j. 2016; 9(1): 31-35

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Relation of Left Ventricular Diastolic Dysfunction to Serum Ferritin in Thalassemia Major

Kurdistan Journal of Applied Research ◽

10.24017/science.2019.ichms.24 ◽

2019 ◽

pp. 224-233

Author(s):

Aso Faeq Salih ◽

Adnan M. Hamawandi

Keyword(s):

Diastolic Dysfunction ◽

Diastolic Function ◽

Serum Ferritin ◽

Ferritin Level ◽

Serum Levels ◽

Thalassemia Major ◽

Left Ventricular ◽

Lv Function ◽

P Value ◽

Lv Diastolic Function

A prospective study was conducted on 60 thalassemia major patients (33 males and 27 females ) in whom the LV function and its parameters where assessed by echocardiography in relation to serum ferritin during the period from 2nd October 2007 to 2nd February 2008, in Sulaimani pediatric teaching hospital. Patient and method: The age ranges of patients were ranged between 3 – 18 years old. The diastolic function of LV was assessed by (M-mode, D-mode, and pulse dopplar study of mitral and pulmonary vein) done. Among 60 patients seen, 25% shows normal LV diastolic function, the rest is 13% relaxation dysfunction, 27% shows Psuedonormal diastolic dysfunction and 35% shows restrictive pattern of LV diastolic dysfunction. Mean serum ferritin in normal and abnormal diastolic LV dysfunction was not so different. In addition nearly all LV parameters as ( LVEDD , LVESD , LA diameter , aortic root diameter , IVC , E ,A ,E/A ratio , A-E time , DT time , S,D,R waves ) where non significantly related to serum ferritin level , this means that P value is less than 0.05 . This means that the level of serum ferritin has no relation with the above parameters in which 75% of those parameters were already abnormal in comparison to the age of the child. Another finding shows that 25% of normal diastolic function is seen in serum levels above 1500 ng/l, in addition, 50% of diastolic dysfunction will associate serum ferritin of less than 500ng/l . P value is more than 0,05 this means that its non-significant. In conclusion, there is a non-significant correlation between serum ferritin and LV diastolic function, so serum ferritin is not a dependant indicator for myocardial iron deposition.

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Correlation between serum ferritin level, cardiac and hepatic T2-star MRI in patients with β-thalassemia major in Al-Diwaniya thalassemia center

International Journal of Research in Pharmaceutical Sciences ◽

10.26452/ijrps.v9ispl1.1392 ◽

2018 ◽

Vol 9 (SPL1) ◽

Author(s):

Alaa Mutter Jabur Al-Shibany ◽

AalanHadi AL-Zamili

Keyword(s):

Iron Overload ◽

Serum Ferritin ◽

Serum Ferritin Level ◽

Chelation Therapy ◽

Ferritin Level ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Iron Level ◽

The Mean ◽

T2 Mri

Patients with transfusion dependent thalassemia major 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) is the preferred method to measure iron level in the liver andthe heart. The goal of our study was to see if there is an association exists between serum ferritin level and T2* MRI results in patients with beta thalassemia major.This study was done in Al-Diwaniya Thalassemia center,Maternity and children teaching hospital,Iraq. During the period from 1st of January to 31st of October. Fifty eight patients with a diagnosis of beta thalassemia major were enrolled in the study. They were older than five years old,transfusion dependent and on chelation therapy. Hepatic and Myocardial T2*MRI and the mean serum ferritin levels were measured during the study period for all patients.There is a significant correlation was observed between serum ferritin level and cardiac T2*MRI (p=0.018 ). also a significant correlation was observed between serum ferritin and hepatic T2*MRI (p=0.02). Neither cardiac T2* MRI nor hepatic T2* MRI show any correlation with the mean age.our study also showa positive correlation between the patients withcardiac T2* MRI and the development of diabetes mellitus in contrast to hepatic T2* MRI in which there is no any correlation. Hypothyroidism was observedno correlation with either cardiac or hepatic T2* MRI.Our results showed a positiveassociation between hepatic, cardiac T2*MRI and serum ferritin levels.

