scholarly journals BETA THALASSEMIA MAJOR IN A DEVELOPING COUNTRY: EPIDEMIOLOGICAL, CLINICAL AND EVOLUTIONARY ASPECT

2013 ◽  
Vol 5 (1) ◽  
pp. e2013002 ◽  
Author(s):  
Mohamed Bejaoui ◽  
Naouel Guirat
Keyword(s):  
Low Income ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Small Towns ◽  
Infectious Complications ◽  
Beta Thalassemia ◽  
North West ◽  
Beta Thalassemia Major ◽  
The North ◽  
Mediterranean Countries

Beta-thalassemia major (TM) remains to be one of the major health problems particularly in developing countries. Tunisia is a part of the Mediterranean countries mostly affected by this disease which is highly concentrated in small towns in families with low-income earners. The main objectives of this study are to provide a description of the demographic, clinical features and transfusion-related complications in patients with TM living in Tunisia. A standardized questionnaire was sent to clinicians throughout 33 different medical institutions caring for thalassemic patients. 391 transfusion dependant thalassemic patients with a median age of 10.7 years (range 3 months- 31 years) were included in the study.The majority were originated from the north west of the country .A moderate overload between 1501 and 2500ng/ml was found in 61patients, while 81 patients (26.9%) had ferritin level more than 2500 ng/ml and greater than 5000ng/ml in 21 patients (6.9%). 51 patients died from complications related to their disease. Heart failure was the main cause of death. The incidence of cardiac, endocrine, and infectious complications will be reviewed. Preventive measures such as health education, carrier screening and premarital screening remain the best ways for lowering the incidence of these diseases, which might be reflected in financial saving, social benefits and health benefits.

scholarly journals Influence of Working Memory on Academic Achievement and Quality of Life in Children with Beta-Thalassemia Major

2019 ◽  
Vol 12 (1) ◽  
pp. 76-83 ◽  
Author(s):  
Uni Gamayani ◽  
Ni Luh Meidha Dini Lestari ◽  
Ahmad Rizal Ganiem ◽  
Ramdan Panigoro
Keyword(s):  
Quality Of Life ◽  
Academic Achievement ◽  
Working Memory ◽  
Digit Span ◽  
Ferritin Level ◽  
Memory Function ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major

Background: Children with beta-thalassemia major may suffer from working memory impairment. For a more refined understanding of this issue, we assessed working memory function in beta-thalassemia children and evaluated its influence on academic achievement and quality of life. Methods: This was a cross-sectional study involving 60 beta-thalassemia children aged 8-12 years. All participants underwent a working memory assessment using the digit span and were interviewed using academic achievement and Indonesian version of PedsQL 4.0 questionnaires. Working memory in beta-thalassemia children significantly influences their quality of life, both directly (β=0.32) and indirectly, through their academic achievement (β=0.639). Longer duration of transfusion (p=0.01) is significantly related to poorer working memory, while lower hemoglobin level (p=0.81) and higher ferritin level (p=0.24) are not significantly associated with working memory. Conclusion: We concluded that working memory influences the quality of life in beta-thalassemia children, both directly and indirectly, through their academic achievement.

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

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.

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

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

scholarly journals Iron chelation therapy needed for serum ferritin overloaded patients of beta thalassemia major

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Wasim Muhammad ◽  
Muhammad Ishaq ◽  
Muhammad J. Khan ◽  
Umair Ahmad ◽  
Muhammad Waseem
Keyword(s):  
Serum Ferritin ◽  
Ferritin Level ◽  
Iron Chelation ◽  
Care Facility ◽  
Health Care Facility ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Cross Sectional ◽  
Beta Thalassemia Major ◽  
Khyber Pakhtunkhwa Province

The main objective of the current study is to evaluate the level and overload of serum ferritin in multi-transfused beta Thalassemia major patients. There is an earnest need to defend the chelation treatment and to make mindfulness about the results of serum ferritin in the patients beta Thalassemia major. This is a Cross sectional analytical study performed in Fatimid foundation Hayatabad, Peshawar, Khyber Pakhtunkhwa province of Pakistan. Those patients who has beta thalassemia major are included in this study. In this study there are total 108 patients in which 54 males and 54 females. The highest mean of serum ferritin level in the category of male was in the age of 12 years were finds 8160.5 ng/mL. Among the female the highest mean of ferritin level was in the age of 17 years were finds 13,349.5 ng/mL. In this study majority of patient’s revealed much high levels of serum ferritin. These levels reveal insufficient chelation. Appropriate chelation of iron load can improve the quality of the life of these patients. The low level of education, Poverty problems, and insufficient health care facility of are the main obstacle in the effective management of ferritin overload in thalassemia patients.

scholarly journals BETA THALASSEMIA MAJOR;

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.

scholarly journals Thyroid profile in patients of thalassemia with multiple blood transfusions and high serum ferritin: a cross-sectional study

2020 ◽  
Vol 7 (8) ◽  
pp. 401-408
Author(s):  
Dr. Rohit Khandelwal ◽  
◽  
Dr. Muralidhar Gundluru ◽  
Dr. Leeni Mehta K. ◽  
◽  
...  
Keyword(s):  
Serum Ferritin ◽  
Thyroid Dysfunction ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
High Serum ◽  
The Body ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Beta Thalassemia Major ◽  
Tsh Levels

