scholarly journals Protective Effects of Selenium in Patients with Beta-Thalassemia Major

2020 ◽  
Vol 26 (1) ◽  
pp. 25-31
Author(s):  
Ajand Aboutalebi ◽  
Abolghasem Jouyban ◽  
Hadi Chavoshi ◽  
Aliakbar Movassaghpour Akbari ◽  
Elnaz Shaseb ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Iron Overload ◽  
Kidney Function ◽  
Hair Loss ◽  
Binding Capacity ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Severe Renal Failure ◽  
Beta Thalassemia Major ◽  
Liver And Kidney

Background : Beta-thalassemia major patients require repeated blood transfusion which is associated with iron overload in different organs such as heart, liver, kidney and their related complications. In this study the effects of selenium in iron overload related complications of patients with beta-thalassemia major were assessed. Methods: In this clinical trial, 34 beta-thalassemia major patients over 12 years old were enrolled. Patients with severe renal failure, history of selenium consumption over the last three months, change of blood transfusion pattern, and any change of chelating agent were excluded from the study. For all patients, tablet of selenium 200 µg/day was administered for a month. Blood samples were taken at baseline and after one-month to assess the level of ferritin, total iron-binding capacity (TIBC), aspartate aminotransferase (AST), alanine aminotransferase (ALT), serum creatinine (Scr), selenium. Hair loss was assessed by questionnaire before and after intervention. Results: From 34 patients, 27 (79.4%) had deficient level of selenium at baseline. The selenium level was increased after intervention (p=0.005). The level of serum ALT and Scr decreased remarkably after one-month selenium consumption (p=0.007 for both). In addition, the AST level decreased remarkably after intervention (p=0.053). Severe hair loss profile has improved significantly after supplementation (p=0.004). Conclusion: One-month selenium consumption improved liver and kidney function related markers remarkably. Moreover, selenium improved hair profile and severe hair loss in thalassemia patients. Further studies are needed on the effect of selenium administration on liver and kidney function.

scholarly journals Left Atrial Strain Identifies Increased Atrial Ectopy in Patients with Beta-Thalassemia Major

Diagnostics ◽  
2020 ◽  
Vol 11 (1) ◽  
pp. 1
Author(s):  
Maria Vlachou ◽  
Vasileios Kamperidis ◽  
Efthymia Vlachaki ◽  
Georgios Tziatzios ◽  
Despoina Pantelidou ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Left Atrial ◽  
Systolic Pressure ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Tape Recording ◽  
Blood Transfusions ◽  
Systolic Strain ◽  
Peak Systolic Strain ◽  
Beta Thalassemia Major

Patients with beta-thalassemia major (β-ΤΜ) may develop cardiac arrhythmias through a multifactorial mechanism. The current study evaluated the association of cardiac structure and function on echocardiography with atrial ectopic burden on 24-hour tape recording in β-ΤΜ patients. This prospective study included consecutive β-ΤΜ patients. Demographic, laboratory, echocardiographic, cardiac magnetic resonance (CMR) T2* and 24-hour tape recording data were prospectively collected. The patients were classified according to the median value of premature atrial contractions (PACs) on 24-hour tape. In total, 50 β-TM patients (37.6 ± 9.1 years old, 50% male) were divided in 2 groups; PACs ≤ 24/day and > 24/day. Patients with PACs > 24/day were treated with blood transfusion for a longer period of time (39.0 ± 8.6 vs. 32.0 ± 8.9 years, p < 0.007), compared to their counterparts. Older age (OR: 1.121, 95% CI: 1.032–1.217, p = 0.007), longer duration of blood transfusion (OR:1.101, 95% CI:1.019–1.188, p = 0.014), larger LV end-diastolic diameter (OR: 4.522, 95% CI:1.009–20.280, p = 0.049), higher values of LA peak systolic strain (OR: 0.869, 95% CI: 0.783–0.964, p = 0.008), higher MV E/E′ average (OR: 1.407, 95% CI: 1.028–1.926, p = 0.033) and higher right ventricular systolic pressure (OR: 1.147, 95% CI: 1.039–1.266, p = 0.006) were univariably associated with PACs > 24/day. LA peak systolic strain remained significantly associated with PACs > 24/day after adjusting for the duration of blood transfusions or for CMR T2*. The multivariable model including blood transfusion duration and LA peak systolic strain was the most closely associated with PACs > 24/day. Receiver operating characteristic curve analysis identified a left atrial peak systolic strain of 31.5%, as the best cut-off value (83% sensitivity, 68% specificity) for prediction of PACs > 24/day. In β-TM patients, LA peak systolic strain was associated with the atrial arrhythmia burden independently to the duration of blood transfusions and CMR T2*.

scholarly journals Liver Enzymes in Children with beta-Thalassemia Major: Correlation with Iron Overload and Viral Hepatitis

2015 ◽  
Vol 3 (2) ◽  
pp. 287-292 ◽  
Author(s):  
Khaled M. Salama ◽  
Ola M. Ibrahim ◽  
Ahmed M. Kaddah ◽  
Samia Boseila ◽  
Leila Abu Ismail ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Iron Overload ◽  
Viral Hepatitis ◽  
Serum Ferritin ◽  
Liver Enzymes ◽  
Transferrin Saturation ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Elevated Liver Enzymes ◽  
Hcv Antibody

BACKGROUND: Beta Thalassemia is the most common chronic hemolytic anemia in Egypt (85.1%) with an estimated carrier rate of 9-10.2%. Injury to the liver, whether acute or chronic, eventually results in an increase in serum concentrations of Alanine transaminase (ALT) and Aspartate transaminase (AST).AIM: Evaluating the potentiating effect of iron overload & viral hepatitis infection on the liver enzymes.PATIENTS AND METHODS: Eighty (80) thalassemia major patients were studied with respect to liver enzymes, ferritin, transferrin saturation, HBsAg, anti-HCV antibody and HCV-PCR for anti-HCV positive patients.RESULTS: Fifty % of the patients were anti-HCV positive and 55% of them were HCV-PCR positive. Patients with elevated ALT and AST levels had significantly higher mean serum ferritin than those with normal levels. Anti-HCV positive patients had higher mean serum ferritin, serum ALT, AST and GGT levels and higher age and duration of blood transfusion than the negative group. HCV-PCR positive patients had higher mean serum ferritin and serum ALT and also higher age and duration of blood transfusion than the negative group.CONCLUSION: Iron overload is a main leading cause of elevated liver enzymes, and presence of HCV infection is significantly related to the increased iron overload.

scholarly journals Guidelines on Beta-thalassemia major – regular blood transfusion therapy: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: project guidelines: Associação Médica Brasileira – 2016

2016 ◽  
Vol 38 (4) ◽  
pp. 341-345 ◽  
Author(s):  
Dante Langhi ◽  
Eugênia Maria Amorim Ubiali ◽  
José Francisco Comenalli Marques ◽  
Mônica de Almeida Verissimo ◽  
Sandra Regina Loggetto ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Transfusion Therapy ◽  
Beta Thalassemia Major

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.

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