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PREVALENCE OF HYPOTHYROIDISM, DELAYED PUBERTY AND DIABETES MELLITUS IN PATIENTS OF Β-THALASSEMIA MAJOR

International Journal of Medical and Biomedical Studies ◽

10.32553/ijmbs.v4i3.1017 ◽

2020 ◽

Vol 4 (3) ◽

Author(s):

Naresh Manne ◽

Bharat Kumar Gupta ◽

Sandeep Kumar Yadav ◽

Saurabh Singhal ◽

Archana Dubey

Keyword(s):

Diabetes Mellitus ◽

Blood Transfusion ◽

Serum Ferritin ◽

Serum Ferritin Level ◽

Ferritin Level ◽

Thalassemia Major ◽

Delayed Puberty ◽

Beta Thalassemia ◽

Endocrine Disorders ◽

Beta Thalassemia Major

Background: Beta-Thalassemia is a genetic disorder which is associated with a lot of complications. Frequent blood transfusions result in increased iron deposition in various tissues leading to dysfunction of many vital organs. Endocrine disorders constitute a major part of such complications increasing the morbidity of thalassemia manifold in the affected patients. Methods: This is a descriptive cross sectional study carried out in 100 diagnosed patients of beta- thalassemia major who had visited the OPD/IPD of Subharti Medical College & affiliated Hospitals, Meerut for routine blood transfusion or for any other complication. Patients were clinically examined and investigated for presence of one or more endocrine disorders on their routine appointments. Results: Endocrine disorders were detected in a total of 82 patients. Diabetes mellitus was detected in 12% patients, hypothyroidism in 36% patients and delayed puberty was found in 72% patients. Mean serum ferritin level was found to be 5831.0±2860.5 ng/ml in beta-thalassemia Major patients, while it was in normal range in control subjects. Conclusion: Research concluded with finding of Delayed puberty (72%), Hypothyroidism (36%) and diabetes mellitus as (12%) in beta thalassemia patients who were on regular blood transfusion therapy. Iron overload as serum ferritin level was found to be highly raised in all study case. On the basis of our study we recommend that early detection and management protocols for these endocrinopathies may improve the life prospects of beta-thalassemia Major patients. Keywords: Endocrine disorders, Hypothyroidism, Delayed puberty, Diabetes Mellitus Serum ferritin, Thalassemia Major.

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Investigation of correlation between H63D and C282Y mutations in HFE gene and serum Ferritin level in beta-thalassemia major patients

Transfusion Clinique et Biologique ◽

10.1016/j.tracli.2019.05.003 ◽

2019 ◽

Vol 26 (4) ◽

pp. 249-252

Author(s):

Romina Rahmani ◽

Parisa Naseri ◽

Ava Safaroghli-Azar ◽

Shahriar Tarighi ◽

Tahereh hosseini ◽

...

Keyword(s):

Serum Ferritin ◽

Serum Ferritin Level ◽

Ferritin Level ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Hfe Gene ◽

Beta Thalassemia Major

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Thyroid Function Status and Echocardiography Abnormalities in Patients with Beta Thalassemia Major in Bahrain

Clinical Medicine Insights Cardiology ◽

10.4137/cmc.s10702 ◽

2013 ◽

Vol 7 ◽

pp. CMC.S10702 ◽

Cited By ~ 1

Author(s):

Taysir S. Garadah ◽

Najat A. Mahdi ◽

Ahmed M. Jaradat ◽

Zuheir A. Hasan ◽

Das S. Nagalla

Keyword(s):