Introduction: Beta-thalassemia major patients undergo regular blood transfusion resulting ingrowth faltering and various endocrine problems including thyroid dysfunction due to iron overloadin the body. This study was conducted to determine the frequency of thyroid dysfunction in childrenpresenting with Beta-thalassemia major on regular blood transfusions. Materials and methods:Sixty children were included with proven beta-thalassemia major who reported to the Department ofPediatrics, VIMS, and RC, Bangalore. Inclusion criteria: 1.Children 4 to 18 years age group .2.Thechild received transfusions for more than 2 years. 3.Children with serum ferritin level >700.Results: In this study, four patients(6.8%) had overt hypothyroidism, eight patients(13.6%) hadsubclinical hypothyroidism and 47 patients(79.7%) had euthyroid status. There was a positivecorrelation between Ferritin and T4, TSH levels. i.e., with an increase in Ferritin level, there was anincrease in T4, TSH levels, and vice versa. However, the correlation was significant with TSH. Therewas a significant negative correlation between Ferritin and T3 levels. i.e with an increase in Ferritinlevel, there was a decrease in T3 levels and vice versa. Conclusion: Thyroid dysfunction can exist inthalassemia patients on multiple transfusions and chelation therapy with high serum ferritin levels.Detection of hypothyroidism is important as inexpensive oral replacement therapy is readilyavailable. Hence regular screening of beta-thalassemia major patients for Serum T3, Serum T4,Serum TSH for early detection and timely treatment could improve the life expectancy and quality oflife of these patients.

scholarly journals HUBUNGAN KADAR HEMOGLOBIN DAN FERITIN DENGAN GAMBARAN KONDUKSI SARAF PADA ANAK TALASEMIA BETA MAYOR

2017 ◽  
Vol 35 (1) ◽  
Author(s):  
Windy Krisanti Kusuma Dewi ◽  
Uni Gamayani ◽  
Nushrotul Lailiyya ◽  
Lelani Reniarti ◽  
Nanan Sekarwana
Keyword(s):  
Nerve Conduction Study ◽  
Nerve Conduction ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Hemoglobin Level ◽  
Sensory Nerves ◽  
Beta Thalassemia ◽  
Cross Sectional ◽  
Beta Thalassemia Major ◽  
The Mean

    CORRELATION BETWEEN HAEMOGLOBINE AND FERRITIN LEVEL WITH NERVE CONDUCTION STUDY IN CHILDREN WITH BETA THALASSEMIA MAJORABSTRACTIntroduction: Beta thalassemia major is inherited hemoglobin synthesis disorder found in thalassemia belt, including Indonesia. Peripheral neuropathy is one of its underdiagnosed neurological complications results in poor management of the patients.Aims: To identify correlation between hemoglobin and ferritin serum with Nerve conduction study (NCS) in Beta thalassemia major patients.Methods: This was a cross-sectional study involving children with beta thalassemia major aged 8-14 years, who regularly underwent blood transfusions in Hasan Sadikin Hospital, Bandung. Nerve conduction study of motor and sensory nerves of all limbs were conducted to the patients. Data was analyzed using Spearman’s correlation analysis.Results: Fifty subjects were included in this study with the mean hemoglobin level (SD) of 6.99 (0.87)g/dL and the mean blood ferritin level (SD) of 3.925 (1.993)μg/L. Based on NCS, most of the subjects had polyneuropathy and 46.94% had axonal demyelinating lesions in sensory and motor nerves. Among patients with neuropathy, there was a statistically significant negative correlation  between mean hemoglobin level and the numbers of the abnormal  nerves and also a statistically significant positive correlation between mean blood ferritin level and the numbers of the abnormal nerves.Discussion: The lower ferritin serum level, the less nerve involved based on NCS.Keyword: Beta thalassemia, ferritin, hemoglobin, nerve conduction study, neuropathyABSTRAKPendahuluan: Talasemia beta mayor merupakan suatu kelainan sintesis hemoglobin yang herediter yang banyak ditemukan di wilayah tropis, termasuk Indonesia. Salah satu komplikasinya adalah neuropati perifer yang sering tidak terdiagnosis, sehingga penyandangnya tidak mendapatkan tata laksana yang adekuat.Tujuan: Mengetahui hubungan antara kadar hemoglobin dan feritin serum dengan gambaran konduksi saraf (nerve conduction study/NCS) pada penyandang talasemia beta mayor.Metode: Studi potong lintang terhadap penyandang talasemia berusia 8-14 tahun yang rutin menjalani transfusi darah di RSUP Dr. Hasan Sadikin, Bandung. Dilakukan pemeriksaan NCS motorik dan sensorik pada keempat ekstremitas. Data dianalisis menggunakan uji korelasi Spearman.Hasil: Diperoleh 50 subjek dengan rerata kadar hemoglobin 6,99+0,87g/dL dan kadar feritin serum 3.925+1.993μg/L. Berdasarkan pemeriksaan NCS, sebagian besar subjek mengalami polineuropati yang 46,94% di antaranya berupa gambaran demielinasi aksonal sensorik motorik. Pada subjek dengan neuropati perifer didapatkan korelasi negatif yang bermakna antara kadar hemoglobin dengan jumlah keterlibatan saraf pada NCS dan korelasi positif yang bermakna antara kadar feritin serum dengan jumlah keterlibatan saraf pada NCS.Diskusi: Semakin rendah kadar feritin serum, maka semakin sedikit jumlah saraf yang terlibat pada NCS, dan sebaliknya.Kata kunci: Feritin, hemoglobin, nerve conduction study, neuropati, talasemia beta mayor

Paradoxically Increased Ferritin Level in a Beta-Thalassemia Major Patient following the Start of Deferasirox Chelation Therapy

2010 ◽  
Vol 123 (2) ◽  
pp. 117-120
Author(s):  
Paolo Ricchi ◽  
Massimiliano Ammirabile ◽  
Anna Spasiano ◽  
Silvia Costantini ◽  
Patrizia Cinque ◽  
...  
Keyword(s):  
Chelation Therapy ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major ◽  
Thalassemia Major Patient

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

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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