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

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.

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

The Open Psychology Journal ◽

10.2174/1874350101912010076 ◽

2019 ◽

Vol 12 (1) ◽

pp. 76-83 ◽

Cited By ~ 1

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.

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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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CUSTOM-MADE SUBPERIOSTEAL IMPLANT IN JAWS OF BETA-THALASSEMIA MAJOR PATIENT

10.26226/morressier.578e3b52d462b80292382e62 ◽

2016 ◽

Author(s):

Daniel Oren

Keyword(s):

Thalassemia Major ◽

Beta Thalassemia ◽

Beta Thalassemia Major ◽

Custom Made ◽

Thalassemia Major Patient ◽

Subperiosteal Implant

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Beta Thalassemia Major with Diabetes Mellitus: A Case Report

VIMS Health Science Journal ◽

10.46858/vimshsj.8208 ◽

2021 ◽

Vol 8 (2) ◽

pp. 85-88

Author(s):

Dr. Sneha Mhaske ◽

Dr. Abhijit Shinde ◽

Dr. Sonal Shinde ◽

Dr. Ramesh Kothari ◽

Dr. Ganesh Misal ◽

...

Keyword(s):

Diabetes Mellitus ◽

Blood Sugar Level ◽

Chelating Agent ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Laboratory Examination ◽

Beta Thalassemia Major ◽

Thalassemia Major Patient ◽

Urine Sugar ◽

Hemoglobin Production

Thalassemia results from defects in normal hemoglobin production, and represents the most common inherited anemia worldwide. Diabetes is a complication of b-thalassemia major. We report a case of Diabetes mellitus in a known case of beta thalassemia major. Patient had undergone Splenectomy 1 year back. Patient is taking chelating agent Defasirox 1000mg orally once a day in the morning. Family history reveals, born through third degree consanguineous marri age. The patient was then subjected for laboratory examination reveals BSL was high, urine ketone 2+,urine sugar 3+, ABG was normal, HbA1c was 13 & 3 month old report of serum ferritin 1200 ng/dl. Multidisciplinary management was instituted. Blood sugar level got controlled over subcutaneous insulin. Patient may have landed in Diabetic ketoacidosis but was promptly diagnosed & treated. This case is presented for its rarity. As the life expectancy of patients with thalassaemia increases, this will also expose our patients potentially to many more years of hyperglycaemia and diabetes. Sustaining metabolic control and controlling cardiovascular risk factors will be critical in the future for preventing complications due to diabetes.

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