Regression Analysis ◽

Serum Ferritin ◽

Serum Ferritin Level ◽

Systolic Function ◽

Ferritin Level ◽

Thalassemia Major ◽

Tissue Doppler ◽

Thyroid Stimulating Hormone ◽

Beta Thalassemia ◽

Pulsed Doppler

Background Thyroid gland dysfunction and echocardiographic cardiac abnormalities are well-documented in patients with transfusion dependent beta-thalassemia major (β-TM). Aim This cross-sectional analytic study was conducted to investigate left ventricle (LV) diastolic and systolic function using pulsed Doppler (PD) and tissue Doppler (TD) echocardiography and correlate that with serum level thyroid stimulating hormone in patients with β-TM. Methods The study was conducted on patients with β-TM (n = 110, age 15.9 ± 8.9 years) and compared with a control group (n = 109, age 15.8 ± 8.9 years). In all participants, echocardiographic indices of PD and TD were performed and blood samples were withdrawn for measuring the serum level of TSH, free T4, and ferritin. A linear regression analysis was performed on TSH level as the dependent variable and serum ferritin as independent. Stepwise multiple regression analysis was used to determine the odds ratio of different biochemical and echo variables on the risk of developing hypothyroidism. Results Patients with β-TM compared with controls had thicker LV septal wall index (0.65 ± 0.26 vs. 0.44 ± 0.21 cm/M2, P < 0.001), posterior wall index (0.65 ± 0.23 vs. 0.43 ± 0.21 cm/m2, P < 0.01) and larger LVEDD index (4.35 ± 0.69 vs.3.88 ± 0.153 mm/m2, P < 0.001). In addition, β-TM patients had higher transmitral E wave velocity (E) (70.81 ± 10.13 vs. 57.53 ± 10.13 cm/s, P = 0.02) and E/A ratio (1.54 ± 0.18 vs. 1.23 ± 0.17, P < 0.01) and shorter deceleration time (DT) (170.53 ± 13.3 vs. 210.50 ± 19.20 m sec, P < 0.01). Furthermore, the ratio of transmitral E wave velocity to the tissue Doppler E wave at the basal septal mitral annulus (E/Em) was significantly higher in the β-TM group (19.68 ± 2.81 vs. 13.86 ± 1.41, P < 0.05). The tissue Doppler systolic wave (Sm) velocity and the early diastolic wave (Em) were significantly lower in the β-TM group compared with controls with Sm, 4.82 ± 1.2 vs. 6.22 ± 2.1 mm/sec, P < 0.05 and (Em), 3.51 ± 2.7 vs. 4.12 ± 2.5 mm/sec. P < 0.05, respectively). The tricuspid valve velocity was significantly higher in β-TM patients compared with controls 2.85 ± 0.56 vs. 1.743 ± 0.47 m sec, respectively, P < 0.01). The prevalence of subclinical hypothyroidism in patients with β-TM was 15.4%, with significantly higher mean serum TSH compared with controls (6.78 ± 1.5 vs. 3.10 ± 1.02 μIU/mL, P < 0.01) and positively correlated with the serum ferritin level ( r = 0.34, P = 0.014). On multiple regression analysis, the LV mass, LVEF%, and E/A ratio were not positive predictors of hypothyroidism in patients with β-TM. Conclusion We conclude that patients with β-TM had a high prevalence of subclinical hypothyroidism of 15.4%. Thyroid stimulating hormone was significantly high and positively correlated with the serum ferritin level. Echo cardiographic pulsed Doppler showed a restrictive LV diastolic pattern suggestive of severe diastolic dysfunction with preserved left ventricle systolic function.

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BETA THALASSEMIA MAJOR;

The Professional Medical Journal ◽

10.29309/tpmj/2017.24.02.521 ◽

2017 ◽

Vol 24 (02) ◽

pp. 315-321

Author(s):

Asma Mehreen ◽

Saeeda Bano ◽

Bushra Ujala

Keyword(s):

Serum Ferritin ◽

Serum Ferritin Level ◽

Controlled Trial ◽

Ferritin Level ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Baseline Serum ◽

Beta Thalassemia Major ◽

Group A ◽

Group B

Introduction: β-Thalassemia major (β-TM) is a genetic haemoglobin disorderwhich is relatively common in some geographical areas. β-TM is characterized by severe anaemia,which needs a continuous blood transfusion regimen starting from the first months of life toprolong survival. Objectives: To compare mean reduction in serum ferritin level with deferasiroxand desferrioxamine when used as an iron chelator in multi-transfused beta thalassemia major.Study design: Randomized controlled trial. Setting: Thalassemia clinic, The Children’s Hospitaland The Institute of Child Health, Lahore. Duration of Study with Dates: Study was carried outover a period of nine months from 28-06-2015 to 27-03-2016. Subjects and Methods: A totalof 100 patients (50 patients in each group). The patients were randomly allocated into twogroups using random numbers stable. Group-A received Deferasirox and group-B receivedDesferrioxamine. Results: Mean age of the patients was 7.42±4.13 and 7.87±4.13 in group-Aand B, respectively. Regarding sex distribution, 26 patients (52.0%) in group-A and 28 patients(56.0%) in group-B were male while 24 patients (48.0%) in group-A and 22 patients (44.0%) ingroup-B were female. Reduction from baseline in group-A was 783.60±413.66 ng/ml and ingroup-B 552.80±155.45 ng/ml (P<0.001) There was more reduction in group-A. In group-Abaseline serum ferritin level was 2495.00±1259.10 ng/ml and at 9 month 1712.00±1019.36 ng/ml (P<0.001). Similarly in group-B baseline serum ferritin level was 2422.80±910.43 ng/ml andat 9 month 1883±862.72 ng/ml (P<0.001). Conclusion: In conclusion, deferasirox was moreeffective in terms of reduction in serum ferritin level when compared with desferrioxamine inmulti-transfused beta thalassemia major patients.

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The correlation between ferritin level and cardiac dysfunction in patients with thalassemia

Paediatrica Indonesiana ◽

10.14238/pi43.1.2003.24-7 ◽

2016 ◽

Vol 43 (1) ◽

pp. 24

Author(s):

Fajar Subroto ◽

Bulan Ginting Munthe ◽

Najib Advani ◽

Agus Firmansyah

Keyword(s):

Serum Ferritin ◽

Cardiac Dysfunction ◽

Serum Ferritin Level ◽

Ferritin Level ◽

Thalassemia Major ◽

Cross Sectional Study ◽

Cross Sectional ◽

Grade Ii ◽

Laboratory Examinations

Background Patients with b-thalassemia major, long-term trans-fusion, extravasal hemolytic, and increased intestinal absorptionof iron result in systemic iron overload, which may accumulate inmyocardium causing cardiac dysfunctions. Congestive heart fail-ure usually develops in adolescence or early adult years, and pa-tient usually dies within 1 year after the onset of symptoms. There-fore, it is important to detect early signs of cardiac dysfunction inpatient with thalassemia.Objective This study aimed to assess the correlation betweenferritin level and cardiac dysfunction in patients with thalassemia.Methods A cross sectional study was conducted on 62 b-thalas-semic patients (34 males and 28 females) with age ranging from3.5 to 23 years. They received 3,150 – 94,985 mL of blood; withthe serum ferritin level of 812.2 – 12,175 ng/mL. Each patient un-derwent laboratory examinations and clinical cardiac evaluationwith ECG and echocardiography.Results Cumulative blood transfusions correlated well with theserum ferritin values (p=0.001). The serum ferritin level did notshow correlation with deferoxamine (DFO) treatment. ECG exami-nation revealed 5 patients (8.1%) with dysrhythmia, LVH, and heartblock grade II and I. Echocardiography examination showed 18patients (29%) with systolic or diastolic dysfunction. There was nocorrelation between the serum ferritin level and cardiac dysfunc-tions (p=0.640). The serum ferritin prediction value against car-diac dysfunctions could not be established.Conclusion There was no correlation between serum ferritin lev-els with cardiac dysfunctions. In detecting cardiac dysfunctions inthalassemic patients, echocardiography was more sensitive thanECG

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Association of Body mass index and serum ferritin level in pediatrics with Beta-thalassemia major disease

Iranian Journal of Pediatric Hematology & Oncology ◽

10.18502/ijpho.v12i1.8359 ◽

2022 ◽

Author(s):

Saeed Yousefian ◽

Ghassem Miri Aliabad ◽

Rana Saleh ◽

Majid Khedmati

Keyword(s):

Body Mass Index ◽

Blood Transfusion ◽

Body Mass ◽

Serum Ferritin ◽

Serum Ferritin Level ◽

Ferritin Level ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Major Disease ◽

Beta Thalassemia Major

Background: Beta-thalassemia major is a type of inherited blood disease that results in variable outcomes such as severe anemia due to haemoglobin chains. Recurrent and lifelong blood transfusions as a treatment in beta-thalassemia major disease lead to iron deposition in various organs and cause the failure of multiple organs. Failure of affected organs leads to Body mass index (BMI) abnormality. This study aimed to evaluate the association between BMI and serum ferritin level as a marker for iron overload. Materials and Methods: A cross-sectional study designed and conducted with total number of 740 paediatrics, with mean age about 14.2±8.7 years old and with beta-thalassemia major requiring recurrent blood transfusion. Patient information, including demographics, serum ferritin level and percentage of BMI, was recorded and analysed by SPSS 25.0 and the statistical significant level, considered as 0.05. Results: A total number of 740 paediatrics with beta-thalassemia major disease (mean age about 14.2±8.7 years) were included to study to examine the association between serum ferritin level and their BMI. The total mean serum level of ferritin calculated about 3326 ± 3859 Nanogram/mililitter (ng/ml). Totally, 447 (60.4%) case of them had BMI percentile less than 5%, 274 (37.02%), 16 (2.16%) and 3 (0.4%) had BMI percentile 5%-85%, 85%-95% and more than 95%. There was no relation between gender and serum ferritin levels. The relationship between age and BMI has been positive (P=0.002). Finally, it resulted that there was a negative relationship between the BMI percentile and mean serum ferritin levels in paediatrics with beta-thalassemia major (P=0.031). Conclusion: Frequent Blood transfusion is associated with elevated serum ferritin level in paediatrics with beta-thalassemia major disease and experiencing lower percentiles of BMI in these patients.

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Relation of Serum Ferritin Level with Serum Hepcidin and Fucose Levels in Children with β-Thalassemia Major

Hemoglobin ◽

10.1080/03630269.2021.1898419 ◽

2021 ◽

Vol 45 (1) ◽

pp. 69-73

Author(s):

Salah H. AL-Zuhairy ◽

Mohammed A. Darweesh ◽

Mohammed A-M. Othman

Keyword(s):

Serum Ferritin ◽

Serum Ferritin Level ◽

Ferritin Level ◽

Thalassemia Major ◽

Serum Hepcidin

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Zinc Status in Beta-Thalassemia Major

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2021.5688 ◽

2021 ◽

Vol 9 (B) ◽

pp. 149-153

Author(s):

Khalaf Hussein Hasan ◽

Hasan Abdulla Aswad ◽

Aspazija Sofijanova

Keyword(s):

Serum Ferritin ◽

Ferritin Level ◽

Disease Onset ◽

Serum Zinc ◽

Thalassemia Major ◽

Adverse Outcomes ◽

Beta Thalassemia ◽

Control Group ◽

Healthy Children ◽

Zinc Status

BACKGROUND: Zinc is one of the most important minerals incorporated in the enzymes of the human body. Zinc may be deficient in patients with the β-thalassemia major with possible adverse outcomes. AIM: The purpose of this study was to assess the serum zinc status in β-thalassemia major patients in Duhok city. PATIENTS, MATERIAL, AND METHODS: In this case–control study, 70 children with β-thalassemia major (2–12 years) of both genders were enrolled and were matched with 70 apparently healthy children for age and sex. A venous blood sample was obtained from each child for the measurement of serum zinc and serum ferritin levels at Jin Center in Duhok City between January 1 and June 30, 2017. RESULTS: The mean serum zinc in the thalassemia patients (74.79 [±25.14] μg/dl) was significantly lower compared to the control group (93.61 [±15.12] μg/dl), (p = 0.0001). The serum zinc was not significantly different in thalassemia patients in terms of age, disease onset, gender, height, weight, body mass index, amount of blood transfusion, and type of chelation. There was a statistically significant correlation between serum zinc levels with a serum ferritin level of patients. CONCLUSION: The study showed that thalassemia patients have significantly lower serum levels of zinc with no relation to medical factors.

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